Vet. Res. (2008) 39:19 DOI: 10.1051/vetres:2007056
Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology
Sylvie L. Benestad1, Jean-Noël Arsac2, Wilfred Goldmann3 and Maria Nöremark4
1 National Veterinary Institute, PO Box 8156 Dep., Oslo, Norway 2 Agence Française de Sécurité Sanitaire des Aliments, 31 av. Tony Garnier, 69364 Lyon Cedex 07, France 3 Neuropathogenesis Unit, Roslin Institute, Ogston Bldg, West Mains Rd, Edinburgh, EH9 3JF, UK 4 National Veterinary Institute, 75189 Uppsala, Sweden
(Received 23 July 2007; accepted 23 October 2007 ; published online 11 January 2008)
Abstract - Atypical/Nor98 scrapie cases in sheep were diagnosed for the first time in Norway in 1998. They are now identified in small ruminants in most European countries and represent an increasingly large proportion of the scrapie cases diagnosed in Europe. Atypical/Nor98 scrapie isolates have shown to be experimentally transmissible into transgenic mice and sheep but the properties of the TSE agent involved, like its biological and biochemical features, are so clearly distinct from the agent involved in classical scrapie that they have provided a challenging diagnostic for many years. No strain diversity has yet been identified among the atypical/Nor98 scrapie sample cases. The genetic predisposition of the sheep affected by atypical/Nor98 scrapie is almost inverted compared to classical scrapie, and the exact origin of this sporadic TSE strain is still speculative, but a spontaneous, non-contagious origin, like sporadic Creutzfeldt-Jakob disease in humans, can not be excluded. Further transmission and epidemiological studies are needed to better address this hypothesis.
Key words: atypical scrapie / Nor98 / transmissible spongiform encephalopathies TSE / genetics / epidemiology
Corresponding author: sylvie.benestad@vetinst.no
© INRA, EDP Sciences 2008
http://www.vetres.org/index.php?option=article&access=doi&doi=10.1051/vetres:2007056
The genetic predisposition of the sheep affected by atypical/Nor98 scrapie is almost inverted compared to classical scrapie, and the exact origin of this
sporadic TSE strain is still speculative, but a spontaneous, non-contagious origin, like sporadic Creutzfeldt-Jakob disease in humans, can not be excluded.
there are 26790 hits on 'sporadic' at pub-med.
there are 12830 hits on 'sporadic disease' at pub-med.
with the first few being ;
Comparison Analysis of Gene Expression Patterns between Sporadic Alzheimer's and Parkinson's Disease.
http://www.ncbi.nlm.nih.gov/pubmed/18198416?ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Sporadic ALS with early onset respiratory failure is not associated with IGHMBP2 gene mutations.
http://www.ncbi.nlm.nih.gov/pubmed/18187479?ordinalpos=10&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
Sporadic Multiple Cerebral Cavernomatosis: Report of a Case and Review of Literature
http://www.ncbi.nlm.nih.gov/pubmed/18195658?ordinalpos=7&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
A sporadic disease is one that occurs only occasionally in a population (i.e., prevalence is zero).
http://mansfield.osu.edu/~sabedon/biol2045.htm
sporadic CJD is NOT a single strain, phenotype.
sporadic CJD is simply a human TSE, which routes, sources, strains, are unknown.
there are 6 different documented phenotypes of the sporadic CJD, with 'UNKNOWN' sub-types growing.
spontaneous TSE in the field ??? never has been proven, and highly unlikely that 85%+ of all human TSE i.e. sporadic CJD, just happen without route and source.
Volume 12, Number 12–December 2006
Perspective
On the Question of Sporadic or Atypical Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease
Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§
*Bethesda, Maryland, USA; †National Institutes of Health, Bethesda, Maryland, USA; ‡University of Verona, Verona, Italy; and §Virginia-Maryland Regional College of Veterinary Medicine, College Park, Maryland, USA
snip...
Sporadic CJD
The possibility that at least some cases of apparently sporadic CJD might be due to infection by sporadic cases of BSE cannot be dismissed outright. Screening programs needed to identify sporadic BSE have yet to be implemented, and we know from already extant testing programs that at least a proportion of infected animals have no symptoms and thus would never be identified in the absence of systematic testing. Thus, sporadic BSE (or for that matter, sporadic disease in any mammalian species) might be occurring on a regular basis at perhaps the same annual frequency as sporadic CJD in humans, that is, in the range of 1 case per million animals.
Whether humans might be more susceptible to atypical forms of BSE cannot be answered at this time. Experimentally transmitted BASE shows shorter incubation periods than BSE in at least 1 breed of cattle, bovinized transgenic mice, and Cynomolgus monkeys (12,13). In humanized transgenic mice, BASE transmitted, whereas typical BSE did not transmit (13). Paradoxically, the other major phenotype (H) showed an unusually long incubation period in bovinized transgenic mice (12).
The limited experimental evidence bearing on a possible relationship between BSE and sporadic CJD is difficult to interpret. The original atypical BASE strain of BSE had a molecular protein signature very similar to that of 1 subtype (type 2 M/V) of sporadic CJD in humans (5). In another study, a strain of typical BSE injected into humanized mice encoding valine at codon 129 showed a glycopattern indistinguishable from the same subtype of sporadic CJD (15). In a third study, the glycopatterns of both the H and L strains of atypical BSE evidently did not resemble any of the known sporadic CJD subtypes (12).
To these molecular biology observations can be added the epidemiologic data accumulated during the past 30 years. The hypothesis that at least some cases of apparently sporadic CJD are due to unrecognized BSE infections cannot be formally refuted, but if correct, we might expect by now to have some epidemiologic evidence linking BSE to at least 1 cluster of apparently sporadic cases of CJD. Although only a few clusters have been found (and still fewer published), every proposed cluster that has been investigated has failed to show any common exposure to bovines. For that matter, no common exposure has been shown to any environmental vehicles of infection, including the consumption of foodstuffs from bovine, ovine, and porcine sources, the 3 livestock species known to be susceptible to transmissible spongiform encephalopathies. Additional negative evidence comes from several large case-control studies in which no statistically significant dietary differences were observed between patients with sporadic CJD and controls (16,17).
On the other hand, the difficulty of establishing a link between BSE and CJD may be compounded by our ignorance of the infectious parameters of a sporadic form of BSE (e.g., host range, tissue distribution of infectivity, route of transmission, minimum infectious dose for humans, whether single or multiple). Presumably, these parameters would resemble those of variant CJD; that is, high infectivity central nervous system and lymphoreticular tissues of an infected cow find their way into products consumed by humans. Transmissions that might have occurred in the past would be difficult to detect because meat products are generally not distributed in a way that results in detectable geographic clusters.
Barring the discovery of a specific molecular signature (as in variant CJD), the most convincing clue to an association will come from the observation of trends over time of the incidence of typical and atypical BSE and of sporadic and variant CJD. With 4 diseases, each of which could have increasing, unchanging, or decreasing trends, there could be 81 (34) possible different combinations. However, it is highly likely that the trends for typical BSE and variant CJD will both decrease in parallel as feed bans continue to interrupt recycled contamination. The remaining combinations are thus reduced to 9 (32), and some of them could be highly informative.
