Sunday, March 28, 2010

Nor-98 atypical Scrapie, atypical BSE, spontaneous TSE, trade policy, sound science ?

Nor-98 atypical Scrapie, atypical BSE, spontaneous TSE, trade policy, sound science ?


Greetings,

I would like to kindly comment on a few assumptions that are being made about the atypical Scrapie Nor-98, and why in my opinion, the human and animal fallout from the atypical scrapie, and the atypical BSE i.e. the H and the L (to date), is unknown (as with the typical scrapie strains and typical c-BSE), due to the fact the decission to classify the Nor-98 atypical scrapie, and the other atypical TSE, as a legal trading commodity, under the guise that they are of no risk to humans or animals. This has simply not been proven, and in fact, there is much science to show that both typical and atypical scrapie, and BSE in sheep and goats, and the typical and atypical BSE in the bovine, ARE a risk to humans and animals, and this has been documented with studies to date (see transmission studies below). The USDA, and the O.I.E. et al have decided that it is o.k. to trade these atypical TSE globally, just for trade, that's all that matters. I urge all Country's to repeal the BSE MRR policy, and declare it as 'just science', as it should have never been implemented in the first place. I urge the scientific community to declare this as so. I also urge that we go back to a new and modern and improved TSE GBR risk assessment, and that trade policy are no long made by the industry i.e. O.I.E., and the U.S.D.A. et al. As these human and animal TSE mutate, they become more virulent in some cases, and this has been proven with atypical l-BSE. h-BSE transmits to humans via lab studies (Kong et al 2009). we know that typical scrapie will transmit by it's non-forced oral consumption of scrapie materials to non-human primate. human cjd will transmit to non-human primate orally. we now have two different strains of CWD in the USA i.e. the new 'Wisconsin strain'. With Scrapie and CWD spreading in the USA. The hyper and drowzy TME in mink. There are over 20 strains of typical scrapie documented to date, with now the Nor-98 atypical scrapie, that the USDA and OIE et al not wanting to call it a scrapie at all, even with science now showing that the Nor-98 atypical Scrapie will in fact consistently transmit within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage. In short, in my opinion, once again, sound science has been trashed, and sadly, political junk science has come forth due to International Trade, and 'we the consumer' could play a heavy toll in the years and decades to come, due to the 'friendly fire' and or the 'pass if forward' modes of transmission of TSE i.e. medical, dental, surgical arenas. PLEASE NOTE HIGH INCIDENT OF SPORADIC CJD AND BSE IN THE ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? > 2 per million, another coincident, or BSE related ? Please remember, BSE will propagate as nvCJD and or sporadic CJD in humanized transgenic mice, and please NOTE ALSO, the close proximity of BASQUE COUNTRY SPAIN TO ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? SO, you have a high rate of sporadic CJD cases in an area of BSE and Nor-98 atypical scrapie cases, another spontaneous coincidence, or a related event ? ALL iCJD is, is sporadic CJD until the route and source of the TSE agent is documented, and second passage of TSE is not always an exact science or exact TSE as the first passage. I think in the name of the almighty dollar and trade, scientific policies are being made that risk human and animal health, and these policies are being made without any sound science at all, as far as risk factor goes via transmission studies. To date there has never been a documented case of any natural field case of spontaneous TSE. Our Governing bodies seem to be playing Russian Roulette in regards to Transmissible Spongiform Encephalopathy's and International Trade, by disregarding sound science, and in doing so, they are risking human and animal health across the globe. It seems that human and animal TSEs, and any death there from, either by consumption and or second, third, fourth... passage via the medical, surgical, and or dental arenas, due to the long incubation period, and the lack of being able to date to identify a route or source have simply become acceptable deaths from spontaneous events, and that is simply not acceptable.



>>> The detection of more intense PrPSc deposits in cerebellum or 10 cerebral cortex rather than in the medulla oblongata may indicate that the prion is likely not to enter the brain through the medulla (DMNV) as described for classical scrapie [58], thus suggesting a rather sporadic aetiology, as observed in human sporadic TSE cases...Atypical/Nor98 scrapie (AS/Nor98), by contrast, seems to occur sporadically or to be minimally contagious [18]. <<<


I disagree for the following reasons ; Subject: [CJD-L] SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION

Numero de focos de Encefalopatia Espongiforme Bovine en Espana 2009 - 1010

2009 = 18 cases BSE

http://www.eeb.es/pags/2009.htm



2010 to date = 4 cases of BSE

http://www.eeb.es/pags/espana.htm#2010



BSE SPAIN

Año 2000

http://www.eeb.es/pags/2000.htm



Año 2001

http://www.eeb.es/pags/2000.htm#2001



Año 2002

http://www.eeb.es/pags/2002.htm



Año 2003

http://www.eeb.es/pags/2003.htm



Año 2004

http://www.eeb.es/pags/2004.htm



Año 2005

http://www.eeb.es/pags/2005.htm



Año 2006

http://www.eeb.es/pags/2006.htm



Año 2007

http://www.eeb.es/pags/2007.htm



Año 2008

http://www.eeb.es/pags/2008.htm



Año 2009

http://www.eeb.es/pags/2009.htm



BSE SPAIN 2010 Situacion en Espana Total casos


http://www.eeb.es/pags/espana.htm#2010



PLEASE NOTE HIGH INCIDENT OF SPORADIC CJD AND BSE IN THE ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? another coincident, or BSE related. remember, BSE will propagate as nvCJD and or sporadic CJD in humanized transgenic mice, and please NOTE ALSO, the close proximity of BASQUE COUNTRY SPAIN TO ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? SO, you have a high rate of sporadic CJD cases in an area of BSE and Nor-98 atypical scrapie cases, another spontaneous coincidence, or a related event ? ...TSS

NOTE THE SPORADIC CJD INCIDENT RATE AT > 2 CASES PER MILLION ;

SEE CJD UPDATE 2010

SEE PAGE 4 ;

Notificaciones* al RNEETH (1993-febrero 2010)

. Por año diagnóstico. Existe 1 caso más sin año diagnóstico

AÑO

CCAA 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 TOTAL

Andalucia 4 6 2 2 5 4 8 11 16 14 9 11 16 15 13 19 11 1 167

Aragon 0 0 1 0 5 4 1 3 4 0 2 0 5 2 2 5 4 1 39

Asturias 2 0 2 0 1 2 3 2 2 0 5 2 1 0 0 0 0 0 22

Baleares 1 2 0 1 1 0 2 0 1 1 1 3 1 0 0 1 4 0 19

Canarias 0 0 0 0 4 4 4 2 2 2 2 2 2 3 3 1 3 0 34

Cantabria 1 2 0 0 2 4 3 3 0 2 0 0 1 0 0 2 0 0 20

Castilla la Mancha 0 1 1 2 2 1 2 2 1 2 0 2 5 3 5 6 7 0 42

Castilla-Leon 2 1 6 2 7 4 4 6 8 5 6 6 7 7 7 12 5 0 95

Cataluna 8 6 3 6 6 15 16 9 13 11 7 19 15 16 13 14 7 0 184

Valencia 1 3 3 7 5 12 11 10 12 13 17 10 8 8 19 16 9 2 166

Extremadura 0 0 0 1 1 2 2 0 0 3 1 0 2 7 5 3 1 0 28

Galicia 1 1 3 0 0 11 3 5 8 7 1 7 6 5 3 2 0 0 63

Madrid 4 4 5 7 5 7 9 10 16 7 12 8 13 16 8 8 13 0 152

Murcia 1 0 0 0 1 0 1 0 2 3 2 2 3 1 6 2 3 0 27

Navarra 1 0 0 2 1 0 2 2 1 0 0 0 4 2 0 0 0 0 15

Pais Vasco 2 3 1 6 4 8 5 8 8 9 7 6 11 10 11 8 4 1 112

La Rioja 2 0 0 0 1 1 0 0 1 0 1 0 1 0 1 0 0 0 8

TOTAL 30 29 27 36 51 79 76 73 95 79 73 78 101 95 96 99 71 5 1193

. Por año diagnóstico. Existe 1 caso más sin año diagnóstico

REGISTRO ESPAÑOL DE EETH

1993-febrero 2010

ECJ = 957

vECJ = 5

IFL = 44

GSS = 2

Esporadico = 905

Familiar = 46

Yatrogenico = 6

* Existen 2 casos mas con diagnóstico pendiente de clasificación

ALSO, ON PAGE 14, NOTE THE HIGH INCIDENCE IN THIS SAME AREA ;

Número de casos de Insomnio Familiar Letal por CA. 1993-febrero 2010

HIGH INCIENCE RATE AT 18 CASES

please see full text ;

REGISTRO NACIONAL DE ENCEFALOPATÍAS ESPONGIFORMES TRANSMISIBLES HUMANAS C.N.E y Servicios de Vigilancia Epidemiológica de CCAA (Situación a 15 de febrero de 2010)

http://www.isciii.es/htdocs/pdf/DatosRegistroCreutzfeldJacob2.pdf



snip...see ;


Sunday, March 28, 2010

SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION

Numero de focos de Encefalopatia Espongiforme Bovine en Espana 2009 - 1010

http://creutzfeldt-jakob-disease.blogspot.com/2010/03/spain-bse-nor-98-atypical-scrapie.html




typical Scrapie ;


1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

snip...

The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

PMID: 6997404

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract



Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6

http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf



Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).

http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html



EMBO reports 4, 5, 530–533 (2003) doi:10.1038/sj.embor.embor827 AOP Published online: 11 April 2003

Widespread PrPSc accumulation in muscles of hamsters orally infected with scrapie

Achim Thomzig, Christine Kratzel, Gudrun Lenz, Dominique Krüger & Michael Beekes

Robert Koch-Institut, P26, Nordufer 20, D-13353 Berlin, Germany

To whom correspondence should be addressed Michael Beekes Tel: +49 30 4547 2396; Fax: +49 30 4547 2609; BeekesM@rki.de

Received 13 February 2003; Accepted 13 March 2003; Published online 11 April 2003.

Abstract

Scrapie, bovine spongiform encephalopathy and chronic wasting disease are orally communicable, transmissible spongiform encephalopathies (TSEs). As zoonotic transmissions of TSE agents may pose a risk to human health, the identification of reservoirs for infectivity in animal tissues and their exclusion from human consumption has become a matter of great importance for consumer protection. In this study, a variety of muscles from hamsters that were orally challenged with scrapie was screened for the presence of a molecular marker for TSE infection, PrPSc (the pathological isoform of the prion protein PrP). Sensitive western blotting revealed consistent PrPSc accumulation in skeletal muscles from forelimb and hindlimb, head, back and shoulder, and in tongue. Previously, our animal model has provided substantial baseline information about the peripheral routing of infection in naturally occurring and orally acquired ruminant TSEs. Therefore, the findings described here highlight further the necessity to investigate thoroughly whether muscles of TSE-infected sheep, cattle, elk and deer contain infectious agents.

EMBO reports 4, 5, 530–533 (2003) doi:10.1038/sj.embor.embor827 AOP Published online: 11 April 2003

http://www.nature.com/embor/journal/v4/n5/full/embor827.html



EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE

This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........

http://web.archive.org/web/20030517224223/http://www.bseinquiry.gov.uk/files/yb/1994/02/07002001.pdf



Atypical Nor-98 Scrapie studies ;

P03.141

Aspects of the Cerebellar Neuropathology in Nor98

Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute,

Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf



PR-26

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway

Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion.

*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.

119

http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf



A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,?? +Author Affiliations

*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)

Abstract Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

http://www.pnas.org/content/102/44/16031.abstract



Monday, December 1, 2008

When Atypical Scrapie cross species barriers

Authors

Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France.

Content

Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence.

The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie.

Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.

Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.

(i) the unsuspected potential abilities of atypical scrapie to cross species barriers

(ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier

These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf



TAFS

INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation (May 16, 2007) TAFS1 Position Paper on Atypical scrapie and Atypical BSE


http://www.tafsforum.org/position_papers/TAFS_POSITION_PAPER_ON_ATYPICAL_SCRAPIE_AND_%20ATYPICAL_BSE_070516.pdf



Although BASE showed a significant barrier to primary transmission to inbred mouse lines resulting in preclinical infection, it remains of considerable concern whether the causal agent is potentially pathogenic for humans. In this regard, the neuropathological and molecular features of BASE—in particular, the biochemical type and deposition patterns of PrPSc—have striking similarities with those of a distinct subgroup of patients with sporadic CJD [16]. This finding requires a cautious interpretation. Nonetheless, the possibility that the origin of CJD in some patients with a sporadic disease phenotype may be related to BASE exposure has to be considered and verified through transmission studies.


http://www.plospathogens.org/article/info:doi/10.1371/journal.ppat.0030031



Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie Lescoutra-Etchegaray1, Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1, Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4, Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4, Jean-Philippe Deslys1

1 Institute of Emerging Diseases and Innovative Therapies, CEA, Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps Florida, Jupiter, Florida, United States of America, 5 Genetics Division, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States of America

Abstract Top Background Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains.

Methodology/Principal Findings Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.

Conclusion/Significance Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.

Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017

Editor: Neil Mabbott, University of Edinburgh, United Kingdom

Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20, 2008

Copyright: © 2008 Comoy et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: This work has been supported by the Network of Excellence NeuroPrion.

Competing interests: CEA owns a patent covering the BSE diagnostic tests commercialized by the company Bio-Rad.

* E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000110/!x-usc:mailto:emmanuel.comoy@cea.fr


http://www.plosone.org/article/info:doi/10.1371/journal.pone.0003017



Saturday, December 01, 2007

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model

Volume 13, Number 12–December 2007 Research

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model

Thierry Baron,* Anna Bencsik,* Anne-Gaëlle Biacabe,* Eric Morignat,* andRichard A. Bessen†*Agence Française de Sécurité Sanitaire des Aliments–Lyon, Lyon, France; and†Montana State University, Bozeman, Montana, USA

Abstract

Transmissible mink encepholapathy (TME) is a foodborne transmissible spongiform encephalopathy (TSE) of ranch-raised mink; infection with a ruminant TSE has been proposed as the cause, but the precise origin of TME is unknown. To compare the phenotypes of each TSE, bovine-passaged TME isolate and 3 distinct natural bovine spongiform encephalopathy (BSE) agents (typical BSE, H-type BSE, and L-type BSE) were inoculated into an ovine transgenic mouse line (TgOvPrP4). Transgenic mice were susceptible to infection with bovine-passaged TME, typical BSE, and L-type BSE but not to H-type BSE. Based on survival periods, brain lesions profiles, disease-associated prion protein brain distribution, and biochemical properties of protease-resistant prion protein, typical BSE had a distint phenotype in ovine transgenic mice compared to L-type BSE and bovine TME.The similar phenotypic properties of L-type BSE and bovine TME in TgOvPrP4 mice suggest that L-type BSE is a much more likely candidate for the origin of TME than is typical BSE.

snip...