For example, if the incidence of atypical BSE declines in parallel with that of typical BSE, its candidacy as a sporadic form of disease would be eliminated (because sporadic disease would not be influenced by current measures to prevent oral infection). If, on the other hand, atypical BSE continues to occur as typical BSE disappears, this would be a strong indication that it is indeed sporadic, and if in addition at least 1 form of what is presently considered as sporadic CJD (such as the type 2 M/V subtype shown to have a Western blot signature like BASE) were to increase, this would suggest (although not prove) a causal relationship (Figure 5).
Recognition of the different forms of BSE and CJD depends upon continuing systematic testing for both bovines and humans, but bovine testing will be vulnerable to heavy pressure from industry to dismantle the program as the commercial impact of declining BSE cases ceases to be an issue. Industry should be aware, however, of the implications of sporadic BSE. Its occurrence would necessitate the indefinite retention of all of the public health measures that exclude high-risk bovine tissues from the animal and human food chains, whereas its nonoccurrence would permit tissues that are now destroyed to be used as before, once orally acquired BSE has disappeared.
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm
THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ;
CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006
The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.
The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.
These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.
"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."
Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
http://www.upi.com/
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm
PAUL BROWN COMMENT TO ME ON THIS ISSUE
Tuesday, September 12, 2006 11:10 AM
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency." ........
Archive Number 20071105.3602 Published Date 05-NOV-2007 Subject PRO/AH/EDR> Prion disease update 2007 (07)
PRION DISEASE UPDATE 2007 (07)
snip...
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
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
JOURNAL OF NEUROLOGY
MARCH 26, 2003
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
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
http://cjdusa.blogspot.com/
Creutzfeldt Jakob Disease
http://creutzfeldt-jakob-disease.blogspot.com/
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
SEAC 99th meeting on Friday 14th December 2007
http://seac992007.blogspot.com/
16 January 2008 - The final minutes of the 98th SEAC meeting have been published.
PUBLIC QUESTION AND ANSWER SESSION 2© SEAC 2007SEAC considered a question about possible links between CJD cases and animal TSEs in the United States of America (USA).
http://www.seac.gov.uk/summaries/seac99_summary.pdf
vCJD case study highlights blood transfusion risk
http://vcjdblood.blogspot.com/
vCJD transfusion-associated Fourth Case UK
http://vcjdtransfusion.blogspot.com/
risk factors for sporadic CJD
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html
Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html
BSE (Mad Cow) Update: Do Reports of sCJD Clusters Matter?
snip... see full text ;
http://cjdtexas.blogspot.com/
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/
doi:10.1016/S1473-3099(03)00715-1Copyright © 2003 Published by Elsevier Ltd.
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003. Volume 3, Issue 8, August 2003, Page 463
"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." ...
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
ANIMAL HEALTH REPORT 2006 (BSE h-BASE EVENT IN ALABAMA, Scrapie, and CWD)
http://animalhealthreport2006.blogspot.com/
P03.141
Aspects of the Cerebellar Neuropathology in Nor98
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E11National 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 avariation 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 synaptophys in, 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.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007
typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=6997404&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVAbstractPlus
full text ;
http://nor-98.blogspot.com/
http://scrapie-usa.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/
ONE other question from Confucius,
IF we are to believe as some has so persistently tried to claim without any proof, if we are to believe that these TSE just happen spontaneous, then why have there been NO other cases of TME in the USA ???
WHERE are these cases of spontaneous BSE/BASE in the USA ???
WHAT about the FSE cases, none in the USA either, no feline spongiform encephalopathy in USA documented to date ???
IF the spontaneous TSE truely exist, would we have not seen these species with TSE here in the USA ???
WHAT about Europe, if the spontanous TSE truely happened, would CWD, TME, not happen in the EU countries as well ???
OR is it just the USDA certified BASE mad cows that are spontaneous ;-) $$$
Saturday, December 01, 2007
Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model
http://transmissible-mink-encephalopathy.blogspot.com/
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
Monday, January 21, 2008
Tuesday, December 4, 2007
SCRAPIE PROGRAM FY REPORT 2007
SCRAPIE PROGRAM FY REPORT 2007
Prepared by National Center for Animal Health Programs Ruminant Health Programs Team November 15, 2007
snip...
Infected and Source Flocks
During FY 2007, there were a total of 76 new infected or source flocks identified. Of those new flocks identified, 30 were infected flocks and 46 were source flocks (Figure 2). As of September 30, 2007, there were 38 scrapie infected and source flocks with open statuses (Figure 3). ...
snip...
In FY 2007, 331 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), including 59* Regulatory Scrapie Slaughter Surveillance (RSSS) cases (Figure 5 and Slide 16). In FY 2007, two field cases, one validation case, and two RSSS cases were consistent with Nor-98 scrapie. The Nor98-like cases originated from flocks in California, Minnesota, Colorado, Wyoming and Indiana respectively. Nineteen cases of scrapie in goats have been reported since 1990 (Figure 6). The last goat case was reported in September 2007.
snip...
see full report here ;
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/yearly_report.pps
P03.141
Aspects of the Cerebellar Neuropathology in Nor98
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E11National Veterinary Insitute, Sweden; 2National Veterinary Institute, Norway
Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was firstdescribed in Norway in 1998. Several features of Nor98 were shown to be differentfrom classical scrapie including the distribution of disease associated prion protein(PrPd) accumulation in the brain. The cerebellum is generally the most affected brainarea in Nor98. The study here presented aimed at adding information on theneuropathology in the cerebellum of Nor98 naturally affected sheep of variousgenotypes 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 histologicallesions and tissue reactions were evaluated. The types of PrPd deposition werecharacterized. The cerebellar cortex was regularly affected, even though there was avariation in the severity of the lesions from case to case. Neuropil vacuolation wasmore marked in the molecular layer, but affected also the granular cell layer. There wasa loss of granule cells. Punctate deposition of PrPd was characteristic. It wasmorphologically and in distribution identical with that of synaptophysin, suggestingthat PrPd accumulates in the synaptic structures. PrPd was also observed in thegranule cell layer and in the white matter. The pathology features of Nor98 in thecerebellum of the affected sheep showed similarities with those of sporadicCreutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007
typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=6997404&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVAbstractPlus
full text ;
http://nor-98.blogspot.com/
http://scrapie-usa.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease inthe United States
http://cjdusa.blogspot.com/
i am reminded of a few things deep throat (high ranking official at usda)told me years ago;
==========================================
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........Dear God,
https://www.blogger.com/comment.g?blogID=7842737484277562285&postID=5759550357128128100
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
TSS
Prepared by National Center for Animal Health Programs Ruminant Health Programs Team November 15, 2007
snip...
Infected and Source Flocks
During FY 2007, there were a total of 76 new infected or source flocks identified. Of those new flocks identified, 30 were infected flocks and 46 were source flocks (Figure 2). As of September 30, 2007, there were 38 scrapie infected and source flocks with open statuses (Figure 3). ...
snip...