Conclusion

These studies provide experimental evidence that the Stetsonville TME agent is distinct from typical BSE but has phenotypic similarities to L-type BSE in TgOvPrP4 mice. Our conclusion is that L-type BSE is a more likely candidate for a bovine source of TME infection than typical BSE. In the scenario that a ruminant TSE is the source for TME infection in mink, this would be a second example of transmission of a TSE from ruminants to non-ruminants under natural conditions or farming practices in addition to transmission of typical BSE to humans, domestic cats, and exotic zoo animals(37). The potential importance of this finding is relevant to L-type BSE, which based on experimental transmission into humanized PrP transgenic mice and macaques, suggests that L-type BSE is more pathogenic for humans than typical BSE (24,38).


http://www.cdc.gov/eid/content/13/12/1887.htm?s_cid=eid1887_e



Saturday, February 28, 2009

NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS TYPE BSE


"All of the 15 cattle tested showed that the brains had abnormally accumulated PrP"

2009 SEAC 102/2


http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html



Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?


http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html



''THE LINE TO TAKE'' ON IBNC $$$ 1995 $$$

1995

page 9 of 14 ;30. The Committee noted that the results were unusual. the questioned whether there could be coincidental BSE infection or contamination with scrapie. Dr. Tyrell noted that the feeling of the committee was that this did not represent a new agent but it was important to be prepared to say something publicly about these findings. A suggested line to take was that these were scientifically unpublishable results but in line with the policy of openness they would be made publicly available and further work done to test their validity. Since the BSE precautions were applied to IBNC cases, human health was protected. Further investigations should be carried out on isolations from brains of IBNC cases with removal of the brain and subsequent handling under strict conditions to avoid the risk of any contamination.31. Mr. Bradley informed the Committee that the CVO had informed the CMO about the IBNC results and the transmission from retina and he, like the Committee was satisfied that the controls already in place or proposed were adequate. ...

snip... see full text

http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf



SEE NEW URL, may take several attempts to load. ...tss


http://collections.europarchive.org/tna/20090114154722/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf



P02.35

Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK-resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.


http://www.neuroprion.com/pdf_docs/conferences/prion2007/abstract_book.pdf



Research Project: Study of Atypical Bse Location: Virus and Prion Diseases of Livestock

Project Number: 3625-32000-086-05 Project Type: Specific Cooperative Agreement

Start Date: Sep 15, 2004 End Date: Sep 14, 2009

Objective: The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.

Approach: This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.

http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490



Wednesday, February 11, 2009

Atypical BSE North America Update February 2009

Both of the BSE cases ascertained in the US native-born cattle were atypical cases (H-type), which contributed to the initial ambiguity of the diagnosis. 174, 185 In Canada, there have been 2 atypical BSE cases in addition to the 14 cases of the classic UK strain of BSE2: one was the H-type, and the other was of the L-type.198

snip...end

source :

Enhanced Abstract Journal of the American Veterinary Medical Association January 1, 2009, Vol. 234, No. 1, Pages 59-72

Bovine spongiform encephalopathy

Jane L. Harman, DVM, PhD; Christopher J. Silva, PhD

http://avmajournals.avma.org/doi/ref/10.2460/javma.234.1.59



Atypical BSE North America Update February 2009


http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html




sporadic CJD is not a single strain, but multiple strains, phenotypes of Creutzfeldt-Jakob disease with some 6 strains documented to date, and with atypical sub-strains of unknown origin growing in the USA ;



2006

USA sporadic CJD cases rising ;

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf



2008 - 2010

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.


http://www.cjdfoundation.org/fact.html



CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.


http://www.cjdsurveillance.com/pdf/case-table.pdf



Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***


http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html



my comments to PLosone here ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd




14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)

18.173 page 189

Experimental Challenge of Cattle with H-type and L-type Atypical BSE

A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada

Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.

Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE.

Results and Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types. Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.


http://www.isid.org/14th_icid/


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


http://www.isid.org/publications/ICID_Archive.shtml




14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

see page 114 ;


http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf




International Society for Infectious Diseases Web: http://www.isid.org

Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009

snip...

I ask Professor Kong ;

Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''

Professor Kong reply ;

.....snip

''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete.

Thanks for your interest.''

Best regards,

Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA

END...TSS

I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS


http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html



for those interested, please see full text ;

Friday, January 29, 2010 14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)


http://bse-atypical.blogspot.com/2010/01/14th-international-congress-on.html



Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION


http://cjdquestionnaire.blogspot.com/




Friday, February 05, 2010


New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review


http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html



Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

Piero Parchi Æ Rosaria Strammiello Æ Silvio Notari Æ Armin Giese Æ Jan P. M. Langeveld Æ Anna Ladogana Æ Inga Zerr Æ Federico Roncaroli Æ Patrich Cras Æ Bernardino Ghetti Æ Maurizio Pocchiari Æ Hans Kretzschmar Æ Sabina Capellari

Received: 30 June 2009 / Revised: 16 August 2009 / Accepted: 17 August 2009 / Published online: 29 August 2009

The Author(s) 2009. This article is published with open access at Springerlink.com

Abstract

Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenotypic features and concurrent PrPSc types has been reported but with inconsistencies among studies in both results and their interpretation. The issue currently complicates diagnosis and classification of cases and also has implications for disease pathogenesis. To explore the issue in depth, we carried out a systematic regional study in a large series of 225 cases. PrPSc types 1 and 2 concurrence was detected in 35% of cases and was higher in MM than in MV or VV subjects. The deposition of either type 1 or 2, when concurrent, was not random and always characterized by the coexistence of phenotypic features previously described in the pure subtypes. PrPSc type 1 accumulation and related pathology predominated in MM and MV cases, while the type 2 phenotype prevailed in VVs. Neuropathological examination best identified the mixed types 1 and 2 features in MMs and most MVs, and also uniquely revealed the cooccurrence of pathological variants sharing PrPSc type 2. In contrast, molecular typing best detected the concurrent PrPSc types in VV subjects and MV cases with kuru plaques. The present data provide an updated disease classification and are of importance for future epidemiologic and transmission studies aimed to identify etiology and extent of strain variation in sporadic Creutzfeldt-Jakob disease.

snip...

Discussion

Finally, the results obtained from the analyses of lesion profiles and clinical features in the subgroups of sCJD cases with mixed features deserve further comment. By showing that the relative ''load'' of each of the two PrPSc types significantly correlates with disease duration, the relative frequency of certain symptoms, and the ratio between cortical and cerebellar pathology, our study provides further strong evidence for the PrPSc type being a major biological determinant in human prion disease. In conclusion, the present data add to our knowledge of the prevalence and phenotypic spectrum of the sCJD variants with mixed molecular and pathological features, provide an updated molecular classification of the disease subtypes and will serve for future epidemiologic and transmission studies aimed at disclosing the etiology and extent of strain variation in sCJD.

Acknowledgments We wish to thank Barbara Polischi and Sabrina Boninsegna for her technical assistance. We also thank all the physicians who provided clinical data and helped in the collection of tissues and all family members who consented to the use of tissue for research. This study was funded in the frame of the bilateral Italy (ISS)-USA (NIH, Office for Rare Diseases) agreement on joint research on rare diseases, by the European Commission (FOOD-CT- 2004-506579), the Italian Ministry of University, Research and Technology (FIRB-2003-RBNE03FMCJ_006), the Federal Ministry of Health (ZV2-1369-340): grant PHS P30 AG010133, and the Gino Galletti Foundation.

Keywords Prion protein Brain mapping Molecular typing Neurodegeneration Classification

P. Parchi R. Strammiello S. Notari S. Capellari Dipartimento di Scienze Neurologiche, Universita` di Bologna, Bologna, Italy A. Giese H. Kretzschmar Institut fu¨r Neuropathologie, Ludwig-Maximilians-Universita¨t Mu¨nchen, Munich, Germany J. P. M. Langeveld Central Veterinary Institute of Wageningen UR, Lelystad, The Netherlands A. Ladogana M. Pocchiari Department of Cell Biology and Neurosciences, Istituto Superiore di Sanita`, Rome, Italy I. Zerr Department of Neurology, National Reference Center for TSE Surveillance, Georg-August University, Go¨ttingen, Germany F. Roncaroli Division of Neuroscience and Mental Health, Department of Clinical Neuroscience, Imperial College, London, UK P. Cras Born-Bunge Institute (BBI), University of Antwerp (UA), Antwerp, Belgium B. Ghetti Department of Pathology, Indiana University, Indianapolis, IN, USA P. Parchi (&) Department of Neurological Sciences, Universtity of Bologna, Via Foscolo 7, 40123 Bologna, Italy e-mail: piero.parchi@unibo.it


http://www.springerlink.com/content/21552482u6761291/fulltext.pdf



MANY, MANY THANKS TO Parchi et al for this study, AND for the public access to full text. ...TSS


Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH

Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.

Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.

Design: Retrospective analysis.

Setting: The Johns Hopkins and Veterans Administration health care systems.

Participants: Eighty-eight patients with definite or probable sCJD.

Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.

Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P .001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.

Arch Neurol. 2009;66(2):208-215

snip...

COMMENT

snip...see full text ;


http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html



Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009


http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



Monday, May 11, 2009

Rare BSE mutation raises concerns over risks to public health


http://bse-atypical.blogspot.com/2009/05/rare-bse-mutation-raises-concerns-over.html



JOURNAL OF NEUROLOGY

MARCH 26, 2003

Send Post-Publication Peer Review to journal:

Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535



THE PATHOLOGICAL PROTEIN

BY Philip Yam

Yam Philip Yam News Editor Scientific American www.sciam.com

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

CHAPTER 14

Laying Odds

Are prion diseases more prevalent than we thought?

Researchers and government officials badly underestimated the threat that mad cow disease posed when it first appeared in Britain. They didn't think bovine spongiform encephalopathy was a zoonosis-an animal disease that can sicken people. The 1996 news that BSE could infect humans with a new form of Creutzfeldt-Jakob disease stunned the world. It also got some biomedical researchers wondering whether sporadic CJD may really be a manifestation of a zoonotic sickness. Might it be caused by the ingestion of prions, as variant CJD is?

Revisiting Sporadic CJD

It's not hard to get Terry Singeltary going. "I have my conspiracy theories," admitted the 49-year-old Texan.1 Singeltary is probably the nation's most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union's Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow. org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people's health.

Singeltary has similar inclinations. ...

snip...

THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.


http://www.thepathologicalprotein.com/



http://books.google.com/books?id=ePbrQNFrHtoC&pg=PA223&lpg=PA223&dq=the+pathological+protein+laying+odds+It%E2%80%99s+not+hard+to+get+Terry+Singeltary+going&source=bl&ots=um0PFAZSZD&sig=JWaGR7M7-1WeAr2qAXq8D6J_jak&hl=en&ei=MhtjS8jMJM2ztgeFoa2iBg&sa=X&oi=book_result&ct=result&resnum=1&ved=0CAcQ6AEwAA#v=onepage&q=&f=false



http://www.springerlink.com/content/r2k2622661473336/fulltext.pdf?page=1



http://www.thepathologicalprotein.com/



http://prionunitusaupdate2008.blogspot.com/2009/04/national-prion-disease-pathology.html



Newsdesk
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003
doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

Tracking spongiform encephalopathies in North America

Xavier Bosch

"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem." 49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)-the relative of mad cow disease seen among deer and elk in the USA. Although his feverish.


http://linkinghub.elsevier.com/retrieve/pii/S1473309903007151



http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(03)00715-1/fulltext



http://www.mdconsult.com/das/article/body/180784492-2/jorg=journal&source=&sp=13979213&sid=0/N/368742/1.html?issn=14733099



Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT


http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT



http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT



DER SPIEGEL (9/2001) - 24.02.2001 (9397 Zeichen) USA: Loch in der Mauer Die BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehördensind lax.Link auf diesen Artikel im Archiv:


http://service.spiegel.de/digas/find?DID=18578755



"Löcher wie in einem Schweizer Käse" hat auch Terry Singeltary im Regelwerk der FDA ausgemacht. Der Texaner kam auf einem tragischen Umweg zu dem Thema: Nachdem seine Mutter 1997 binnen weniger Wochen an der Creutzfeldt-Jakob-Krankheit gestorben war, versuchte er, die Ursachen der Infektion aufzuspüren. Er klagte auf die Herausgabe von Regierungsdokumenten und arbeitete sich durch Fachliteratur; heute ist er überzeugt, dass seine Mutter durch die stetige Einnahme von angeblich kräftigenden Mitteln erkrankte, in denen - völlig legal - Anteile aus Rinderprodukten enthalten sind.

Von der Fachwelt wurde Singeltary lange als versponnener Außenseiter belächelt. Doch mittlerweile sorgen sich auch Experten, dass ausgerechnet diese verschreibungsfreien Wundercocktails zur Stärkung von Intelligenz, Immunsystem oder Libido von den Importbeschränkungen ausgenommen sind. Dabei enthalten die Pillen und Ampullen, die in Supermärkten verkauft werden, exotische Mixturen aus Rinderaugen; dazu Extrakte von Hypophyse oder Kälberföten, Prostata, Lymphknoten und gefriergetrocknetem Schweinemagen. In die USA hereingelassen werden auch Blut, Fett, Gelatine und Samen. Diese Stoffe tauchen noch immer in US-Produkten auf, inklusive Medizin und Kosmetika. Selbst in Impfstoffen waren möglicherweise gefährliche Rinderprodukte enthalten. Zwar fordert die FDA schon seit acht Jahren die US-Pharmaindustrie auf, keine Stoffe aus Ländern zu benutzen, in denen die Gefahr einer BSE-Infizierung besteht. Aber erst kürzlich verpflichteten sich fünf Unternehmen, darunter Branchenführer wie GlaxoSmithKline, Aventis und American Home Products, ihre Seren nur noch aus unverdächtigem Material herzustellen.

"Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA regulations. ...


http://www.spiegel.de/spiegel/print/d-18578755.html



http://wissen.spiegel.de/wissen/image/show.html?did=18578755&aref=image024/E0108/SCSP200100901440145.pdf&thumb=false



http://service.spiegel.de/digas/servlet/find/DID=18578755



Suspect symptoms

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

28 Mar 01

Like lambs to the slaughter 31 March 2001 by Debora MacKenzie Magazine issue 2284. Subscribe and get 4 free issues. FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.

Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.

Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.

As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.

"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.

But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.

People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.

But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."

There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.

Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.


http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html




2 January 2000

British Medical Journal

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well


http://www.bmj.com/cgi/eletters/320/7226/8/b#6117




15 November 1999

British Medical Journal

vCJD in the USA * BSE in U.S.


http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406




MAD COW FEED IN COMMERCE USA BY TONNAGE AND POUNDS TO 2010 (what i found only)

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

CODE

Cattle feed delivered between 01/12/2007 and 01/26/2007

RECALLING FIRM/MANUFACTURER

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

42,090 lbs.

DISTRIBUTION

WI

___________________________________

PRODUCT

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

REASON

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

9,997,976 lbs.

DISTRIBUTION

ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007


http://www.fda.gov/bbs/topics/enforce/2007/ENF00996.html



NEW URL


http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm



Tuesday, March 2, 2010

Animal Proteins Prohibited in Ruminant Feed/Adulterated/Misbranded Rangen Inc 2/11/10 USA


http://madcowfeed.blogspot.com/2010/03/animal-proteins-prohibited-in-ruminant.html



Monday, March 1, 2010

ANIMAL PROTEIN I.E. MAD COW FEED IN COMMERCE A REVIEW 2010


http://madcowfeed.blogspot.com/2010/03/animal-protien-ie-mad-cow-feed-in.html



Sunday, January 17, 2010

BSE USA feed inspection violations 01/01/2009 to 01/17/2010 FDA BSE/Ruminant Feed Inspections Firms Inventory Report


http://madcowfeed.blogspot.com/2010/01/bse-usa-feed-inspection-violations.html



Thursday, March 19, 2009

MILLIONS AND MILLIONS OF POUNDS OF MAD COW FEED IN COMMERCE USA WITH ONGOING 12 YEARS OF DENIAL


http://madcowfeed.blogspot.com/2009/03/millions-and-millions-of-pounds-of-mad.html



The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1

1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to:

John Collinge, E-mail: j.collinge@prion.ucl.ac.uk

Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002

--------------------------------------------------------------------------------

Abstract

Variant Creutzfeldt-Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

Keywords:BSE, Creutzfeldt-Jakob disease, prion, transgenic


http://www.nature.com/emboj/journal/v21/n23/abs/7594869a.html



Monday, May 19, 2008

SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS


http://bseinquiry.blogspot.com/



Sunday, August 10, 2008

A New Prionopathy OR more of the same old BSe and sporadic CJD


http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html



Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks

BMC Veterinary Research 2010, 6:17 doi:10.1186/1746-6148-6-17

http://www.biomedcentral.com/content/pdf/1746-6148-6-17.pdf



Sunday, March 28, 2010

Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks

http://nor-98.blogspot.com/2010/03/atypicalnor98-scrapie-in-basque-country.html



Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010

Greetings,

Unusual event if you consider the officials hypothisis that Nor-98 atypical scrapie is a spontaneous event. seems there was a great deal of spontaneous mutations for this time period ;-)...TSS

http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html



Thursday, March 11, 2010

CANADA TYPICAL AND ATYPICAL SCRAPIE REPORT TO MARCH 2010

http://nor-98.blogspot.com/2010/03/canada-typical-and-atypical-scrapie.html



TRADING TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY GLOBALLY

Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html



OIE Scrapie Chapter Revision • Current draft recognizes Nor98-like scrapie as a separate disease from classical scrapie • USDA provided comments on the draft to OIE

http://www.animalagriculture.org/Solutions/Proceedings/Annual%20Meeting/2009/Sheep%20&%20Goat/Myers,%20Thomas.pdf



Atypical scrapie/Nor 98 October 2009

Last year, after examining member country submissions and investigating rigorous scientific research, the World Organisation for Animal Health (OIE) decided that Nor 98 should not be listed in its Terrestrial Animal Health Code. The Code sets out trade recommendations or restrictions for listed diseases or conditions, and the OIE determined there was no need for such recommendations around Nor 98.

http://www.nzfsa.govt.nz/publications/ce-column/ce-web-nor98.htm



http://www.biosecurity.govt.nz/files/pests/atypical-scrapie/atypical-scrapie-faq-oct09.pdf



Sutton reported that USDA has urged the World Organization for Animal Health (OIE) to categorize Nor98-like scrapie as a separate disease from classical scrapie. Currently, the OIE has proposed a draft revision of their scrapie chapter that would exclude Nor98-like scrapie from the chapter. USDA will be submitting it's comments on this proposal soon.

http://www.ohiosheep.org/Events/ScrapieNewsletterMarch09.pdf



What is Nor98-Like (Nonclassical) Scrapie?

Nor98-like scrapie is a prion disease. The prion diseases include classical scrapie in sheep and goats, Bovine Spongiform Encephalopathy (BSE) primarily of cattle, chronic wasting disease (CWD) of deer and elk, and in humans Creutzfeldt-Jakob Disease (CJD) and variant Creutzfeldt-Jakob Disease (vCJD). What each of these diseases has in common is that they cause a progressive and ultimately fatal degeneration of the nervous system. While the underlying cause(s) are still debated, the theory most widely accepted in the scientific community is that the agent is a prion – an abnormal form of a normally occurring cellular protein.

Unlike BSE, classical scrapie and Nor98-like scrapie have not been shown to be a threat to human health. Classical scrapie has been known to exist for over 250 years, and cases have occurred in the United States since 1947. It is likely that Nor98-like scrapie has also existed for a long time; however, it was first identified in Norway in 1998, hence the name Nor98. This type of scrapie is referred to as “atypical scrapie”, “Nor98 scrapie”, “Nor98-like scrapie”, or “nonclassical scrapie” in the literature.

Since 1998, almost every country in Europe, as well as the Falkland Islands, New Zealand, Canada, and the United States have found similar cases. The first cases in the United States were identified in 2007.

Nor98-like (nonclassical) scrapie and classical scrapie are separate diseases with distinct features.

Transmission

Classical scrapie is an infectious disease that is transmitted to other sheep and goats under natural conditions.

Nor98-like scrapie is either not transmitted or is poorly transmitted, under natural conditions. Many scientists believe that Nor98-like scrapie is not an infectious disease under natural conditions and that it is instead caused by a random conversion of the normal prion protein into the abnormal form (often referred to as “sporadic”).

Distribution

Nor98-like scrapie has been found in all countries where extensive surveillance has been conducted using sensitive test methods; whereas, classical scrapie has not been reported in some of these countries.

Nor98-like scrapie cases are widely distributed and proportionate with sheep/goat populations; whereas, classical scrapie cases often occur in clusters.

Number of affected sheep or goats in a flock or herd

Classical scrapie usually infects more than one animal in an infected flock. In the US, approximately 18 percent of the mature, genetically susceptible sheep in an infected flock are infected.

Nor98-like scrapie is rarely found in more than one animal in a flock or herd. When an additional case is found, it is usually in flocks with more than 500 sheep.

Average age of onset of clinical signs in animals

With classical scrapie, clinical signs typically first appear and result in death in animals that are between 3-5 years of age.

In Nor98-like scrapie, clinical signs are rarely documented and the animals are typically diagnosed when they are sampled at slaughter, usually at greater than 5 years of age.

Variations in protection against disease conferred by genotype

Sheep with genotypes that are resistant to classical scrapie are susceptible to Nor98-like scrapie.

Clinical signs associated with disease

Unlike classical scrapie, clinical signs are rarely reported in Nor98-like scrapie cases.

In the few Nor98-like scrapie cases where clinical signs were reported, the signs observed were indistinguishable from those described for classical scrapie. These include incoordination, gait abnormalities, collapse while running, tremors, loss of condition, leg biting, nibble response and/or behavioral changes.

One potential clinical difference is that intense rubbing is a frequently occurring clinical sign in classical scrapie cases; whereas, it has not been reported in Nor98-like scrapie cases.

Laboratory findings readily distinguish Nor98-like scrapie from classical scrapie.

In 2009, the World Animal Health Organization (OIE) recognized Nor98-like scrapie as a separate disease from classical scrapie because of differences in laboratory findings, transmissibility, and distribution. This determination means that Nor98-like scrapie is not a reportable disease to OIE, and should be of no trade concern.

How has APHIS’ policy changed regarding Nor98-like scrapie?

APHIS will no longer require the depopulation or movement restriction of Nor98-like scrapie exposed sheep and goats. APHIS will propose changes to the Code of Federal Regulations (CFR) in 2010 to allow the APHIS Administrator to eliminate or reduce post exposure requirements for certain scrapie types (or certain/specific types of scrapie)-- such as Nor98-like scrapie -- that are determined to pose minimal risk of lateral transmission under natural conditions.

In the meantime, APHIS is conducting a national scrapie control pilot project for Nor98-like scrapie. The project is for flocks and herds in which animals positive to Nor98-like scrapie were born, lambed, or kidded.

What will the pilot project do for producers?

The pilot project will allow producers that have sheep and goats exposed to Nor98-like (nonclassical) scrapie to retain, sell, exhibit or move them for any purpose.

What will happen if Nor98-like scrapie case is found on or traced back to an owner’s flock or herd?

The owner will be contacted by a Federal or State veterinarian who will schedule a visit to the farm with the owner. The activities listed below will be performed.

Confirm the identification of the positive animal.

Provide the owner with information about scrapie and its control.

Determine if the positive animal was born in or gave birth in the flock/herd. If so, the veterinarian will:

Work with state animal health authority to ensure animals are not moved from the premises until they have been officially identified;

Develop a Nor98-like scrapie flock plan and a 5 year monitoring plan with the producer;

If not already officially identified, apply official eartags to sheep and goats exposed to Nor98-like scrapie; and

Inventory all sheep and goats, and any sheep or goat embryos.

The officially identified sheep and goats will then be classified and handled as low-risk exposed animals, allowing the owner to move the animals from the premises for any purpose including sale.

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/nor98-like_information.pdf



October 21, 2007

II.A. USAHA/AAVLD President ’s Reception and Dinner Joint President’s Award Lee Myers and Barbara Powers Dr. Powers and I would like to present a Joint Presidents’ Award this evening to a special individual that has been of great benefit and support to both the AAVLD and USAHA. This evening, it is our pleasure to present a Joint Presidents’ Award to Dr. Ron DeHaven. Dr. DeHaven has more than two decades of experience with the Animal Plant Health Inspection Service (APHIS) and gained national prominence in 2003 and 2004 when chronic wasting disease and bovine spongiform encephalopathy were making headlines. Dr. DeHaven became the public face of the nation’s response to BSE following the discovery of the disease at just before Christmas in 2003 in the United States.

SNIP...

The National Animal Health Laboratory Network and NAIS are just two ways we’re using science and technology to protect animal health. On a global scale, we believe in international accepted science-based standards for animal health. We think it is appropriate to establish worldwide trade based on these standards. We’ve made a commitment to work toward that goal, and we expect our trading partners to do the same. We took a major step forward in May, when the OIE formally classified the United States as a controlled risk country for BSE. I’ll continue to press our trading partners to align their own import regulations with OIE guidelines, and give us full market access for our live cattle, beef and beef products, regardless of age.

SNIP...

Dr. DeHaven has often represented the United States in delicate and often difficult trade negotiations. As the former U.S. Chief Veterinary Officer and U.S. delegate to the OIE, he routinely used his diplomatic skills as he facilitated agreements that are science-based. He was instrumental in building consensus that led to the current OIE BSE chapter

SNIP...

http://www.usaha.org/meetings/2007/2007_USAHA_Proceedings.pdf



GOC RELEASES CONSULTATION DOCUMENT ON NEW BSE IMPORT POLICY IN LINE WITH OIE:

The Canadian Food Inspection Agency (CFIA) is inviting public comment on a proposed new Canadian Import Policy to prevent bovine spongiform encephalopathy (BSE) in bovine animals and their products. The proposed policy would bring Canada’s approach in line with new World Organization for Animal Health (OIE) standards as well as the proposed North American import standard announced on March 29, 2005. It is based on the recognition that international knowledge of bovine spongiform encephalopathy (BSE) and me asures to mitigate its transmission have advanced significantly since Canada’s existing import policy for controlling BSE was established in 1997. The new policy would be less restrictive than the current one. Canada’s current policy permits the importation of live ruminants including, cattle, sheep and goats, and products derived from them, only after the exporting country has been officially recognized as BSE-free. Current science recognizes that the “ BSE-free” requirement is unnecessarily restrictive. The draft policy is based on a proposed new OIE three-tier system for classifying bovine-trading countries based on their BSE risk management regimes. In all cases, exporting countries would also have to continue to meet other non-BSE food safety and animal health

GAIN Report - CA5038 Page 3 of 4

UNCLASSIFIED USDA Foreign Agricultural Service requirements before becoming eligible to ship to Canada under any of the new BSE risk categories. A consultation period ending on July 22, 2005 is being provided to allow interested parties the opportunity to provide comments on the draft policy. Notice of this consultation is being published in the Canada Gazette.

http://www.fas.usda.gov/gainfiles/200505/146129759.pdf




“The U.S. has lower sanitary and phyto-sanitary standards (SPS) for imports than many other countries, especially those concerning bovine spongiform encephalopathy (BSE). These low standards have made the U.S. a dumping ground for beef from the countries that have experienced BSE problems. Food Safety and SPS issues continue to be problematic for our industry, as some countries comply with OIE standards, while others ignore them either for cultural reasons, or too often use them as trade barriers. The USITC October 7, 2008 release reported, ‘U.S. beef processors and beef cattle ranchers lose billions of dollars in export opportunities each year because of animal health and food safety measures in other countries that are inconsistent with international standards and vary by country.

http://www.cattlenetwork.com/USCA-Testifies--Before-USITC/2010-03-03/Article_Latest_News.aspx?oid=996238&fid=CN-LATEST_NEWS_




LIKE i said before, the OIE not only sold their soul to the devil over the BSE MRR, they sold yours too ;


Wednesday, February 10, 2010

NAIS MAD COW TRACEABILITY DUMPED BY USDA APHIS 2010

http://naiscoolyes.blogspot.com/2010/02/nais-mad-cow-traceability-dumped-by.html



The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.

http://www.oie.int/boutique/extrait/06heim937950.pdf



Updated :08-Dec-2003

OIE Expert Ad Hoc group reviews 'atypical' BSE cases reported by Japan and Italy

An Ad hoc group of world renowned experts, from the OIE Reference Laboratories in the UK, Switzerland and Japan as well as experts from Italy was convened at OIE headquarters on December 4, 2003 to review available data on recently notified atypical BSE cases from Japan and Italy. The experts concluded that they did not believe that the data from these respective countries identified a link between the Japanese and the Italian cases. While acknowledging that the observations reported had not previously been described, further investigations already planned or in progress should clarify their significance. The experts also stressed that even if the data did represent the existence of alternative strains of BSE, this did not necessarily mean that they were new.