In FY 2007, 331 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), including 59* Regulatory Scrapie Slaughter Surveillance (RSSS) cases (Figure 5 and Slide 16). In FY 2007, two field cases, one validation case, and two RSSS cases were consistent with Nor-98 scrapie. The Nor98-like cases originated from flocks in California, Minnesota, Colorado, Wyoming and Indiana respectively. Nineteen cases of scrapie in goats have been reported since 1990 (Figure 6). The last goat case was reported in September 2007.
snip...
see full report here ;
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/yearly_report.pps
P03.141
Aspects of the Cerebellar Neuropathology in Nor98
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E11National Veterinary Insitute, Sweden; 2National Veterinary Institute, Norway
Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was firstdescribed in Norway in 1998. Several features of Nor98 were shown to be differentfrom classical scrapie including the distribution of disease associated prion protein(PrPd) accumulation in the brain. The cerebellum is generally the most affected brainarea in Nor98. The study here presented aimed at adding information on theneuropathology in the cerebellum of Nor98 naturally affected sheep of variousgenotypes 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 histologicallesions and tissue reactions were evaluated. The types of PrPd deposition werecharacterized. The cerebellar cortex was regularly affected, even though there was avariation in the severity of the lesions from case to case. Neuropil vacuolation wasmore marked in the molecular layer, but affected also the granular cell layer. There wasa loss of granule cells. Punctate deposition of PrPd was characteristic. It wasmorphologically and in distribution identical with that of synaptophysin, suggestingthat PrPd accumulates in the synaptic structures. PrPd was also observed in thegranule cell layer and in the white matter. The pathology features of Nor98 in thecerebellum of the affected sheep showed similarities with those of sporadicCreutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007
typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=6997404&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVAbstractPlus
full text ;
http://nor-98.blogspot.com/
http://scrapie-usa.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease inthe United States
http://cjdusa.blogspot.com/
i am reminded of a few things deep throat (high ranking official at usda)told me years ago;
==========================================
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........Dear God,
https://www.blogger.com/comment.g?blogID=7842737484277562285&postID=5759550357128128100
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
TSS
Thursday, November 22, 2007
UPDATE ATYPICAL SCRAPIE NOR-98 RESEARCH ARTICLES
Research article
Experimental transmission of atypical scrapie to sheep
Marion M Simmons1 , Timm Konold1 , Hugh A Simmons3 , Yvonne I Spencer1 , Richard Lockey1 , John Spiropoulos1 , Sharon Everitt2 and Derek Clifford3
1Department of Pathology, Veterinary Laboratories Agency – Weybridge, Woodham Lane, New Haw, Addlestone, Surrey KT15 3NB, UK2Department of Molecular Pathogenesis and Genetics, Veterinary Laboratories Agency – Weybridge, Woodham Lane, New Haw, Addlestone, Surrey KT15 3NB, UK3Animal Services Unit, Veterinary Laboratories Agency – Weybridge, Woodham Lane, New Haw, Addlestone, Surrey KT15 3NB, UK
author email corresponding author email
BMC Veterinary Research 2007, 3:20doi:10.1186/1746-6148-3-20
The electronic version of this article is the complete one and can be found online at: http://www.biomedcentral.com/1746-6148/3/20
Received: 26 March 2007 Accepted: 28 August 2007 Published: 28 August 2007
© 2007 Crown copyright; licensee BioMed Central Ltd.This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
AbstractBackgroundActive surveillance for transmissible spongiform encephalopathies in small ruminants has been an EU regulatory requirement since 2002. A number of European countries have subsequently reported cases of atypical scrapie, similar to previously published cases from Norway, which have pathological and molecular features distinct from classical scrapie. Most cases have occurred singly in flocks, associated with genotypes considered to be more resistant to classical disease. Experimental transmissibility of such isolates has been reported in certain ovinised transgenic mice, but has not previously been reported in the natural host. Information on the transmissibility of this agent is vital to ensuring that disease control measures are effective and proportionate.
ResultsThis report presents the successful experimental transmission, in 378 days, of atypical scrapie to a recipient sheep of homologous genotype with preservation of the pathological and molecular characteristics of the donor. This isolate also transmitted to ovinised transgenic mice (Tg338) with a murine phenotype indistinguishable from that of Nor 98.
ConclusionThis result strengthens the opinion that these cases result from a distinct strain of scrapie agent, which is potentially transmissible in the natural host under field conditions.
Background
snip...
DiscussionTo our knowledge this is the first report of the successful experimental transmission of atypical scrapie within the natural host. The retention of the molecular and pathological characteristics of the scrapie agent following this experimental transmission supports the hypothesis that disease in the donor animal was caused by a stable strain of the TSE agent which has phenotypic characteristics distinct from those associated with classical scrapie. Although clinical signs were not recorded from the donor animal because it presented dead (fallen stock), the signs seen in the challenged animal were consistent with those reported for the small number of passive surveillance cases so far identified in the UK [14,15] and elsewhere [6,16], which were characterised by gait abnormalities, abnormal behaviour and a relative lack of pruritic signs.
The transmission characteristics of atypical scrapie from different countries in Tg338 mice [12] and emerging data from similar transmissions of the isolate in this report indicate that at least one strain of atypical scrapie compatible with/indistinguishable from Nor98 is widespread across Europe, despite its relatively recent identification.
Although it has now been demonstrated to be experimentally transmissible within and between species via the intracerebral route, there is little epidemiological evidence for ease of transmission of this strain under field conditions, where scrapie is most likely transmitted via the oral route. This raises the question of how it came to be so geographically widespread. One hypothesis is that this represents a spontaneous genetic disease in sheep similar to the familial forms of TSE in man (e.g. GSS, familial CJD and FFI etc.), in which the resultant disease can subsequently be transmitted experimentally [17,18].
Horizontal transmission of classical scrapie has been shown to occur in adult sheep exposed to an affected flock [19] and even in animals which have had contact only with a contaminated environment (GI Dexter and SJ Bellworthy, personal communication). The environmental persistence of the classical scrapie agent is also supported by epidemiological evidence from Icelandic repopulation studies [20,21]. However, the low molecular mass (<14 kD) band of PrPSc seen in Western blots of atypical isolates is associated with a reduction in PK resistance of this disease-associated protein. It is possible that this relative protease susceptibility may make the agent less robust in field conditions and thereby reduce the extent to which it is transmissible by natural routes, and so limit the number of cases. It could be argued that this in turn supports the hypothesis that the natural disease may arise spontaneously. However, although genetic studies have indicated a PrP genotype host range for atypical scrapie which, for the majority of cases, is almost fully distinct from that of the classical forms of disease, they have not identified a single genetic feature (such as the point mutations in some human forms [22]) which might account for this.
This relative PrPSc fragility [23] compared to the PrPSc in classical scrapie, may also account for the total absence of intracellular PrPSc immunolabelling. The different PK cleavage sites in PrP associated with different strains of TSE infecting small ruminants can be used to differentiate them, both in blots and by immunohistochemistry. Intracellular immunostaining is significantly reduced in sheep infected with BSE compared to classical scrapie, when the appropriate monoclonal antibodies are applied, and it has been speculated that this demonstrates a partial digestion of the PrPSc molecule by the endogenous proteases within the cells [24]. If the PrPSc is more protease sensitive, then this loss of intracellular immunolabelling even with an antibody to the core of the protein (such as MAb 2G11) might indicate that cells can more effectively catabolise this abnormal form of the protein than more 'classical' forms, and therefore succumb to clinical disease much later, if at all. This may also account for the apparent absence of disease-related PrPSc in lymphoid tissues. It is interesting to note that in familial CJD (E200K mutation) also, PrP labelling was seen as a uniformly fine deposit throughout all cortical layers [25].
This study confirms that 'atypical scrapie' is transmissible within the natural host species, via the intracerebral route, without alteration of the pathological and molecular characteristics. This is the first report from a larger study which will explore more widely the effect of the PrP gene on the experimental susceptibility and resultant phenotype following intracerebral or oral challenge with this type of scrapie isolate.
ConclusionAt present the significance of this result, in terms of the transmissibility or pathogenicity under 'field conditions' of this agent strain in any species remains speculative, but it supports the need for appropriate control measures protecting both the animal and the human food chain to encompass atypical scrapie cases specifically.
snip...END...TSS
http://www.biomedcentral.com/1746-6148/3/20
Tissue distribution. For atypical scrapie, what is PrPres andinfectivity distribution within sheep of different genotypes,particularly with respect to SRM removal? For classicalscrapie and experimental BSE in sheep, tissue distribution ofinfectivity is widespread. Thus, even with SRM controls inplace, an infected sheep poses around 1000 times the risk tohuman health than does an infected cow22. Does thedistribution depend on whether infection is by the oral or21 Gubbins S. Prevalence of BSE in sheep: interpreting the results ofretrospective andprospective testing of sheep TSE cases. SEAC 84 open meeting22 paper presented to Food Standards Agency board on 9 December 2004.http://www.foodstandards.gov.uk/multimedia/pdfs/fsa041204.pdfAlso see paper SEAC/84/2 Annex 2: McLean, A.Page 13© SEAC 27 February 2006intracerebral route? Are some VRQ sheep carriers with noneurological symptoms?
SEAC SHEEP SUBGROUPPOSITION STATEMENT
http://www.seac.gov.uk/pdf/positionstatement-sheep-subgroup.pdf
Published online before print October 20, 2005Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102Medical Sciences
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes
( sheep prion transgenic mice )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 **Virologie Immunologie Moléculaires and Génétique Biochimique etCytogénétique, Institut National de la Recherche Agronomique, 78350Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de laRecherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, InteractionsHôte Agent Pathogène, 31066 Toulouse, France; Agence Française de SécuritéSanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels,69364 Lyon, France; **Pathologie Infectieuse et Immunologie, InstitutNational 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,and approved September 12, 2005 (received for review March 21, 2005)
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 amisfolded 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 tothe 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 goatflocks andmay have important implications in terms of scrapie control andpublic health.
--------------------------------------------------------------------------------
Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R.,T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B.contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data;and H.L. wrote the paper.A.L.D. and V.B. contributed equally to this work.To whom correspondence should be addressed.Hubert Laude,
E-mail: laude@jouy.inra.fr
www.pnas.org/cgi/doi/10.1073/pnas.0502296102
http://www.pnas.org/cgi/content/abstract/0502296102v1
OF COURSE, the USDA et al once was concerned for human health from typical scrapie, and rightly so, typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
http://www.ncbi.nlm.nih.gov/entrez/query.fcgicmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
ALSO, The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate.
http://www.pnas.org/cgi/content/full/041490898v1
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
Although most atypical cases occur singly in flocks, there are some instances where two affected sheep have been identified in flocks. This may indicate that natural transmission may occur, or that the sheep were infected from a common alternative source(22, 29). Possible indications of an association with the feeding of vitamins and mineral feed supplements were detected in Norway, but remain to be proven(22).
snip...
Atypical BSE may arise spontaneously in a small proportion of cattle. The existence of sporadic CJD in humans has led to postulation that disease could arise spontaneously in any animal, but this is still not proven to happen. Despitethe small numbers of atypical BSE detected so far, in some countries the numbers are too great to suggest that they all arise spontaneously, if it were assumed that such a phenomenon occurred at the same frequency as sporadic CJD in humans.
http://www.tseandfoodsafety.org/position_papers/TAFS_POSITION_PAPER_ON_ATYPICAL_SCRAPIE_AND_%20ATYPICAL_BSE_070516.pdf
TSS
Experimental transmission of atypical scrapie to sheep
Marion M Simmons1 , Timm Konold1 , Hugh A Simmons3 , Yvonne I Spencer1 , Richard Lockey1 , John Spiropoulos1 , Sharon Everitt2 and Derek Clifford3
1Department of Pathology, Veterinary Laboratories Agency – Weybridge, Woodham Lane, New Haw, Addlestone, Surrey KT15 3NB, UK2Department of Molecular Pathogenesis and Genetics, Veterinary Laboratories Agency – Weybridge, Woodham Lane, New Haw, Addlestone, Surrey KT15 3NB, UK3Animal Services Unit, Veterinary Laboratories Agency – Weybridge, Woodham Lane, New Haw, Addlestone, Surrey KT15 3NB, UK
author email corresponding author email
BMC Veterinary Research 2007, 3:20doi:10.1186/1746-6148-3-20
The electronic version of this article is the complete one and can be found online at: http://www.biomedcentral.com/1746-6148/3/20
Received: 26 March 2007 Accepted: 28 August 2007 Published: 28 August 2007
© 2007 Crown copyright; licensee BioMed Central Ltd.This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
AbstractBackgroundActive surveillance for transmissible spongiform encephalopathies in small ruminants has been an EU regulatory requirement since 2002. A number of European countries have subsequently reported cases of atypical scrapie, similar to previously published cases from Norway, which have pathological and molecular features distinct from classical scrapie. Most cases have occurred singly in flocks, associated with genotypes considered to be more resistant to classical disease. Experimental transmissibility of such isolates has been reported in certain ovinised transgenic mice, but has not previously been reported in the natural host. Information on the transmissibility of this agent is vital to ensuring that disease control measures are effective and proportionate.
ResultsThis report presents the successful experimental transmission, in 378 days, of atypical scrapie to a recipient sheep of homologous genotype with preservation of the pathological and molecular characteristics of the donor. This isolate also transmitted to ovinised transgenic mice (Tg338) with a murine phenotype indistinguishable from that of Nor 98.
ConclusionThis result strengthens the opinion that these cases result from a distinct strain of scrapie agent, which is potentially transmissible in the natural host under field conditions.
Background
snip...
DiscussionTo our knowledge this is the first report of the successful experimental transmission of atypical scrapie within the natural host. The retention of the molecular and pathological characteristics of the scrapie agent following this experimental transmission supports the hypothesis that disease in the donor animal was caused by a stable strain of the TSE agent which has phenotypic characteristics distinct from those associated with classical scrapie. Although clinical signs were not recorded from the donor animal because it presented dead (fallen stock), the signs seen in the challenged animal were consistent with those reported for the small number of passive surveillance cases so far identified in the UK [14,15] and elsewhere [6,16], which were characterised by gait abnormalities, abnormal behaviour and a relative lack of pruritic signs.
The transmission characteristics of atypical scrapie from different countries in Tg338 mice [12] and emerging data from similar transmissions of the isolate in this report indicate that at least one strain of atypical scrapie compatible with/indistinguishable from Nor98 is widespread across Europe, despite its relatively recent identification.
Although it has now been demonstrated to be experimentally transmissible within and between species via the intracerebral route, there is little epidemiological evidence for ease of transmission of this strain under field conditions, where scrapie is most likely transmitted via the oral route. This raises the question of how it came to be so geographically widespread. One hypothesis is that this represents a spontaneous genetic disease in sheep similar to the familial forms of TSE in man (e.g. GSS, familial CJD and FFI etc.), in which the resultant disease can subsequently be transmitted experimentally [17,18].