The most important relevance of the results of this meeting is that the experts did not believe that the available evidence justified any changes in the current surveillance methods, disease control, or in measure taken to protect human health. Therefore, there was no basis for suggesting that the risk to animals or humans had changed, and that there was no case for the moment for changes to international trade rules.

The experts recommended that existing links between the various OIE Reference Laboratories should be strengthened for a uniformed application of diagnostic methodologies as well as sharing scientific knowledge around the world, and that they will continue to jointly advance the current work on case definition of BSE.

In concluding, they recommended that national laboratories should consult with OIE Reference Laboratories before publishing significant findings that could influence case definition of BSE, or which had potential implications with regard to the protection of human and animal health, or international trade.

These recommendations were immediately endorsed by the OIE' Scientific and Terrestrial Animal Health Code Commissions, which were meeting simultaneously.

December 2003


http://www.oie.int/ENG/press/en_031208.htm



http://www.rr-asia.oie.int/representation/programmes/programme_f/workshops_011.html




WHY WAS THIS DELETED ?


Article 3.8.5.6. Epidemiological situation concerning all animal TSE in the country or zone Assumptions:

- BSE may have originated from scrapie of sheep. Countries with scrapie may be at greater risk than those which have demonstrated scrapie freedom.

- Theoretically, scrapie in small ruminants might mask the presence of BSE and no field methods are available to differentiate between different TSEs.

- Available evidence suggests there is no link between chronic wasting disease of cervids and BSE.

- It has been suggested that transmissible mink encephalopathy may be an indicator of a hitherto undefined and hypothetical TSE of cattle.

- If a hypothetical ‘spontaneous’ TSE of cattle is assumed to occur, it must also be assumed to occur in all countries at a similar rate.

Question to be answered:

Have other animal TSEs been identified in the country? What surveillance is there for TSEs?

Rationale: Surveillance programmes generate a picture of the epidemiological situation of animal TSE. The greater the surveillance effort, the greater the power of the information. Adequately targeted surveillance for BSE, such as described in Appendix 3.8.4., provides more powerful information than generic animal disease surveillance.

Evidence required:

Documentation on awareness and surveillance programmes targeting all TSEs of livestock, their legal basis, scale, duration, and data generated.

see full text ;


http://ec.europa.eu/food/international/organisations/eu_written_comments_terrestrial_code_may08.pdf




position: Post Doctoral Fellow Atypical BSE in Cattle

Closing date: December 24, 2009

Anticipated start date: January/February 2010

Employer: Canadian and OIE Reference Laboratories for BSE CFIA Lethbridge Laboratory, Lethbridge/Alberta

The Canadian and OIE reference laboratories for BSE are extensively involved in prion diseases diagnosis and research. With a recent increase in research activities and funding, the laboratory is looking to fill two post doctoral fellow positions. Both positions will be located at the Canadian Food Inspection Agency (CFIA) Lethbridge Laboratory which offers biosaftey level 3 (BSL3) and BSL2 laboratory space and is well equipped for molecular and morphologic prion research. The facility also has a BSL3 large animal housing wing and a state of the art post mortem room certified for prion work. Successful candidates will have the opportunity to visit other laboratories to cooperate in various aspects of the projects and to be trained in new techniques and acquire new skills. With a recent increase in prion disease expertise and research in Alberta and Canada, these positions will offer significant exposure to cutting edge prion science via videoconferencing, meetings, workshops and conferences. These interactions will also provide a valuable opportunity to present research findings and discuss potential future work opportunities and collaborations with other Canadian and international research groups.

Atypical BSE in Cattle

BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD.

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

Responsibilities include:

Driving research at the National and OIE BSE reference lab to ensure project milestones are met successfully. Contributing to the preparation of project progress reports. Directing technical staff working on the project. Communicating and discussing results, progress and future direction with project principle investigator(s). Communicating with collaborative project partners. Qualifications:

Successful completion of a PhD degree in an area focusing on or related to prion diseases. Extensive experience with molecular and/or morphologic techniques used in studying prion diseases and/or other protein misfolding disorders. Ability to think independently and contribute new ideas. Excellent written and oral communication skills. Ability to multitask, prioritize, and meet challenges in a timely manner. Proficiency with Microsoft Office, especially Word, PowerPoint and Excel. How to apply:

Please send your application and/or inquiry to: Dr. Stefanie Czub, DVM, Ph.D. Head, National and OIE BSE Reference Laboratory Canadian Food Inspection Agency Lethbridge Laboratory P.O. Box 640, Township Road 9-1 Lethbridge, AB, T1J 3Z4 Canada

phone: +1-403-382-5500 +1-403-382-5500 ext. 5549 email: stefanie.czub@inspection.gc.ca

Contact Info:

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2



P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS

Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA

Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C. The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.

III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)

http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf



Monday, November 23, 2009

BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.

http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html



IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,

http://www.oie.int/eng/Session2007/RF2006.pdf



Docket APHIS-2006-0026 Docket Title Bovine Spongiform Encephalopathy; Animal Identification and Importation of Commodities Docket Type Rulemaking Document APHIS-2006-0026-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Public Submission APHIS-2006-0026-0012 Public Submission Title Comment from Terry S Singletary


http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801e47e1



Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028 Public Submission Title Comment from Terry S Singletary

Comment 2006-2007 USA AND OIE POISONING GLOBE WITH BSE MRR POLICY

THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted products from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone. These are the facts as I have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species.

MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???

go figure. ...


http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f8151



Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028.1 Public Submission Title Attachment to Singletary comment

January 28, 2007

Greetings APHIS,

I would kindly like to submit the following to ;

BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01


http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f8152&disposition=attachment&contentType=msw8



THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.

...snip

IT'S as obvious as day and night, either Larry, Curley, and Mo have been at the helm of the USDA/APHIS/FSIS/FDA/CDC/NIH et al for many many years, or the incompetence of these agencies are so inept, either through ignorance and or just too overweight with industry reps., they then should be all done away with and a single agency brought forth, and if not, how will you correct this ongoing problem ?

Thank you, I am sincerely disgusted,

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

...snip

full text ;

Sunday, February 14, 2010

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)


http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html



PLEASE SEE FULL TEXT 98 PAGES HERE ;


http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf



HARVARD RESPONSE

Response to Public Comments on the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005

..... RESPONSE TO COMMENTS FROM TERRY S. SINGELTARY SR. ...

HARVARD RESPONSE

Response to Public Comments on the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005

..... RESPONSE TO COMMENTS FROM TERRY S. SINGELTARY SR. ...

RESPONSE TO COMMENTS FROM TERRY S. SINGELTARY SR.

Comment #1: SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientists, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues?

Response: The original (October 2003) and the revised (October 2005) Harvard BSE risk assessments underwent external peer review. Subsequently, revisions were made to the analysis. In the most recent review, the most significant revisions have been: 1) the addition of explicit modeling of the poultry litter pathway for the potential recycling of bovine protein into cattle feed; and 2) a decrease in the assumed effectiveness of ante mortem inspection in the identification of animals with BSE.

Comment #2: WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few months that consisted of some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May, IT should seem prudent to ask why our feed bans continue to fail in 2006, and continue to fail today?

Response: This question about feed bans is a matter for policy. As such, it is not addressed in this response.

Comment #3: WHY still now only partial ruminant feed ban, with the fact that now we seem to have 3 cases of nvCJD to humans i.e. human bovineTSE that were responsible from blood, and the fact the last 2 mad cows documented in the USA were that of an Atypical strain, would it not seem prudent to remove blood as well from ruminant feed? WOULD it not seem prudent to improve and expand the SRM list now? as per your own thinking; If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.

Response: This comment pertains to policy. As such, it is not addressed here.

Tuesday, March 2, 2010

Animal Proteins Prohibited in Ruminant Feed/Adulterated/Misbranded Rangen Inc 2/11/10 USA


http://madcowfeed.blogspot.com/2010/03/animal-proteins-prohibited-in-ruminant.html



Comment #4: WHAT does USDA/FDA ET AL intend to do about the risks of atypical BSE/TSE in cattle now that infectivity shows in tissue samples other than CNS in Japan, the fact now that the last Texas mad cow and that last mad cow in Alabama were indeed of the atypical strain, the fact that the studies long ago in Mission, Texas of USA sheep scrapie transmission to the USA bovine, which proved an 'atypical tse' in the USA bovine, the fact also that USDA/FDA are still floundering on the last SRM regulations, but with the BASE strain now in cattle that is not similar to nvCJD, but very similar to the sporadic CJD, and sporadic CJD has tripled in the last few years in the USA. WHAT do you plan to do to protect human health from these atypical strains of TSE, in relations to SRMs ?

- 19 -

Response: The BSE risk assessment simulation model characterizes the disease history of BSE, including the agent's spread within the body of the animal over time. It also quantifies the agent's persistence during the feed manufacture process, and ultimately the agent's ability to cause disease in other exposed animals. There is no definitive evidence that these properties differ substantially for atypical BSE strains, compared to the typical BSE agent.

Comment #5: THE 2004 Enhanced BSE surveillance program, that tested all those cows, but then we found just how terribly flawed the program was, from testing protocols, to testing the most likely to have BSE i.e. high risk, to the geographical distribution of the testing and high risk areas, to letting the tissue samples of one mad cow sit on a shelf for 7+ months and then having to have an act of Congress to ever get that cow finally confirmed, to that other Texas mad cow they decided to not even bother testing at all, just rendered that very suspect cow, too suspect to test evidently, back to that Alabama mad cow that they could only give a guess as to age with dentition where we all know that the age of that cow was so close to 10 years it could have been 9 years 7 months to 10 years 3 months, thus possibly being an BAPB i.e. USA 'born after partial ban', to all those rabies suspect cows that did not have rabies, and DID NOT get tested for BSE/TSE in that June 2004 enhanced surveillance program, even though the common lay person knows the suspect rabies negative cows are suppose to be BSE/TSE tested, how does one correct all these blatant failures and will they be corrected?

Response: This comment pertains to policy. As such, it is not addressed here.

Comment #6: WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported sheep from Belgium that were confiscated and slaughtered from the Faillace's, what sort of TSE did these animals have?

Response: It is not clear how the test results referred to in this comment are relevant to the Harvard BSE Risk Assessment Update. Sheep were not considered in the risk assessment.

Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010


http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html



Comment #7: WHY is it that the Farm of the Mad Sheep of Mad River Valley were quarantined for 5 years, but none of these farms from Texas and Alabama with Atypical TSE in the Bovine, they have not been quarantined for 5 years, why not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

Response: This comment pertains to policy. As such, it is not addressed here.

- 20 -...snip...end...TSS


http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf



Monday, October 26, 2009

MAD COW DISEASE, AND U.S. BEEF TRADE

MAD COW DISEASE, CJD, TSE, SOUND SCIENCE, COMMERCE, AND SELLING YOUR SOUL TO THE DEVIL


http://usdameatexport.blogspot.com/2009/10/mad-cow-disease-and-us-beef-trade.html



Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types

hmmm, this is getting interesting now...

Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,

see also ;

All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

http://cjdusa.blogspot.com/2009/09/co-existence-of-scrapie-prion-protein.html



see full text ;

Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types


http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html




Sunday, March 28, 2010

SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION

Numero de focos de Encefalopatia Espongiforme Bovine en Espana 2009 - 1010


http://creutzfeldt-jakob-disease.blogspot.com/2010/03/spain-bse-nor-98-atypical-scrapie.html



Wednesday, February 24, 2010

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

http://transmissiblespongiformencephalopathy.blogspot.com/



SCRAPIE, ATYPICAL, OVINE - AUSTRALIA: (WESTERN AUSTRALIA) SUSPECTED

*******************************************************************

A ProMED-mail post ProMED-mail is a program of the International Society for Infectious Diseases

[1] Date: Fri 12 Mar 2010

Source: The Australian [edited]



A West Australian sheep has been found to have signs characteristic of the fatal brain disease atypical scrapie. It comes as Australia faces growing anger from its trade partners over the Rudd government's surprise decision to extend a ban on the importation of beef from countries exposed to mad cow disease for a further 2 years.

Australia's chief veterinarian, Andy Carroll, told the ABC an indicative case of the atypical scrapie had been confirmed but said it posed no risk to human or animal health or the safety of eating meat and animal products.

Nor does atypical scrapie carry the dire trade consequences associated with classical scrapie.

Classical scrapie is in the same transmissible spongiform encephalopathies (TSE) family as BSE, better known as mad cow disease, from which humans can be fatally infected.

Dr Carroll said samples from the sheep's brain were being sent to the World Reference Laboratory in Britain.

Neither atypical scrapie nor classical scrapie has been seen in Australia before, but a sheep in New Zealand tested positive to the atypical form last year [2009].

Atypical scrapie is a relatively recently discovered disease and the common scientific view is that it occurs spontaneously or naturally in very small numbers of older sheep in countries all over the world.

[Byline: Jodie Minus]

-- Communicated by: Sabine Zentis Castleview Pedigree English Longhorns Gut Laach 52385 Nideggen Germany

****** [2] Date: Wed 10 Mar 2010 Source: ABC News (Australian Broadcasting Corporation) [edited]

Animal health authorities are testing a sheep's brain for what could be Australia's 1st case of the disease atypical scrapie.

Although not confirmed, the sheep is thought to be from Western Australia.

This type of scrapie is described as a sporadic degenerative brain condition affecting older sheep, and is not contagious.

Ed Klim, from national advisory group SafeMeat, says a 2nd round of testing is now taking place. "We've been made aware that the Australian Animal Health Laboratory is conducting further routine testing on a sheep sample," he says.

"The disease isn't considered a health risk nor should have any impact on food safety or export markets for sheep meat of live sheep."

Australia's chief veterinarian and WA's Department of Agriculture of Food are both aware of the testing but will not comment.

-- Communicated by: Terry S Singeltary Sr

[Although atypical scrapie is not yet ruled out, it is important to realize this is a type of scrapie that thus far has only tended to appear as a sporadic condition in older animals. Currently it has not been shown to follow the same genetic tendencies for propagation as the usual scrapie.

However, the atypical phenotypic appearance has been shown to be preserved on experimental passage.

Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries, as Australia may join that list. It is likely that this case will be sent to the UK for definitive conformation.