Horizontal transmission of classical scrapie has been shown to occur in adult sheep exposed to an affected flock [19] and even in animals which have had contact only with a contaminated environment (GI Dexter and SJ Bellworthy, personal communication). The environmental persistence of the classical scrapie agent is also supported by epidemiological evidence from Icelandic repopulation studies [20,21]. However, the low molecular mass (<14 kD) band of PrPSc seen in Western blots of atypical isolates is associated with a reduction in PK resistance of this disease-associated protein. It is possible that this relative protease susceptibility may make the agent less robust in field conditions and thereby reduce the extent to which it is transmissible by natural routes, and so limit the number of cases. It could be argued that this in turn supports the hypothesis that the natural disease may arise spontaneously. However, although genetic studies have indicated a PrP genotype host range for atypical scrapie which, for the majority of cases, is almost fully distinct from that of the classical forms of disease, they have not identified a single genetic feature (such as the point mutations in some human forms [22]) which might account for this.
This relative PrPSc fragility [23] compared to the PrPSc in classical scrapie, may also account for the total absence of intracellular PrPSc immunolabelling. The different PK cleavage sites in PrP associated with different strains of TSE infecting small ruminants can be used to differentiate them, both in blots and by immunohistochemistry. Intracellular immunostaining is significantly reduced in sheep infected with BSE compared to classical scrapie, when the appropriate monoclonal antibodies are applied, and it has been speculated that this demonstrates a partial digestion of the PrPSc molecule by the endogenous proteases within the cells [24]. If the PrPSc is more protease sensitive, then this loss of intracellular immunolabelling even with an antibody to the core of the protein (such as MAb 2G11) might indicate that cells can more effectively catabolise this abnormal form of the protein than more 'classical' forms, and therefore succumb to clinical disease much later, if at all. This may also account for the apparent absence of disease-related PrPSc in lymphoid tissues. It is interesting to note that in familial CJD (E200K mutation) also, PrP labelling was seen as a uniformly fine deposit throughout all cortical layers [25].
This study confirms that 'atypical scrapie' is transmissible within the natural host species, via the intracerebral route, without alteration of the pathological and molecular characteristics. This is the first report from a larger study which will explore more widely the effect of the PrP gene on the experimental susceptibility and resultant phenotype following intracerebral or oral challenge with this type of scrapie isolate.
ConclusionAt present the significance of this result, in terms of the transmissibility or pathogenicity under 'field conditions' of this agent strain in any species remains speculative, but it supports the need for appropriate control measures protecting both the animal and the human food chain to encompass atypical scrapie cases specifically.
snip...END...TSS
http://www.biomedcentral.com/1746-6148/3/20
Tissue distribution. For atypical scrapie, what is PrPres andinfectivity distribution within sheep of different genotypes,particularly with respect to SRM removal? For classicalscrapie and experimental BSE in sheep, tissue distribution ofinfectivity is widespread. Thus, even with SRM controls inplace, an infected sheep poses around 1000 times the risk tohuman health than does an infected cow22. Does thedistribution depend on whether infection is by the oral or21 Gubbins S. Prevalence of BSE in sheep: interpreting the results ofretrospective andprospective testing of sheep TSE cases. SEAC 84 open meeting22 paper presented to Food Standards Agency board on 9 December 2004.http://www.foodstandards.gov.uk/multimedia/pdfs/fsa041204.pdfAlso see paper SEAC/84/2 Annex 2: McLean, A.Page 13© SEAC 27 February 2006intracerebral route? Are some VRQ sheep carriers with noneurological symptoms?
SEAC SHEEP SUBGROUPPOSITION STATEMENT
http://www.seac.gov.uk/pdf/positionstatement-sheep-subgroup.pdf
Published online before print October 20, 2005Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102Medical Sciences
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes
( sheep prion transgenic mice )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 **Virologie Immunologie Moléculaires and Génétique Biochimique etCytogénétique, Institut National de la Recherche Agronomique, 78350Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de laRecherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, InteractionsHôte Agent Pathogène, 31066 Toulouse, France; Agence Française de SécuritéSanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels,69364 Lyon, France; **Pathologie Infectieuse et Immunologie, InstitutNational 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,and approved September 12, 2005 (received for review March 21, 2005)
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 amisfolded 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 tothe 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 goatflocks andmay have important implications in terms of scrapie control andpublic health.
--------------------------------------------------------------------------------
Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R.,T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B.contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data;and H.L. wrote the paper.A.L.D. and V.B. contributed equally to this work.To whom correspondence should be addressed.Hubert Laude,
E-mail: laude@jouy.inra.fr
www.pnas.org/cgi/doi/10.1073/pnas.0502296102
http://www.pnas.org/cgi/content/abstract/0502296102v1
OF COURSE, the USDA et al once was concerned for human health from typical scrapie, and rightly so, typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
http://www.ncbi.nlm.nih.gov/entrez/query.fcgicmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
ALSO, The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate.
http://www.pnas.org/cgi/content/full/041490898v1
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
Although most atypical cases occur singly in flocks, there are some instances where two affected sheep have been identified in flocks. This may indicate that natural transmission may occur, or that the sheep were infected from a common alternative source(22, 29). Possible indications of an association with the feeding of vitamins and mineral feed supplements were detected in Norway, but remain to be proven(22).
snip...
Atypical BSE may arise spontaneously in a small proportion of cattle. The existence of sporadic CJD in humans has led to postulation that disease could arise spontaneously in any animal, but this is still not proven to happen. Despitethe small numbers of atypical BSE detected so far, in some countries the numbers are too great to suggest that they all arise spontaneously, if it were assumed that such a phenomenon occurred at the same frequency as sporadic CJD in humans.
http://www.tseandfoodsafety.org/position_papers/TAFS_POSITION_PAPER_ON_ATYPICAL_SCRAPIE_AND_%20ATYPICAL_BSE_070516.pdf
TSS
Tuesday, October 9, 2007
NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 RISES TO 5 DOCUMENTED CASES
Thursday, November 01, 2007 6:38 AM UPDATE
ATYPICAL NOR-98 SCRAPIE LOCATION UPDATE ON 5 DOCUMENTED CASES THIS YEAR ;
The flocks of origin are WY, CO, CA, IN, and MN.
personal communication USDA et al. ...TSS
USA NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 RISES TO 5 DOCUMENTED CASES
http://nor-98.blogspot.com/
Government Accountability Project
https://www.blogger.com/comment.g?blogID=3995372399492420922&postID=295754279213239559
TSS
Subject: NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 (RISES TO 5 DOCUMENTED CASES IN USA)
Date: October 9, 2007 at 7:15 am PST
Greetings, seems the NOR-98 atypical scrapie cases are the rise in the USA. ...tss
INFECTED AND SOURCE FLOCKS AS of August 31, 2007, there were 33 scrapie infected and source flocks with open statuses (Figure 3). Five new source flocks and one new infected flock were reported n August (Figure 4) with a total of 64 reported for FY 2007(Figure 5).
snip...
IN FY 2007 TWO FIELD CASES, ONE VALIDATION CASE, AND TWO RSSS CASES WERE CONSISTENT WITH NOR-98 SCRAPIE. ...
(BRINGS A TOTAL OF 5 NOR-98 CASES DOCUMENTED IN 2007 IN USA. ...TSS)
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
please note sporadic cjd cases on the rise with 'unknown type' increasing drastically. ...tss
NOW, why in the world is no one much speaking about the lates 3 cases of theNOR-98 case in the USA, and what are the potential ramifications thereof;
Subject: Aspects of the Cerebellar Neuropathology in Nor98
Date: September26, 2007 at 4:06 pm PST
P03.141
Aspects of the Cerebellar Neuropathology in Nor98
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1NationalVeterinary 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 studyhere presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Swedenand 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 thegranule 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.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
what does the Nobel Prize Winner Stanely Prusiner say about atypical scrapie, lets look at that first ;
Published online before print October 20, 2005Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102Medical Sciences
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes
( sheep prion transgenic mice )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 **Virologie Immunologie Moléculaires and Génétique Biochimique etCytogénétique, Institut National de la Recherche Agronomique, 78350Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de laRecherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, InteractionsHôte Agent Pathogène, 31066 Toulouse, France; Agence Française de SécuritéSanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels,69364 Lyon, France; **Pathologie Infectieuse et Immunologie, InstitutNational 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,and approved September 12, 2005 (received for review March 21, 2005)
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 amisfolded 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 tothe 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 goatflocks and
may have important implications in terms of scrapie control andpublic health.
--------------------------------------------------------------------------------
Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R.,T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B.contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data;and H.L. wrote the paper.A.L.D. and V.B. contributed equally to this work.To whom correspondence should be addressed.Hubert Laude,
E-mail: laude@jouy.inra.fr
www.pnas.org/cgi/doi/10.1073/pnas.0502296102
http://www.pnas.org/cgi/content/abstract/0502296102v1
OF COURSE, the USDA et al once was concerned for human health from typical scrapie, and rightly so, typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
J Infect Dis. 2004 Aug 1;190(3):653-60.
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.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=6997404&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVAbstractPlus
ALSO, The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate.
http://www.pnas.org/cgi/content/full/041490898v1
SO, in my opinion, the _myth_ that typical scrapie does not transmit to humans, is just that, a myth. there have never been transmission studies done on man, and I would bet my last bottom dollar, anyone making the statement, would not eat a handful of scrapie infected brains. you do know about james alford don't you, whether or not it was the sheep brains he was fed in the middle east, or something else, this war hero has cjd, and he is very young. this was a tragic story too. dont hear much of that now either. ...
http://www.blackfive.net/main/2004/10/a_dying_hero_ss.html
http://www.blackfive.net/main/2004/03/ssg_james_alfor.html
PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increase in
''TYPE UNKNOWN''. ...TSS
1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17 inconclusive and 9 pending (1 from 2006, 8from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006, 26 from 2007)
http://www.cjdsurveillance.com/pdf/case-table.pdf
TSS
From: "Terry S. Singeltary Sr."
Sent: Tuesday, August 21, 2007 9:50 AM
Subject: TWO MORE Nor98 atypical Scrapie cases detected in USA bringingtotal to 3 cases to date
Infected and Source Flocks As of June 30, 2007, there were .....
snip...
One field case and one validation case were consistent with Nor-98 scrapie.
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
IN the February 2007 Scrapie report it only mentions; ''One case was consistent with Nor98 scrapie.''
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/
(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS USDA, APHIS, VS ET AL. ...TSS)
NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=14553
An evaluation of scrapie surveillance in the United States
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427
FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=10451
NEW SCIENTIST MAGAZINE 4/02/01
NEW SCIENTIST EDITORIAL PAGE 3MAD SHEEP DISEASE?
IF THERE is one categorical pronouncement you can safely make about prion diseases like BSE or CJD, it is that one should not make categorical pronouncements. "British beef is safe" and "there is no BSE in Germany" come to mind. Now there are two more: "scrapie is safe", and "people don't catch sporadic CJD". Scrapie is the most widespread prion disease, infecting untold numbers of sheep worldwide. Sporadic CJD is theold-fashioned pre-BSE kind that is supposed to happen spontaneously in unlucky people. But a surprise observation in France suggests some sCJD cases--though by no means all--maybe linked to scrapie after all (see p 4). For years, British authorities asserted that BSE was harmless because it was a form of scrapie. In fact, the only evidence scrapie is safe is some broad-brush epidemiology, good as far as it goes but unable to reveal occasional risks for some people from some sheep. Alarm bells should have rung in 1980 when researchers gave monkeys scrapie by feeding them infected brains. But that research, like so much other work on prion diseases, was never followed up. We still have little idea what BSE does in pigs and chickens. The Queniborough vCJD outbreak (see p 5) would be easier to understand if we knew how much brain we must eat to be infected. As for scrapie, it shouldn't take a chance finding to tell us that there may be dangerous sheep out there.
Suspect symptoms
What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?
Exclusive from New Scientist magazine
Four years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease.................
full text url follows
By Debora MacKenzie
Suspect Symptoms
http://www.newscientist.com/channel/health/bse/mg16922840.300
if url dead, go here for 'SUSPECT SYMPTOMS'
you can access article here also;
http://www.mad-cow.org/UKCJD/CJD_news52.html#29%20Mar%2001%20-%20CJD%20-%20Suspect%20symptoms
http://www.organicconsumers.org/meat/scrapiecjd.cfm
Then follow up with PNAS studies from which new scientist article written from;
Published online before print March 20, 2001
Proc. Natl. Acad. Sci. USA, 10.1073/pnas.041490898
Abstract of this ArticleReprint (PDF) Version of this ArticleSimilar articles found in:
PNAS OnlinePubMedPubMed CitationSearch Medline for articles by:
Lasmézas, C. I. Deslys, J.-P.Alert me when: new articles cite this article Download to Citation Manager Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates andcomparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Virginie Nouvel*,Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [] , DominiqueDormont*, and Jean-Philippe Deslys** Commissariat à l'Energie Atomique, Service de Neurovirologie,Direction des Sciences du Vivant/Département de Recherche Medicale,Centre de Recherches du Service de Santé des Armées 60-68, Avenue duGénéral Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; [Dagger] Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003Lyon, France; § Laboratoire de Neuropathologie, Hôpital de laSalpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital,Crewe Road, Edinburgh EH4 2XU, United Kingdom; and [] Institute forAnimal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH93JF, United KingdomEdited by D. Carleton Gajdusek, Centre National de la RechercheScientifique, Gif-sur-Yvette, France, and approved December 7, 2000(received for review October 16, 2000)
Abstract
There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.