[Ref: M Simmons, T Konold, L Thurston, et al. BMC Veterinary Research 2010, 6:14 [provisional abstract available at ]

"Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]

"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."

Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG]

The HealthMap/ProMED-mail interactive map of Australia is available at . - Sr.Tech.Ed.MJ]

[see also: 2009 ---- Scrapie, atypical, ovine - New Zealand (02) 20091029.3740 Scrapie, atypical, ovine - New Zealand 20090220.0714 2007 ---- Scrapie, atypical, sheep - USA (WY): 1st report 20070318.0949 2005 ---- Scrapie, atypical, ovine - Falkland Islands 20051120.3371 2004 ---- Scrapie, atypical, sheep - UK and Ireland 20041210.3274 Scrapie, atypical, sheep - UK (02) 20040409.0965 Scrapie, atypical, sheep - UK 20040408.0952 Scrapie, atypical, sheep - France: OIE 20040201.0390] ...................................sb/tg/mj/lm

*#################################################



http://www.promedmail.org/pls/apex/f?p=2400:1001:3033292671016132::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,81729



I personally think that the timing of this _suspect_ atypical scrapie case in Australia should be regarded with great suspicion.

Kind of like the declaration of extra ordinary of emergency of atypical TSE of foreign origin in the USA that was declared some decade ago, and a farm hijacked by the USDA, with all animals slaughtered, and it was a farce from the beginning. those damn sheep never had nothing. not even typical scrapie. so, don't be fooled, as with Canada now, and them taking up the USDA habit of hiding mad cows, our Governments are capable of both, hiding mad cows, or only testing healthy cows for BSE, or claiming an animal has a particular disease, but knowing it never did, only for political purposes those sheep were slaughtered i.e. the mad sheep of mad river valley, that farm was raided, all animals slaughtered, and a make believe TSE was reported (had to be atypical scrapie or BSE), just because those sheep were allowed into this country by mistake, they should never have been let in. The farmers did nothing wrong, it was the USDA, and then they pulled _false flag_ type intervention. my government never ceases to amaze me. ...


Monday, September 1, 2008

RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [No. 00-072-1] September 1, 2008


http://foiamadsheepmadrivervalley.blogspot.com/2008/09/re-foia-of-declaration-of-extraordinary.html




Saturday, February 27, 2010

FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27, 2010


http://foiamadsheepmadrivervalley.blogspot.com/2010/02/final-report-of-testing-of-belgian.html




AUSTRALIA

COMMONWEALTH OF AUSTRALIA

Proof Committee Hansard

SENATE RURAL AND REGIONAL AFFAIRS AND TRANSPORT REFERENCES COMMITTEE Reference: Import restrictions on beef

FRIDAY, 5 FEBRUARY 2010 CANBERRA CONDITIONS OF DISTRIBUTION

This is an uncorrected proof of evidence taken before the committee. It is made available under the condition that it is recognised as such. BY AUTHORITY OF THE SENATE [PROOF COPY] TO EXPEDITE DELIVERY, THIS TRANSCRIPT HAS NOT BEEN SUBEDITED

SNIP...

Friday, 5 February 2010 Senate RRA&T 1

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

Committee met at 9.01 am

CHAIR (Senator Nash)—I declare open this public hearing of the Rural and Regional Affairs and Transport References Committee. The committee is hearing evidence on the committee’s inquiry into the impact and consequences of the government’s decision to relax import restrictions on beef. Before the committee starts taking evidence I remind all witnesses that, in giving evidence to the committee, they are protected by parliamentary privilege. It is unlawful for anyone to threaten or disadvantage a witness on account of evidence given to a committee and such action may be treated by the Senate as a contempt. It is also a contempt to give false or misleading evidence to a committee. The committee prefers all evidence to be given in public but, under the Senate’s resolutions, witnesses have the right to request to be heard in private session. It is important that witnesses give the committee notice if they intend to ask to give evidence in camera. If a witness objects to answering a question, the witness should state the ground upon which the objection is taken and the committee will determine whether it will insist on an answer, having regard to the ground which is claimed. If the committee determines to insist on an answer, a witness may request that the answer be given in camera. Such a request may, of course, also been made at any other time. On behalf of the committee, I thank all those who have made submissions and sent representatives here today for their cooperation in this inquiry.

RRA&T 2 Senate Friday, 5 February 2010

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

[9.03 am]

BELLINGER, Mr Brad, Chairman, Australian Beef Association

CARTER, Mr John Edward, Director, Australian Beef Association

CHAIR—Welcome. Would you like to make an opening statement?

Mr Bellinger—Thank you. The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He states, and rightfully so:

You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heinemann variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. He goes on to state:

The OIE— the International Organisation of Epizootics, the arm of the WTO— is a failed global agent that in my opinion is bought off via bogus regulations for global trade and industry reps. I have done this all these years for nothing but the truth. I am a consumer, I eat meat, but I do not have to sit idly by and see the ignorance and greed of it all while countless numbers of humans and animals are being exposed to the TSE agents. All the USA is interested in is trade, nothing else matters.

Even Dr Stanley Prusiner, who incidentally won the Nobel Health Prize in 1997 for his work on the prion—he invented the word ‘prion’, or it came from him—states:

The BSC policy was set up for one purpose only, trade—the illegal trading of all strains of TSE globally throughout North America, which is home to CBSC, IBSC and HBSC, many scrapie strains and two strains of CJD to date. (please note typo error, those should have read cBSE, lBSE, and hBSE...tss)

I would also like, while I have the opportunity, to explain the beef-off-the-shelves myth. At the first Senate hearing on 14 December, it was explained that the reason why they allowed BSC beef into Australia was the beef-off-the-shelves policy, whereby if we found a case of BSC in Australia they would have to recall all—

Friday, 5 February 2010 Senate RRA&T 3

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

Senator HEFFERNAN—Which of course is total BS.

Mr Bellinger—Correct. This is written in the FSANZ document—Food Standards Australia New Zealand. Why isn’t this same policy in New Zealand? It is not—it is only in Australia. We are the only country in the world to have this idiotic policy. So we again call for the tabling of the WTO obligations paperwork. We do not believe that exists.

Mr Carter—We have an additional concern about human health. We are not scientists, but on 18 December, four days after the last hearing here, the BBC reported a new wave of deaths due to variant CJD linked to eating BSE infected beef could be underway. This is based on the work of Professor John Collinge of the National Prion Clinic, who reported that a 2009 death in Scotland was from a different genetic pool to that of the 166 deaths already reported in the UK. Those are all thought to share one gene, but Professor Collinge and his colleagues estimate that up to 350 people in this new group, represented by the person who died in Scotland, could get CJD. He thinks that CJD has moved into a new phase, and the incubation period is a long one. We tender the Australian Red Cross donor policy sheet, which bears out what Senator Back brought up last time, questioning the Chief Medical Officer, and we say that blood from people who were in the UK between 1980 and 1996 is not acceptable. That is the current ruling. We believe this now should be extended to anyone who has visited the UK, and this new evidence should ensure that Australia revisits the science of CJD.

CHAIR—Thank you, Mr Carter. Before we kick off, can I just remind colleagues that we are short of time today, so I ask that we do not traverse ground the we have previously covered and make sure that we stick to new information that is required. Mr Bellinger, when you started you referred to your view that this decision to allow the importation was politically based. I know you are going to go into this in the course of the next 20 minutes or so, but could you just give us a quick outline of what your definition of politically based is and why you think the decision was politically based?

Mr Bellinger—On the lowering of BSE standards: if you go back to 2006, for example, there were five categories for describing countries that had BSE and Australia was in the category for BSE free. Suddenly, by the time the United States got their third instance of BSE, through the influence of Robert Zoellick—who was the trade minister that signed the BSE corresponding side letter in 2004 and was George Bush’s appointment to the WTO—they suddenly changed the five categories to three categories and, instead of being BSE free, Australia became BSE negligible risk. At the time I put out a press release alerting the media to the dangers of this happening, and we are coming to the stage here when suddenly our government is saying, ‘Now let’s allow the importation of beef from BSE affected countries.’ I believe that the WTO has been influenced by large multinational meat processors and retailers to change and allow the trading of BSE beef throughout the world.

CHAIR—Thanks, Mr Bellinger.

Mr Carter—Of course, the side letter that Minister Vaile signed was at the request of Mr Zoellick, who is now in the position that Mr Bellinger has explained.

Senator HEFFERNAN—I just want to put the committee on notice that, if we do not get through what we have got to get through today, I suggest we have another hearing, because this

RRA&T 4 Senate Friday, 5 February 2010

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

is the greatest ambush of Australia’s farmers of all time by a government. The evidence given at the last meeting was deadset lies. The proposition that this whole change of government policy was led by the industry is a deadset lie. While Simon Crean might want to change his mind because of the WTO and his lack of knowledge, the Australian beef industry, as you know, is under great challenge, not only from the currency but also from the undermining of our markets. This is a disgrace.

SNIP...PLEASE SEE FULL TEXT ;

*************

Tuesday, March 16, 2010

COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef FRIDAY, 5 FEBRUARY 2010 AUSTRALIA

COMMONWEALTH OF AUSTRALIA

Proof Committee Hansard

snip...see full text 110 pages ;



http://www.aph.gov.au/hansard/senate/commttee/S12742.pdf



for those interested, please see much more here ;



http://docket-aphis-2006-0041.blogspot.com/2010/03/commonwealth-of-australia-hansard.html




Thursday, March 18, 2010

The natural atypical scrapie phenotype is preserved on experimental transmission and sub-passage in PRNP homologous sheep


http://nor-98.blogspot.com/2010/03/natural-atypical-scrapie-phenotype-is.html



Friday, April 18, 2008

Statement by Agriculture Secretary Ed Schafer on South Korea Reopening Market to U.S. Beef and Beef Products in Line with International Standards


http://usdameatexport.blogspot.com/2008/04/statement-by-agriculture-secretary-ed.html



Friday, May 9, 2008

USDA VS KOREA typical or atypical BSe Honorable people of Korea


http://usdavskorea.blogspot.com/2008/05/usda-vs-korea-typical-or-atypical-bse.html



Joint Fact-Finding Mission to South Korea Final Report Seoul, South Korea July 21–24, 2008


http://material.ahrchk.net/docs/AHRC-SPR-006-2008-SouthKorea.pdf



http://minbyun.org/?module=file&act=procFileDownload&file_srl=27364&sid=0b0c8d8b3089eef17f3ae37d0d8538d2




However, i can tell you that it was the media and the womans doctor here in the USA that first reported she had human form of mad cow disease. and that's what PD NOTEBOOK and many other media outlets published, until the USDA (?) which first announced that it was not nvCJD. ...


Portsmouth woman did not die of mad cow-related condition, ___USDA___ says


By Nancy Young The Virginian-Pilot © May 7, 2008

Preliminary test results indicate that a 22-year-old Portsmouth woman who died in April did not have an illness that has been associated with eating contaminated beef, a U.S. Department of Agriculture official said this week.

"The epidemiologic characteristics of the illness and preliminary results of the neuropathologic testing of brain tissue obtained at autopsy indicate that the patient did not die of" a variant of Creutzfeldt-Jakob Disease (vCJD), which has been associated with mad cow disease, said Richard Raymond, USDA undersecretary for food safety. He was speaking Sunday to a group of Korean and American reporters in Washington.

"The U.S. Centers for Disease Control and Prevention has just provided us with that information, and I felt it was important to share with you today," he told the group. The comment appears on a statement on the safety of the U.S. beef supply that is available on the USDA's Food Safety and Inspection Service Web site.

Aretha Vinson died April 9 at Bon Secours Maryview Medical Center in Portsmouth. She had a degenerative brain condition that can be caused by a wide variety of things, and concerns were raised that she might have had vCJD. The state health department, CDC and the University of Virginia were involved in testing to determine the cause of death and whether it indicated a public health concern.

"There's no threat to the general public," said Michelle Peregoy, a Virginia Department of Health spokeswoman. She would not say more, citing patient confidentiality issues.

Nancy Young, (757) 446-2947, nancy.young@pilotonline.com


http://hamptonroads.com/2008/05/portsmouth-woman-did-not-die-mad-cowrelated-condition-usda-says



Virginia State University student Aretha Vinson has contracted a rare brain disorder called Creutzfeldt-Jakob Disease, a variant of Mad Cow Disease. Virginia state health officials are investigating how Vinson contracted the disease. Doctors suspect Vinson contracted the disease after having gastric bypass surgery, possibly from tainted medical instruments. Vinson's family says her health worsened after having gastric bypass surgery three months prior to the diagnosis.

UPDATE: Vinson passed away at 5:30pm on Wednesday, April 9, 2008.


http://www.mahalo.com/stub/aretha-vinson



The program, which now faces charges for playing up the possibility that the woman died of vCJD, said, “The CDC last Thursday announced the cause of death of Aretha Vinson, who died of symptoms similar to vCJD.” It quoted the CDC as saying although the suspected case received international media attention, the National Prion Disease Pathology Surveillance Center determined that the cause of death was not due to vCJD, a finding, it pointed out, that was similar to an announcement by the Department of Agriculture.


http://english.chosun.com/w21data/html/news/200806/200806170006.html



22 year old sporadic CJD ???

we will never know ;

In a telephone interview with the Chosun Ilbo, CDC spokesman Dave Daigle on Monday said the centers posted the announcement after performing their own checkup once the NPDPSC finished its investigation. He added that because the CDC only provide information on diseases, they have no plans to make a separate press release on the issue including the result of the investigation. ...


http://english.chosun.com/w21data/html/news/200806/200806170006.html



Now, the cdc et al usually hide behind patient confidentiality to hide cjd cases. but in this 22 years old, confidentiality was not the case, she was well known around the world, and the cdc et al chose to hide her final diagnosis. why, because it was another young victim in the USA with sporadic CJD ???