Introduction
The recognition of a variant of the human transmissible spongiform encephalopathy (TSE) Creutzfeldt-Jakob Disease (vCJD) in the U.K. in 1996 raised the major concern that it would correspond to human infection with the agent responsible for bovine spongiform encephalopathy (BSE; ref. 1). Transmission of BSE to macaques provided the first experimental evidence as it produced a disease close to vCJD in humans (2). Strain typing in inbred mice (consisting of measuring the incubation period and establishing lesion profiles corresponding to the strain-specific distribution of brain vacuolation) allows reliable identification of TSE strains (3). This method, together with biochemical methods, has revealed a single phenotype for the agents ofBSE and the British cases of vCJD (4-6). Mice expressing only the bovine prion protein (PrP) were highly susceptible to vCJD and BSE, which induced the same disease (7). Thus, it is now well established that BSE has caused vCJD, probably by alimentary contamination. In this respect,the finding of abnormal PrP labeling in the gastrointestinal tract and lymphatic tissues of orally BSE-contaminated lemurs shows that the BSE agent can infect primates by the oral route (8). About 1 million contaminated cattle may have entered the human food chain, and thefuture number of vCJD cases could range from 63 to 136,000 depending on the incubation period of BSE in humans (9). Unlike sporadic CJD (sCJD) and iatrogenic CJD (iCJD) linked to the administration of contaminated growth hormone extracted from human hypophyses, in vCJD, the infectious agent seems to be widely distributed in lymphoid organs, as pathological PrP (PrPres) can be detected in tonsils, lymph nodes, spleen, and appendix even in the preclinical phase of the disease (10, 11). This raises a public health issue with regard to the risk of iatrogenic transmission of vCJD through surgical instruments, grafts, blood transfusion, or parenteral administration of biological products of human origin. However, this risk is difficult to assess, because it largely depends on factors such as the virulence of the BSE agent adapted to primates and the efficiency of secondary transmission to humans by a peripheral route such as the i.v. one. A further issue is whether vCJD accidentally acquired from humans would be recognized. The latter poses the question of a phenotypic variation of the BSE agentafter successive transmissions in humans: does it retain its strain characteristics, and does it induce a pathology similar to that observed in the previous host? A 9-year history of transmission of BSE to primates and mice enables us today to clarify a number of these important points. Although BSE has mainly affected the U.K., two definite cases and one probable case of vCJD have now been reported in France in people who have never resided in the U.K. (12, 13). We strain-typed the first of these cases to establish its origin. Strain typing in C57BL/6 mice of BSE, French, and British vCJD was compared with that of BSE passaged in nonhuman primates, thus allowing us to study the effect of serial passages in primates. Comparisons were also made with French cases of sCJD and iCJD and two strains of scrapie (one of French and one of U.S. origin).
Our findings provide experimental demonstration that the same agent, namely that responsible for the cattle disease BSE, has causedvCJD both in France and in the U.K., in line with biochemical data and with the fact that, until 1996, about 10% of the beef consumed in France was imported from the U.K. We found that the BSE agent in nonhuman primates is similar to that causing vCJD in humans and tends to evolve rapidly toward a primate-adapted variant.
Furthermore, we showed that the strain responsible for iCJD is closely related to that of one patient with sCJD, and, more unexpectedly, that these agents were similar to the French scrapie strain studied (but different from theU.S. scrapie strain). This finding requires a cautious interpretation for several reasons, not least because of the inevitably limited number of TSE strains that can be studied by such a cumbersome method as straintyping. Nonetheless, it also prompts reconsideration of the possibility that, in some instances, sheep and human TSEs can share a common origin.
snip...
http://www.pnas.org/cgi/content/full/041490898v1
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 ScrapieA]
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 is 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 duringthe 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 ofAgriculture 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 one 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 scrapie problem urgent if the sheep industry is not to suffer grievously.
snip...
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Subject: FSIS NOTICE SAMPLE COLLECTION FROM CATTLE UNDER THE BOVINESPONGIFORM ENCEPHALOPATHY (BSE)ONGOING SURVEILLANCE PROGRAMFrom: "Terry S. Singeltary Sr."Reply-To: Sustainable Agriculture Network Discussion GroupDate: Fri, 2 Feb 2007 17:32:58 -0600
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&P=720
ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007 Date: Mon, 24 Sep 2007 21:31:55 -0500
I suggest that you all read the data out about h-BASE and sporadic CJD, GSS,blood, and some of the other abstracts from the PRION2007. ...
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744
*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!!
THE PRICE OF POKER INDEED GOES UP. ...TSS
USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 200711:52 PM. ...TSS
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779
see full text 143 pages ;
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
ATYPICAL NOR-98 SCRAPIE LOCATION UPDATE ON 5 DOCUMENTED CASES THIS YEAR ;
The flocks of origin are WY, CO, CA, IN, and MN.
personal communication USDA et al. ...TSS
USA NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 RISES TO 5 DOCUMENTED CASES
http://nor-98.blogspot.com/
Government Accountability Project
https://www.blogger.com/comment.g?blogID=3995372399492420922&postID=295754279213239559
TSS
Subject: NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 (RISES TO 5 DOCUMENTED CASES IN USA)
Date: October 9, 2007 at 7:15 am PST
Greetings, seems the NOR-98 atypical scrapie cases are the rise in the USA. ...tss
INFECTED AND SOURCE FLOCKS AS of August 31, 2007, there were 33 scrapie infected and source flocks with open statuses (Figure 3). Five new source flocks and one new infected flock were reported n August (Figure 4) with a total of 64 reported for FY 2007(Figure 5).
snip...
IN FY 2007 TWO FIELD CASES, ONE VALIDATION CASE, AND TWO RSSS CASES WERE CONSISTENT WITH NOR-98 SCRAPIE. ...
(BRINGS A TOTAL OF 5 NOR-98 CASES DOCUMENTED IN 2007 IN USA. ...TSS)
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
please note sporadic cjd cases on the rise with 'unknown type' increasing drastically. ...tss
NOW, why in the world is no one much speaking about the lates 3 cases of theNOR-98 case in the USA, and what are the potential ramifications thereof;
Subject: Aspects of the Cerebellar Neuropathology in Nor98
Date: September26, 2007 at 4:06 pm PST
P03.141
Aspects of the Cerebellar Neuropathology in Nor98
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1NationalVeterinary 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 studyhere presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Swedenand 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 thegranule 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.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
what does the Nobel Prize Winner Stanely Prusiner say about atypical scrapie, lets look at that first ;
Published online before print October 20, 2005Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102Medical Sciences
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes
( sheep prion transgenic mice )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 **Virologie Immunologie Moléculaires and Génétique Biochimique etCytogénétique, Institut National de la Recherche Agronomique, 78350Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de laRecherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, InteractionsHôte Agent Pathogène, 31066 Toulouse, France; Agence Française de SécuritéSanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels,69364 Lyon, France; **Pathologie Infectieuse et Immunologie, InstitutNational 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,and approved September 12, 2005 (received for review March 21, 2005)
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 amisfolded 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 tothe 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 goatflocks and
may have important implications in terms of scrapie control andpublic health.
--------------------------------------------------------------------------------
Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R.,T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B.contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data;and H.L. wrote the paper.A.L.D. and V.B. contributed equally to this work.To whom correspondence should be addressed.Hubert Laude,
E-mail: laude@jouy.inra.fr
www.pnas.org/cgi/doi/10.1073/pnas.0502296102
http://www.pnas.org/cgi/content/abstract/0502296102v1
OF COURSE, the USDA et al once was concerned for human health from typical scrapie, and rightly so, typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
J Infect Dis. 2004 Aug 1;190(3):653-60.
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.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=6997404&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVAbstractPlus
ALSO, The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate.
http://www.pnas.org/cgi/content/full/041490898v1
SO, in my opinion, the _myth_ that typical scrapie does not transmit to humans, is just that, a myth. there have never been transmission studies done on man, and I would bet my last bottom dollar, anyone making the statement, would not eat a handful of scrapie infected brains. you do know about james alford don't you, whether or not it was the sheep brains he was fed in the middle east, or something else, this war hero has cjd, and he is very young. this was a tragic story too. dont hear much of that now either. ...
http://www.blackfive.net/main/2004/10/a_dying_hero_ss.html
http://www.blackfive.net/main/2004/03/ssg_james_alfor.html
PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increase in
''TYPE UNKNOWN''. ...TSS
1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17 inconclusive and 9 pending (1 from 2006, 8from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006, 26 from 2007)
http://www.cjdsurveillance.com/pdf/case-table.pdf
TSS
From: "Terry S. Singeltary Sr."