SEE FULL TEXT ;

Tuesday, June 17, 2008

Portsmouth woman did not die of mad cow-related condition, USDA says

UPDATE Updated Jun.17, 2008 08:34 KST


http://cjdmadcowbaseoct2007.blogspot.com/2008/06/portsmouth-woman-did-not-die-of-mad-cow.html




Wednesday, January 13, 2010 High Court Rules In Favor of PD Notebook 01-13-2010 21:21

High Court Rules In Favor of PD Notebook



snip...see full text ;


http://usdavskorea.blogspot.com/2010/01/high-court-rules-in-favor-of-pd.html




Monday, November 30, 2009

Taiwan, USDA, and USA beef, what the consumer does not know, could kill them The beef referendum is necessary


http://usdavskorea.blogspot.com/2009/11/taiwan-usda-and-usa-beef-what-consumer.html



Tuesday, December 29, 2009

Taiwan to resume USA beef ban over mad cow disease threat Dec 29, 2009 Taiwan to resume beef ban


http://usdavskorea.blogspot.com/2009/12/taiwan-to-resume-usa-beef-ban-over-mad.html



Tuesday, November 10, 2009

Surveillance On the Bovine Spongiform Encephalopathy and rabies in Taiwan and USA


http://usdavskorea.blogspot.com/2009/11/surveillance-on-bovine-spongiform.html



Tuesday, January 5, 2010

JOINT STATEMENT FROM USTR, USDA ON TAIWAN'S ACTIONS TO UNJUSTIFIABLY RESTRICT U.S. BEEF IMPORTS IN VIOLATION OF OUR BILATERAL AGREEMENT Release No. 0002.10 Contact: USTR, Nefeterius McPherson (202) 395-3230 USDA, Caleb Weaver (202) 720-4623


http://usdavskorea.blogspot.com/2010/01/joint-statement-from-ustr-usda-on.html




Tuesday, May 26, 2009

OIE upgrades Japan's BSE status to "controlled risk" Tue May 26, 2009 11:06am EDT

* OIE upgrades Japan's BSE status to "controlled risk"

* Decision to add pressure on Japan to accept U.S. imports


http://docket-aphis-2006-0041.blogspot.com/2009/05/oie-upgrades-japans-bse-status-to.html




Monday, July 28, 2008

Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION Subject: Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION Date: August 24, 2005 at 2:47 pm PST

August 24, 2005

Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION

Greetings APHIS ET AL,

My name is Terry S. Singeltary Sr.

I would kindly like to comment on [Docket No. 05-004-1] RIN 0579-AB93 ;


http://madcowfeed.blogspot.com/2008/07/importation-of-whole-cuts-of-boneless.html




see answers to commentors ;


http://edocket.access.gpo.gov/2005/05-24057.htm




Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA Posted Apr 09 2009 7:13pm Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA


https://web01.aphis.usda.gov/BSEcom.nsf/0/b78ba677e2b0c12185256dd300649f9d?OpenD




PLEASE SEE FULL TEXT BELOW ;


-------- Original Message --------

Subject: USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA Date: Mon, 3 Nov 2003 11:05:09 -0600 From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy To: BSE-L@uni-karlsruhe.de

In Reply to:

USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA

[Docket No. 03-080-1] posted by TSS on November 3, 2003 at 10:21 am:

-------- Original Message --------

Subject: Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA

Date: Mon, 03 Nov 2003 12:20:52 -0600

From: "Terry S. Singeltary Sr." To: regulations@aphis.usda.gov

I would like to kindly comment on Docket No. 03-080-1

USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA ;

Under this proposal, ruminant and ruminant products eligible for entry into the United States from a BSE minimal risk region would include:


snip...full text ;


http://madcowfeed.blogspot.com/2008/07/docket-no-03-080-1-usda-issues-proposed.html



Prions: Protein Aggregation and Infectious Diseases

ADRIANO AGUZZI AND ANNA MARIA CALELLA

Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland

snip...

3. Sporadic Creutzfeldt-Jakob disease Approximately 85% of all human prion diseases are sporadic forms of CJD. For sCJD, there is no association with a mutant PRNP allele, nor is there any epidemiological evidence for exposure to a TSE agent through contact with people or animals infected with TSEs. sCJD cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the size and glycoform ratio of proteaseresistant prion protein identified on western blot (type 1 or type 2) (174). Heterozygosity (Met/Val) at PrP codon 129 appears to be associated with a lower risk (378) and/or prolonged incubation time (119, 387). The lack of routine laboratory testing for preclinical diagnosis makes the search for agent sources and other risk factors extremely difficult. At present, the means of acquisition of a TSE agent in these patients remains a mystery.

So far, there is no evidence for spontaneous PrPSc formation in any animal or human TSE.

In humans, the peak age incidence of sporadic CJD is 55–60 years. However, if spontaneous misfolding were the primary event, one might expect a continuously increasing incidence with age because more time would allow more opportunity for rare misfolding events.

snip...

Physiol Rev • VOL 89 • OCTOBER 2009 • www.prv.org

http://physrev.physiology.org/cgi/content/abstract/89/4/1105


Science 24 September 2004: Vol. 305. no. 5692, pp. 1918 - 1921 DOI: 10.1126/science.1103581

Perspectives BIOMEDICINE:

A Fresh Look at BSE

Bruce Chesebro*

snip...

BSE caused by spontaneous misfolding of the prion protein has not been proven.

snip...

What can we conclude so far about BSE in North America? Is the BSE detected in two North American cows sporadic or spontaneous or both? "Sporadic" pertains to the rarity of disease occurrence. "Spontaneous" pertains to a possible mechanism of origin of the disease. These are not equivalent terms. The rarity of BSE in North America qualifies it as a sporadic disease, but this low incidence does not provide information about cause. For the two reported North American BSE cases, exposure to contaminated MBM remains the most likely culprit. However, other mechanisms are still possible, including cross-infection by sheep with scrapie or cervids with CWD, horizontal transmission from cattle with endemic BSE, and spontaneous disease in individual cattle. Based on our understanding of other TSEs, the spontaneous mechanism is probably the least likely. Thus, "idiopathic" BSE--that is, BSE of unknown etiology--might be a better term to describe the origin of this malady.

snip...

References

S. B. Prusiner, Proc. Natl. Acad. Sci. U.S.A 95, 13363 (1998) [Medline]. P. G. Smith, R. Bradley, Br. Med. Bull. 66, 185 (2003) [Medline]. C. Weissmann, A. Aguzzi, Curr. Opin. Neurobiol. 7, 695 (1997) [Medline]. A. F. Hill et al., J. Gen. Virol. 80, 11 (1999) [Medline]. R. Chiesa et al., J. Virol. 77, 7611 (2003) [Medline]. G. Legname et al., Science 305, 673 (2004). D. Westaway et al., Cell 76, 117 (1994) [Medline]. B. Chesebro, Science 279, 42 (1998). A. G. Biacabe et al., EMBO Rep. 5, 110 (2004) [Medline]. Y. Yamakawa et al., Jpn. J. Infect. Dis. 56, 221 (2003) [Medline]. C. Casalone et al., Proc. Natl. Acad. Sci. U.S.A. 101, 3065 (2004) [Medline]. E. F. Houston et al., J. Gen. Virol. 83, 1247 (2002) [Medline].

Laboratory of Persistent Viral Diseases Bruce W. Chesebro, M.D., Chief The Laboratory of Persistent Viral Diseases (LPVD) is concerned with studies of persistent active or latent viral or prion disease infections. Investigators place particular emphasis on persistent infections of the nervous system and of the hemopoietic and lymphoid systems. The laboratory is also studying the roles of persistent infection in the development of retrovirus-induced immunosuppression. Models being examined include prion diseases of various species, murine and human retroviruses, and tick-borne encephalitis viruses.

http://www.sciencemag.org/cgi/content/full/305/5692/1918



DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN ABOUT EXPORTING CANADIAN CATTLE AND THE RISK FROM BSE INTO THE USA, AND THE FACT THAT THEY ;

''they don't wanna know, the dont' care'' "they don't want our comments'' ''level of absolute ignorance'' ''beef export'' ''nothing else matters'' ''yes, i think prions are bad to eat, and you can die from them''



http://maddeer.org/video/embedded/prusinerclip.html





Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks

Subject: Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks

Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks

Since 2002, an active surveillance program for transmissible spongiform encephalopathy in small ruminants in European Union countries allowed identification of a considerable number of atypical cases in small ruminant with similarities to the previously identified atypical scrapie cases termed Nor98.

Results: Here we report molecular and neuropathological features of eight atypical/Nor98 scrapie cases detected between 2002 and 2009. Significant features of the affected sheep included: their relatively high ages (mean age 7.9 years, range between 4.3 and 12.8), their breed (all Latxa) and their PRNP genotypes (AFRQ/ALRQ, ALRR/ALRQ, AFRQ/AFRQ, AFRQ/AHQ, ALRQ/ALRH, ALRQ/ALRQ).

All the sheep were confirmed as atypical scrapie by immunohistochemistry and immunoblotting. Two cases presented more PrP immunolabelling in cerebral cortex than in cerebellum.

Conclusions: This work indicates that atypical scrapie constitutes the most common small ruminant transmissible spongiform encephalopathy form in Latxa sheep in the Spanish Basque Country.

Moreover, a new genotype (ALRQ/ALRH) was found associated to atypical scrapie.

Author: Ana Rodriguez-MartinezJoseba GarridoSonia MazaLeyre BenedictoMarivi GeijoNieves GomezEsmeralda MinguijonSylvie BenestadRamon Juste Credits/Source: BMC Veterinary Research 2010, 6:17


http://7thspace.com/headlines/339700/atypicalnor98_scrapie_in_the_basque_country_a_case_report_of_eight_outbreaks.html




another outbreak of spontaneous atypical scrapie ???



FURTHER INTO THIS STUDY ;



Case presentation

Between 2002 and 2008 the mean number of sheep analysed per year in the Basque Country was 764 and until September 2009, the total number of animals screened added up to 5620. Of these, eight Latxa ewes with molecular and pathological features of AS/Nor98 were found. The cases were detected widely distributed within this region and amounted to a significantly (p=0.0196) higher prevalence (0.15 %) than

6

that of classical scrapie (0.02 %) (Table 1). The first two cases were fallen stock and appeared in 2004, the third one was a slaughtered ewe tested in 2005 that was confirmed by Western blotting as Nor98 at the Norwegian National Veterinary Institute. The three following cases were detected in 2008, the first two cases at the beginning of the year and the third at the end of the year. The two last cases were detected at the beginning of 2009 and had been slaughtered for human consumption. Clinical symptoms were reported in only one of the cases (M31 (2008)). It drew the attention of the veterinary inspector at the slaughterhouse because it showed slight neurological signs such as ataxia, and poor condition. There were no further veterinary inspection reports of clinical signs for the remaining slaughtered animals or any of the fallen stock. In this context, it needs to be emphasized that due to frequent lack of clinical records as a consequence of the inefficiency of passive surveillance, there is no adequate clinical information on these scrapie cases in general. The mean age of all the eight cases was 7.9 years (range between 4.3 and 12.8).

SNIP...

We described eight atypical scrapie cases detected between 2002 and 2009 from the Basque Country. All AS/Nor98 cases were found in Latxa sheep which breed represents 85% of the Basque Country sheep population [54,55,57]. The occurrence of these cases seemed to be random and, in agreement with other AS surveillance studies [22], there was no apparent temporal trend,. Geographically, the distribution of atypical scrapie cases was in accordance with that described in other regions of the world. First, a single positive sheep per affected flock was detected, as observed in the majority of other AS cases [1,19,20]. Second, they presented a wide distribution in the Basque Country, with reports of cases in all three provinces. At this point it should be mentioned that the highest proportion was observed in Guipuzcoa (62.5%) but it may be due to the fact that it constituted the province with the highest rate of sheep slaughter

9

(over 90%) analysed in the Basque Country. Nevertheless, the sample size was still too small to draw any definite conclusion. Moreover, the occurrence of atypical scrapie cases pointed to an absence of time clustering, since there were long periods with no detection of cases and then, within a few months 2 or 3 affected animals were detected. However, it must be taken into consideration that, (i) not all sheep older than 18 months of age were analysed as a consequence of the random sampling procedure contrary to the exhaustive one legislated for cattle, (ii) the brain sampling may not have been optimal, e.g. only the medulla oblongata was collected or, particularly in the case of fallen stock, the brain samples were sometimes severely autolytic and liquefied, thus increasing the chances of sampling an area where PrPSc was absent, (iii) due to the young age of some animals, the stage of the disease, the small sampling site and the relatively low number of animals and short period of time involved, the possibility of longer and more tenuous temporal and spatial trends in PrPSc distribution could not be excluded. For these reasons, the number of AS and CS cases may be underrepresented and could suffer from a certain bias. One of the cases (M45) described here was confirmed to be Nor98. PrPSc deposition, distribution and molecular profile of the cases M72, M31, M15, M9-1 and M9-2 were identical to the features of this Nor98-confirmed case and to previous descriptions [1,38]. Moreover, the mean age observed was in accordance to other observations for atypical scrapie [36]. Among some of the features our cases had in common with M45, the following should be emphasised: i) the molecular protein profile showed a characteristic low molecular weight band under 14 kDa, ii) the cerebellum was the most affected region, iii) PrPSc was mainly detected in the neuropil predominantly as fine granular deposits, and iv) a faint to moderate PrPSc signal intensity was seen. The detection of more intense PrPSc deposits in cerebellum or

10

cerebral cortex rather than in the medulla oblongata may indicate that the prion is likely not to enter the brain through the medulla (DMNV) as described for classical scrapie [58], thus suggesting a rather sporadic aetiology, as observed in human sporadic TSE cases. Cases M15 and M27 however, presented some differences. Albeit PrPSc molecular pattern was similar to the Nor98 confirmed case, both animals showed more PrPSc deposits in the frontal lobe of cerebral cortex than in the cerebellum by immunohistochemistry (IHC) and also by immunoblot (WB) for case M27. By contrast, case M15 showed small differences between IHC and WB results since the signal in the pooled obex and cerebellum in WB was more intense than in the cerebellum and medulla oblongata in IHC. This could have been biased by the sampling for frozen tissues and by severe tissue autolysis and could be the explanation for a negative and extremely faint PrPSc signal in WB of case M7 in the cerebellum and obex, respectively. The fact that these cases showed more PrPSc accumulation in cerebral cortex than in cerebellum might be influenced by other still unknown environmental or genetics factors. Alternatively, this might happen more commonly than observed because of the limited number of AS/Nor98 cases where both the cerebellar and the cerebral cortices are available for analysis. When the sampling of brain is carried out with a spoon through the foramen magnum some cerebellum can also be collected along with the medulla oblongata and this can be targeted as the optimum sample for WB testing, particularly if the IHC results indicate a possible atypical scrapie case. Unfortunately, cerebrum is not routinely collected by this method so little is known about its PrPSc status. The availability of this brain region for M7 would have been useful to clear up any doubt on its diagnosis and classification. This case was questionable because we did not obtain a clear pattern in the WB with the band lower than 14kDa size and because the detected signal in the cerebellum by means of IHC was extremely faint. The poor

11

condition of the sample and the scant material available did not allow us to obtain, after repetitions of the analyses, a clear evidence of being an AS/Nor98 case. The fact that it was confirmed for the National Reference Laboratory allowed arguing that it was a scrapie case. Besides, there were several features supporting it as an atypical scrapie case. First, for CS, we would have expected to obtain a more intense signal in the WB and a clear three bands pattern of PrPSc in the region of the obex [17]. Second, even not having an optimal signal in the WB that showed the lower band characteristic of atypical cases and having evidences according to which the case was positive by means of IHC and rapid test, the possibility of an atypical scrapie case should not be excluded. In this case, it could have happened that there was little amount of abnormal PrPSc so that after PK digestion the amount of resistant PrPSc was reduced considerably below the detection threshold of WB, as observed in the cerebellum of case M15. Third, the age of this sheep was higher than the mean age of CS cases described in Latxa breed [59] and in other breeds [60,61]. Finally, this was the only detected case in its herd, which was in agreement with the epidemiology of the AS/Nor98 [1,19,20].

The majority of PrP genotypes described herein were observed in other AS/Nor98 cases [36]. We found an over-representation of animals carrying AF141RQ and AL141RQ alleles, suggesting that these alleles may confer more susceptibility to atypical scrapie in Latxa breed sheep. We also described a novel genotype associated to AS that has not been previously described (AL141RQ/AL141RH).

Case reports from this study and other case reports from Spain (http://www.eeb.es/pags/espana.htm) and Portugal [35], indicate a high frequency of atypical cases compared to CS outbreaks in the Iberian Peninsula. It could be speculated that AS/Nor98 is the traditional form of scrapie in the Iberian part of the Basque country, whilst in the French part, the classical form has been predominant [59]. This

12

would point to some unidentified epidemiological features limiting the spread of classical scrapie in the Iberian Peninsula. However, since the analysis of all sheep can not be guaranteed, it is difficult to test this hypothesis. The analysis of all sheep would provide more information about the epidemiology and pathology of this disease. Moreover, it could contribute in assessing whether AS is present in the Basque Country with the same high frequencies as others human TSEs, such as sporadic Creutzfeldt- Jakob disease [60] and Fatal Familiar Insomnia compared to other Spanish autonomous communities (National Epidemiology Centre:





http://www.isciii.es/htdocs/centros/epidemiologia/epidemiologia_listado_ecj.jsp).



Conclusions

This work indicates that AS/Nor98 constitutes the most common small ruminant Transmissible Spongiform Encephalopathy form in the Latxa breed in the Spanish Basque Country, where it also affects a genotype (ALRQ/ALRH) not previously associated to this form of TSE.


SNIP....SEE FULL TEXT ;


Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks BMC Veterinary Research 2010, 6:17 doi:10.1186/1746-6148-6-17




http://www.biomedcentral.com/content/pdf/1746-6148-6-17.pdf




TSS

Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010

Greetings,

Unusual event if you consider the officials hypothisis that Nor-98 atypical scrapie is a spontaneous event. seems there was a great deal of spontaneous mutations for this time period ;-)...TSS


http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html



Thursday, March 11, 2010

CANADA TYPICAL AND ATYPICAL SCRAPIE REPORT TO MARCH 2010


http://nor-98.blogspot.com/2010/03/canada-typical-and-atypical-scrapie.html



Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE


http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html



Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types

hmmm, this is getting interesting now...

Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,

see also ;

All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.


http://cjdusa.blogspot.com/2009/09/co-existence-of-scrapie-prion-protein.html



see full text ;

Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types


http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html



P03.141

Aspects of the Cerebellar Neuropathology in Nor98

Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute,

Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.


http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


PR-26

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway

Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion.

*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.

119


http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf


A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,?? +Author Affiliations

*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)

Abstract Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.


http://www.pnas.org/content/102/44/16031.abstract


Monday, December 1, 2008

When Atypical Scrapie cross species barriers

Authors

Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France.

Content

Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence.

The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie.

Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.

Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.

(i) the unsuspected potential abilities of atypical scrapie to cross species barriers

(ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier

These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf

SCRAPIE, ATYPICAL, OVINE - AUSTRALIA: (WESTERN AUSTRALIA) SUSPECTED ******************************************************************* A ProMED-mail post ProMED-mail is a program of the International Society for Infectious Diseases

[1] Date: Fri 12 Mar 2010

Source: The Australian [edited]



A West Australian sheep has been found to have signs characteristic of the fatal brain disease atypical scrapie. It comes as Australia faces growing anger from its trade partners over the Rudd government's surprise decision to extend a ban on the importation of beef from countries exposed to mad cow disease for a further 2 years.

Australia's chief veterinarian, Andy Carroll, told the ABC an indicative case of the atypical scrapie had been confirmed but said it posed no risk to human or animal health or the safety of eating meat and animal products.

Nor does atypical scrapie carry the dire trade consequences associated with classical scrapie.

Classical scrapie is in the same transmissible spongiform encephalopathies (TSE) family as BSE, better known as mad cow disease, from which humans can be fatally infected.

Dr Carroll said samples from the sheep's brain were being sent to the World Reference Laboratory in Britain.

Neither atypical scrapie nor classical scrapie has been seen in Australia before, but a sheep in New Zealand tested positive to the atypical form last year [2009].

Atypical scrapie is a relatively recently discovered disease and the common scientific view is that it occurs spontaneously or naturally in very small numbers of older sheep in countries all over the world.

[Byline: Jodie Minus]

-- Communicated by: Sabine Zentis Castleview Pedigree English Longhorns Gut Laach 52385 Nideggen Germany

****** [2] Date: Wed 10 Mar 2010 Source: ABC News (Australian Broadcasting Corporation) [edited]

Animal health authorities are testing a sheep's brain for what could be Australia's 1st case of the disease atypical scrapie.

Although not confirmed, the sheep is thought to be from Western Australia.

This type of scrapie is described as a sporadic degenerative brain condition affecting older sheep, and is not contagious.

Ed Klim, from national advisory group SafeMeat, says a 2nd round of testing is now taking place. "We've been made aware that the Australian Animal Health Laboratory is conducting further routine testing on a sheep sample," he says.

"The disease isn't considered a health risk nor should have any impact on food safety or export markets for sheep meat of live sheep."

Australia's chief veterinarian and WA's Department of Agriculture of Food are both aware of the testing but will not comment.

-- Communicated by: Terry S Singeltary Sr

[Although atypical scrapie is not yet ruled out, it is important to realize this is a type of scrapie that thus far has only tended to appear as a sporadic condition in older animals. Currently it has not been shown to follow the same genetic tendencies for propagation as the usual scrapie.

However, the atypical phenotypic appearance has been shown to be preserved on experimental passage.

Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries, as Australia may join that list. It is likely that this case will be sent to the UK for definitive conformation.

[Ref: M Simmons, T Konold, L Thurston, et al. BMC Veterinary Research 2010, 6:14 [provisional abstract available at ]

"Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]

"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."

Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG]

The HealthMap/ProMED-mail interactive map of Australia is available at . - Sr.Tech.Ed.MJ]

[see also: 2009 ---- Scrapie, atypical, ovine - New Zealand (02) 20091029.3740 Scrapie, atypical, ovine - New Zealand 20090220.0714 2007 ---- Scrapie, atypical, sheep - USA (WY): 1st report 20070318.0949 2005 ---- Scrapie, atypical, ovine - Falkland Islands 20051120.3371 2004 ---- Scrapie, atypical, sheep - UK and Ireland 20041210.3274 Scrapie, atypical, sheep - UK (02) 20040409.0965 Scrapie, atypical, sheep - UK 20040408.0952 Scrapie, atypical, sheep - France: OIE 20040201.0390] ...................................sb/tg/mj/lm

*##########################################################* ************************************************************



http://www.promedmail.org/pls/apex/f?p=2400:1001:3033292671016132::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,81729






Thursday, March 18, 2010

The natural atypical scrapie phenotype is preserved on experimental transmission and sub-passage in PRNP homologous sheep



http://nor-98.blogspot.com/2010/03/natural-atypical-scrapie-phenotype-is.html



1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

snip...

The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

PMID: 6997404


http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract


Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6


http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf



Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).


http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html



EMBO reports 4, 5, 530–533 (2003) doi:10.1038/sj.embor.embor827 AOP Published online: 11 April 2003

Widespread PrPSc accumulation in muscles of hamsters orally infected with scrapie

Achim Thomzig, Christine Kratzel, Gudrun Lenz, Dominique Krüger & Michael Beekes

Robert Koch-Institut, P26, Nordufer 20, D-13353 Berlin, Germany

To whom correspondence should be addressed Michael Beekes Tel: +49 30 4547 2396; Fax: +49 30 4547 2609; BeekesM@rki.de

Received 13 February 2003; Accepted 13 March 2003; Published online 11 April 2003.

Abstract

Scrapie, bovine spongiform encephalopathy and chronic wasting disease are orally communicable, transmissible spongiform encephalopathies (TSEs). As zoonotic transmissions of TSE agents may pose a risk to human health, the identification of reservoirs for infectivity in animal tissues and their exclusion from human consumption has become a matter of great importance for consumer protection. In this study, a variety of muscles from hamsters that were orally challenged with scrapie was screened for the presence of a molecular marker for TSE infection, PrPSc (the pathological isoform of the prion protein PrP). Sensitive western blotting revealed consistent PrPSc accumulation in skeletal muscles from forelimb and hindlimb, head, back and shoulder, and in tongue. Previously, our animal model has provided substantial baseline information about the peripheral routing of infection in naturally occurring and orally acquired ruminant TSEs. Therefore, the findings described here highlight further the necessity to investigate thoroughly whether muscles of TSE-infected sheep, cattle, elk and deer contain infectious agents.

EMBO reports 4, 5, 530–533 (2003) doi:10.1038/sj.embor.embor827 AOP Published online: 11 April 2003



http://www.nature.com/embor/journal/v4/n5/full/embor827.html





EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE

This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........



http://web.archive.org/web/20030517224223/http://www.bseinquiry.gov.uk/files/yb/1994/02/07002001.pdf


Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

Thursday, March 18, 2010

The natural atypical scrapie phenotype is preserved on experimental transmission and sub-passage in PRNP homologous sheep

The natural atypical scrapie phenotype is preserved on experimental transmission and sub-passage in PRNP homologous sheep

BMC Veterinary Research 2010, 6:14 doi:10.1186/1746-6148-6-14 Marion M Simmons (m.m.simmons@vla.defra.gsi.gov.uk) Timm Konold (t.konold@vla.defra.gsi.gov.uk) Lisa Thurston (l.thurston@vla.defra.gsi.gov.uk) Susan J Bellworthy (s.j.bellworthy@vla.defra.gsi.gov.uk) Melanie J Chaplin (m.j.chaplin@vla.defra.gsi.gov.uk) S JO Moore (j.s.moore@vla.defra.gsi.gov.uk)

ISSN 1746-6148 Article type Research article Submission date 26 October 2009 Acceptance date 10 March 2010 Publication date 10 March 2010 Article URL http://www.biomedcentral.com/1746-6148/6/14

Like all articles in BMC journals, this peer-reviewed article was published immediately upon acceptance. It can be downloaded, printed and distributed freely for any purposes (see copyright notice below).

Articles in BMC journals are listed in PubMed and archived at PubMed Central. For information about publishing your research in BMC journals or any BioMed Central journal, go to http://www.biomedcentral.com/info/authors/

The natural atypical scrapie phenotype is preserved on experimental transmission and sub-passage in PRNP homologous sheep

Marion M Simmons1*, Timm Konold1, Lisa Thurston1, Susan J Bellworthy1, Melanie J Chaplin2, S Jo Moore1

1Department of Pathology, Veterinary Laboratories Agency Weybridge, New Haw, Addlestone KT15 3NB, UK

2Molecular Pathogenesis and Genetics Department, Veterinary Laboratories Agency Weybridge, New Haw, Addlestone KT15 3NB, UK

*Corresponding author Email addresses: MMS: m.m.simmons@vla.defra.gsi.gov.uk TK: t.konold@vla.defra.gsi.gov.uk LT: l.thurston@vla.defra.gsi.gov.uk SJB: s.j.bellworthy@vla.defra.gsi.gov.uk MJC: m.j.chaplin@vla.defra.gsi.gov.uk SJM: j.s.moore@vla.defra.gsi.gov.uk

Abstract

Background

Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries. Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. The first successful challenge of a sheep with ‘field’ atypical scrapie from an homologous donor sheep was reported in 2007.

Results

This study demonstrates that atypical scrapie has distinct clinical, pathological and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

Conclusions

Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage.

snip...

Discussion

This data confirms the experimental transmissibility of atypical scrapie, and the stability of disease phenotype – clinical, biochemical and pathological – on PRNP homologous experimental transmission in AHQ/AHQ animals. The mean incubation time of these cases (751 days) is approximately 50% of the average age at death in field cases with atypical scrapie [20]. This observation that intracerebral challenge shortens the incubation period is consistent with the substantial reductions in incubation period which are seen in the classical scrapie controls which are 90% (for VRQ/VRQ) and 60% (for VRQ/ARQ) less than the average age at onset of field cases (Ortiz-Pelaez, personal communication) in these genotypes.

There was a slight shortening of mean incubation period between the initial experimentally challenged sheep (790 days) and those which succumbed following sub-passage (695 days), but the group sizes are too small to draw any robust conclusions from this observation.

The wide range of incubation periods seen cannot be readily interpreted due to the very small numbers of animals in this study (a necessary restriction due to the very limited amount of suitable donor material, and limited number of recipients of a suitable genotype). It is interesting to note that the outliers (cases 1 and 2, Table 1) received the same inoculum. All donor inocula give similar incubation periods in ovinised transgenic (Tg338) mice when inoculated as part of a separate study (Spiropoulos, personal communication), although some difference in titre is likely to be masked by the sensitivity of these mice. In one titration of an atypical scrapie isolate the mean incubation period did not change more than 10% over the first three log dilutions (Spiropoulos and Simmons, unpublished data).

The absence of any visible lymphoreticular system (LRS) involvement in the experimentally challenged animals is consistent with what has been observed in natural cases of atypical scrapie [13] and cannot be attributed to the experimental route of exposure, since the classical scrapie controls challenged by the same route had widespread labelling of disease-associated PrP (PrPSc) in the lymphoid tissues. The clinical presentation of atypical scrapie cases was different to classical scrapie or BSE in sheep: atypical scrapie did not appear to cause evident pruritus, whereas the scrapie and BSE cases described here usually presented with pruritus. Compulsive behaviour, such as circling, was only observed in atypical scrapie, and an impaired menace response was considerably more frequent in atypical scrapie cases. The difference in the clinical picture does not appear to be confined to the genotype or the route of inoculation since similar signs in the absence of pruritus have been observed in naturally affected atypical scrapie cases of various genotypes [1,10,29], whereas pruritus is frequent in naturally affected classical scrapie cases [26] or sheep orally challenged or naturally infected with the BSE agent under experimental settings [24]. Ataxia with hypermetria, head tremor and an absent menace response, which were predominantly observed in sheep with atypical scrapie, are signs indicative of a dysfunction of the cerebellum [30]. This concurs with the lesion and PrPSc immunolabelling pattern distribution in atypical scrapie, which is particularly prominent in the cerebellum. However, the lateralisation of clinical signs (circling to one side or a unilaterally deficient menace response) seen in atypical scrapie is unusual and to the authors’ knowledge has not been described elsewhere in ovine BSE or classical scrapie. It is unlikely that the intracerebral route was responsible for the apparent lateralisation of clinical signs because it was not observed in ovine cases of BSE [24] and classical scrapie inoculated by the same route (cases 18-20, Table 1). Compulsive circling in the absence of vestibular signs like head tilt and nystagmus is suggestive of an asymmetric lesion of the forebrain, usually on the same side as the circling direction [31]. A unilateral absent menace response has been associated with a unilateral cerebellar lesion on the same side as the deficit [32]. This would suggest that the signs in case 2 (Table 1), which presented with a left absent menace response and circling to the left, was predominantly caused by a lesion of the left side of the brain. Unfortunately, the whole brain of this case was not available to evaluate symmetry of neuropathological changes, but there was no evidence of any pronounced asymmetrical lesion or PrPSc distribution in the bilateral sections examined in case 6, which also circled to the left.

Conclusions

This study shows that a single PRNP genotype, AHQ/AHQ can display a range of disease phenotypes which are consistent and readily distinguishable from each other, supporting the suggestion that such characteristics can be attributable, predominantly, to the agent strain. However, the relative over-representation of the AHQ genotype in the atypical naturally-affected populations supports the hypothesis that field strains have strong genotype ‘tropisms’ or that there is genotype selection of strains, as proposed by Spiropoulos [15]. The consistent disease phenotype seen in natural disease, primary experimental transmission and subsequent passage, supports the experimental inoculation model as a valid tool for the study of atypical scrapie, regardless of whether the disease in the field is acquired or spontaneous. It also enables the creation of atypical scrapie samples in a consistent manner for test evaluation and/or quality assurance purposes, helping to improve surveillance approaches for disease detection and confirmation in the field.


http://www.biomedcentral.com/content/pdf/1746-6148-6-14.pdf




Archive Number 20100312.0803
Published Date 12-MAR-2010
Subject PRO/AH/EDR> Scrapie, atypical, ovine - Australia: (WA) susp


SCRAPIE, ATYPICAL, OVINE - AUSTRALIA: (WESTERN AUSTRALIA) SUSPECTED ******************************************************************* A ProMED-mail post ProMED-mail is a program of the International Society for Infectious Diseases

[1] Date: Fri 12 Mar 2010 Source: The Australian [edited]

A West Australian sheep has been found to have signs characteristic of the fatal brain disease atypical scrapie. It comes as Australia faces growing anger from its trade partners over the Rudd government's surprise decision to extend a ban on the importation of beef from countries exposed to mad cow disease for a further 2 years.



snip...


[Although atypical scrapie is not yet ruled out, it is important to realize this is a type of scrapie that thus far has only tended to appear as a sporadic condition in older animals. Currently it has not been shown to follow the same genetic tendencies for propagation as the usual scrapie.

However, the atypical phenotypic appearance has been shown to be preserved on experimental passage.

Atypical scrapie was first identified in Norwegian sheep in 1998 and has subsequently been identified in many countries, as Australia may join that list. It is likely that this case will be sent to the UK for definitive conformation.

[Ref: M Simmons, T Konold, L Thurston, et al. BMC Veterinary Research 2010, 6:14 [provisional abstract available at ]

"Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]

"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."

Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG]

The HealthMap/ProMED-mail interactive map of Australia is available at . - Sr.Tech.Ed.MJ]




http://www.promedmail.org/pls/apex/f?p=2400:1001:589791602457438::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,81729



hmmm, this is getting interesting now...


Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine (reticular) deposits,

see also ;

All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.


http://cjdusa.blogspot.com/2009/09/co-existence-of-scrapie-prion-protein.html



see full text ;

Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types


http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html




Epidemiology of Scrapie in the United States 1977


http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf


http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf




Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010


http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html



Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010


http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html



Tuesday, April 28, 2009

Nor98-like Scrapie in the United States of America


http://nor-98.blogspot.com/2009/04/nor98-like-scrapie-in-united-states-of.html



P03.141

Aspects of the Cerebellar Neuropathology in Nor98

Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute,

Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.


http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf



PR-26

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway

Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion.

*** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.

119


http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf



A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,?? +Author Affiliations

*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)

Abstract Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.


http://www.pnas.org/content/102/44/16031.abstract



Monday, December 1, 2008

When Atypical Scrapie cross species barriers

Authors

Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France.

Content

Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence.

The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie.

Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.

Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.

(i) the unsuspected potential abilities of atypical scrapie to cross species barriers

(ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier

These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.


http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf



12/10/76

AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to Scrapie A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries.

The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.

It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.

Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6


http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf




Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).


http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html



1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

snip...


The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.


PMID: 6997404


http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract



Like lambs to the slaughter
31 March 2001
by Debora MacKenzie Magazine issue 2284

FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in ...


http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html



14th ICID International Scientific Exchange Brochure - Final Abstract Number: ISE.114 Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. SingeltaryBacliff, TX, USA

Background:An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:12 years independent research of available data

Results:I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

see page 114 ;

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf



Tuesday, March 16, 2010

COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef FRIDAY, 5 FEBRUARY 2010 AUSTRALIA

COMMONWEALTH OF AUSTRALIA

Proof Committee Hansard


SENATE RURAL AND REGIONAL AFFAIRS AND TRANSPORT REFERENCES COMMITTEE Reference: Import restrictions on beef

FRIDAY, 5 FEBRUARY 2010 CANBERRA CONDITIONS OF DISTRIBUTION

This is an uncorrected proof of evidence taken before the committee. It is made available under the condition that it is recognised as such. BY AUTHORITY OF THE SENATE [PROOF COPY] TO EXPEDITE DELIVERY, THIS TRANSCRIPT HAS NOT BEEN SUBEDITED

SNIP...

Friday, 5 February 2010 Senate RRA&T 1

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

Committee met at 9.01 am

CHAIR (Senator Nash)—I declare open this public hearing of the Rural and Regional Affairs and Transport References Committee. The committee is hearing evidence on the committee’s inquiry into the impact and consequences of the government’s decision to relax import restrictions on beef. Before the committee starts taking evidence I remind all witnesses that, in giving evidence to the committee, they are protected by parliamentary privilege. It is unlawful for anyone to threaten or disadvantage a witness on account of evidence given to a committee and such action may be treated by the Senate as a contempt. It is also a contempt to give false or misleading evidence to a committee. The committee prefers all evidence to be given in public but, under the Senate’s resolutions, witnesses have the right to request to be heard in private session. It is important that witnesses give the committee notice if they intend to ask to give evidence in camera. If a witness objects to answering a question, the witness should state the ground upon which the objection is taken and the committee will determine whether it will insist on an answer, having regard to the ground which is claimed. If the committee determines to insist on an answer, a witness may request that the answer be given in camera. Such a request may, of course, also been made at any other time. On behalf of the committee, I thank all those who have made submissions and sent representatives here today for their cooperation in this inquiry.

RRA&T 2 Senate Friday, 5 February 2010

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

[9.03 am]

BELLINGER, Mr Brad, Chairman, Australian Beef Association

CARTER, Mr John Edward, Director, Australian Beef Association

CHAIR—Welcome. Would you like to make an opening statement?

Mr Bellinger—Thank you. The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He states, and rightfully so:

You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heinemann variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. He goes on to state:

The OIE— the International Organisation of Epizootics, the arm of the WTO— is a failed global agent that in my opinion is bought off via bogus regulations for global trade and industry reps. I have done this all these years for nothing but the truth. I am a consumer, I eat meat, but I do not have to sit idly by and see the ignorance and greed of it all while countless numbers of humans and animals are being exposed to the TSE agents. All the USA is interested in is trade, nothing else matters.

Even Dr Stanley Prusiner, who incidentally won the Nobel Health Prize in 1997 for his work on the prion—he invented the word ‘prion’, or it came from him—states:

The BSC policy was set up for one purpose only, trade—the illegal trading of all strains of TSE globally throughout North America, which is home to CBSC, IBSC and HBSC, many scrapie strains and two strains of CJD to date. (please note typo error, those should have read cBSE, lBSE, and hBSE...tss)

I would also like, while I have the opportunity, to explain the beef-off-the-shelves myth. At the first Senate hearing on 14 December, it was explained that the reason why they allowed BSC beef into Australia was the beef-off-the-shelves policy, whereby if we found a case of BSC in Australia they would have to recall all—

Friday, 5 February 2010 Senate RRA&T 3

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

Senator HEFFERNAN—Which of course is total BS.

Mr Bellinger—Correct. This is written in the FSANZ document—Food Standards Australia New Zealand. Why isn’t this same policy in New Zealand? It is not—it is only in Australia. We are the only country in the world to have this idiotic policy. So we again call for the tabling of the WTO obligations paperwork. We do not believe that exists.

Mr Carter—We have an additional concern about human health. We are not scientists, but on 18 December, four days after the last hearing here, the BBC reported a new wave of deaths due to variant CJD linked to eating BSE infected beef could be underway. This is based on the work of Professor John Collinge of the National Prion Clinic, who reported that a 2009 death in Scotland was from a different genetic pool to that of the 166 deaths already reported in the UK. Those are all thought to share one gene, but Professor Collinge and his colleagues estimate that up to 350 people in this new group, represented by the person who died in Scotland, could get CJD. He thinks that CJD has moved into a new phase, and the incubation period is a long one. We tender the Australian Red Cross donor policy sheet, which bears out what Senator Back brought up last time, questioning the Chief Medical Officer, and we say that blood from people who were in the UK between 1980 and 1996 is not acceptable. That is the current ruling. We believe this now should be extended to anyone who has visited the UK, and this new evidence should ensure that Australia revisits the science of CJD.

CHAIR—Thank you, Mr Carter. Before we kick off, can I just remind colleagues that we are short of time today, so I ask that we do not traverse ground the we have previously covered and make sure that we stick to new information that is required. Mr Bellinger, when you started you referred to your view that this decision to allow the importation was politically based. I know you are going to go into this in the course of the next 20 minutes or so, but could you just give us a quick outline of what your definition of politically based is and why you think the decision was politically based?

Mr Bellinger—On the lowering of BSE standards: if you go back to 2006, for example, there were five categories for describing countries that had BSE and Australia was in the category for BSE free. Suddenly, by the time the United States got their third instance of BSE, through the influence of Robert Zoellick—who was the trade minister that signed the BSE corresponding side letter in 2004 and was George Bush’s appointment to the WTO—they suddenly changed the five categories to three categories and, instead of being BSE free, Australia became BSE negligible risk. At the time I put out a press release alerting the media to the dangers of this happening, and we are coming to the stage here when suddenly our government is saying, ‘Now let’s allow the importation of beef from BSE affected countries.’ I believe that the WTO has been influenced by large multinational meat processors and retailers to change and allow the trading of BSE beef throughout the world.

CHAIR—Thanks, Mr Bellinger.

Mr Carter—Of course, the side letter that Minister Vaile signed was at the request of Mr Zoellick, who is now in the position that Mr Bellinger has explained.

Senator HEFFERNAN—I just want to put the committee on notice that, if we do not get through what we have got to get through today, I suggest we have another hearing, because this

RRA&T 4 Senate Friday, 5 February 2010

RURAL AND REGIONAL AFFAIRS AND TRANSPORT

is the greatest ambush of Australia’s farmers of all time by a government. The evidence given at the last meeting was deadset lies. The proposition that this whole change of government policy was led by the industry is a deadset lie. While Simon Crean might want to change his mind because of the WTO and his lack of knowledge, the Australian beef industry, as you know, is under great challenge, not only from the currency but also from the undermining of our markets. This is a disgrace.



SNIP...PLEASE SEE FULL TEXT ;



*************


Tuesday, March 16, 2010


COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef FRIDAY, 5 FEBRUARY 2010 AUSTRALIA

COMMONWEALTH OF AUSTRALIA

Proof Committee Hansard


snip...see full text 110 pages ;


http://www.aph.gov.au/hansard/senate/commttee/S12742.pdf



for those interested, please see much more here ;


http://docket-aphis-2006-0041.blogspot.com/2010/03/commonwealth-of-australia-hansard.html



2008 - 2010

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.


http://www.cjdfoundation.org/fact.html



CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.


http://www.cjdsurveillance.com/pdf/case-table.pdf



Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***


http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html



my comments to PLosone here ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd



Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION


http://cjdquestionnaire.blogspot.com/



Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009


http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

Thursday, March 11, 2010

CANADA TYPICAL AND ATYPICAL SCRAPIE REPORT TO MARCH 2010

CANADA TYPICAL AND ATYPICAL SCRAPIE REPORT TO MARCH 2010

Flocks infected with scrapie in Canada in 2010 The CFIA, in co-operation with provincial governments and industry, launched a national scrapie surveillance program in 2005. Under the program, producers are encouraged to report animals that die on the farm or exhibit symptoms of the disease.

In addition, scrapie is a reportable disease under the Health of Animals Regulations. This means that all suspected cases must be reported to the CFIA.

The following table lists sheep flocks and/or goat herds confirmed to be infected with scrapie in Canada in 2010.

Updated: 2010-02-28

Date confirmed Location Animal type infected


January 21 Alberta Sheep


January 25* Ontario Sheep


February 5 Quebec Sheep


February 5* Saskatchewan Sheep


*Atypical scrapie


http://www.inspection.gc.ca/english/anima/disemala/rep/2010scrtree.shtml




BSE, BOVINE - CANADA: (ALBERTA) ******************************* A
ProMED-mail post



http://www.promedmail.org/pls/apex/f?p=2400:1001:2081979993451657::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,81711




Canada - Case of BSE (Mad cow disease) in 6 year old cow 17th domestic case


http://bseusa.blogspot.com/2010/03/canada-case-of-bse-mad-cow-disease-in-6.html




Wednesday, March 3, 2010

NOR-98 ATYPICAL SCRAPIE USA 4 CASES DETECTED JANUARY 2010


http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-usa-4-cases.html




Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518