Sent: Tuesday, August 21, 2007 9:50 AM
Subject: TWO MORE Nor98 atypical Scrapie cases detected in USA bringingtotal to 3 cases to date
Infected and Source Flocks As of June 30, 2007, there were .....
snip...
One field case and one validation case were consistent with Nor-98 scrapie.
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
IN the February 2007 Scrapie report it only mentions; ''One case was consistent with Nor98 scrapie.''
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/
(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS USDA, APHIS, VS ET AL. ...TSS)
NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=14553
An evaluation of scrapie surveillance in the United States
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427
FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=10451
NEW SCIENTIST MAGAZINE 4/02/01
NEW SCIENTIST EDITORIAL PAGE 3MAD SHEEP DISEASE?
IF THERE is one categorical pronouncement you can safely make about prion diseases like BSE or CJD, it is that one should not make categorical pronouncements. "British beef is safe" and "there is no BSE in Germany" come to mind. Now there are two more: "scrapie is safe", and "people don't catch sporadic CJD". Scrapie is the most widespread prion disease, infecting untold numbers of sheep worldwide. Sporadic CJD is theold-fashioned pre-BSE kind that is supposed to happen spontaneously in unlucky people. But a surprise observation in France suggests some sCJD cases--though by no means all--maybe linked to scrapie after all (see p 4). For years, British authorities asserted that BSE was harmless because it was a form of scrapie. In fact, the only evidence scrapie is safe is some broad-brush epidemiology, good as far as it goes but unable to reveal occasional risks for some people from some sheep. Alarm bells should have rung in 1980 when researchers gave monkeys scrapie by feeding them infected brains. But that research, like so much other work on prion diseases, was never followed up. We still have little idea what BSE does in pigs and chickens. The Queniborough vCJD outbreak (see p 5) would be easier to understand if we knew how much brain we must eat to be infected. As for scrapie, it shouldn't take a chance finding to tell us that there may be dangerous sheep out there.
Suspect symptoms
What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?
Exclusive from New Scientist magazine
Four years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease.................
full text url follows
By Debora MacKenzie
Suspect Symptoms
http://www.newscientist.com/channel/health/bse/mg16922840.300
if url dead, go here for 'SUSPECT SYMPTOMS'
you can access article here also;
http://www.mad-cow.org/UKCJD/CJD_news52.html#29%20Mar%2001%20-%20CJD%20-%20Suspect%20symptoms
http://www.organicconsumers.org/meat/scrapiecjd.cfm
Then follow up with PNAS studies from which new scientist article written from;
Published online before print March 20, 2001
Proc. Natl. Acad. Sci. USA, 10.1073/pnas.041490898
Abstract of this ArticleReprint (PDF) Version of this ArticleSimilar articles found in:
PNAS OnlinePubMedPubMed CitationSearch Medline for articles by:
Lasmézas, C. I. Deslys, J.-P.Alert me when: new articles cite this article Download to Citation Manager Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates andcomparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Virginie Nouvel*,Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [] , DominiqueDormont*, and Jean-Philippe Deslys** Commissariat à l'Energie Atomique, Service de Neurovirologie,Direction des Sciences du Vivant/Département de Recherche Medicale,Centre de Recherches du Service de Santé des Armées 60-68, Avenue duGénéral Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; [Dagger] Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003Lyon, France; § Laboratoire de Neuropathologie, Hôpital de laSalpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital,Crewe Road, Edinburgh EH4 2XU, United Kingdom; and [] Institute forAnimal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH93JF, United KingdomEdited by D. Carleton Gajdusek, Centre National de la RechercheScientifique, Gif-sur-Yvette, France, and approved December 7, 2000(received for review October 16, 2000)
Abstract
There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.
Introduction
The recognition of a variant of the human transmissible spongiform encephalopathy (TSE) Creutzfeldt-Jakob Disease (vCJD) in the U.K. in 1996 raised the major concern that it would correspond to human infection with the agent responsible for bovine spongiform encephalopathy (BSE; ref. 1). Transmission of BSE to macaques provided the first experimental evidence as it produced a disease close to vCJD in humans (2). Strain typing in inbred mice (consisting of measuring the incubation period and establishing lesion profiles corresponding to the strain-specific distribution of brain vacuolation) allows reliable identification of TSE strains (3). This method, together with biochemical methods, has revealed a single phenotype for the agents ofBSE and the British cases of vCJD (4-6). Mice expressing only the bovine prion protein (PrP) were highly susceptible to vCJD and BSE, which induced the same disease (7). Thus, it is now well established that BSE has caused vCJD, probably by alimentary contamination. In this respect,the finding of abnormal PrP labeling in the gastrointestinal tract and lymphatic tissues of orally BSE-contaminated lemurs shows that the BSE agent can infect primates by the oral route (8). About 1 million contaminated cattle may have entered the human food chain, and thefuture number of vCJD cases could range from 63 to 136,000 depending on the incubation period of BSE in humans (9). Unlike sporadic CJD (sCJD) and iatrogenic CJD (iCJD) linked to the administration of contaminated growth hormone extracted from human hypophyses, in vCJD, the infectious agent seems to be widely distributed in lymphoid organs, as pathological PrP (PrPres) can be detected in tonsils, lymph nodes, spleen, and appendix even in the preclinical phase of the disease (10, 11). This raises a public health issue with regard to the risk of iatrogenic transmission of vCJD through surgical instruments, grafts, blood transfusion, or parenteral administration of biological products of human origin. However, this risk is difficult to assess, because it largely depends on factors such as the virulence of the BSE agent adapted to primates and the efficiency of secondary transmission to humans by a peripheral route such as the i.v. one. A further issue is whether vCJD accidentally acquired from humans would be recognized. The latter poses the question of a phenotypic variation of the BSE agentafter successive transmissions in humans: does it retain its strain characteristics, and does it induce a pathology similar to that observed in the previous host? A 9-year history of transmission of BSE to primates and mice enables us today to clarify a number of these important points. Although BSE has mainly affected the U.K., two definite cases and one probable case of vCJD have now been reported in France in people who have never resided in the U.K. (12, 13). We strain-typed the first of these cases to establish its origin. Strain typing in C57BL/6 mice of BSE, French, and British vCJD was compared with that of BSE passaged in nonhuman primates, thus allowing us to study the effect of serial passages in primates. Comparisons were also made with French cases of sCJD and iCJD and two strains of scrapie (one of French and one of U.S. origin).
Our findings provide experimental demonstration that the same agent, namely that responsible for the cattle disease BSE, has causedvCJD both in France and in the U.K., in line with biochemical data and with the fact that, until 1996, about 10% of the beef consumed in France was imported from the U.K. We found that the BSE agent in nonhuman primates is similar to that causing vCJD in humans and tends to evolve rapidly toward a primate-adapted variant.
Furthermore, we showed that the strain responsible for iCJD is closely related to that of one patient with sCJD, and, more unexpectedly, that these agents were similar to the French scrapie strain studied (but different from theU.S. scrapie strain). This finding requires a cautious interpretation for several reasons, not least because of the inevitably limited number of TSE strains that can be studied by such a cumbersome method as straintyping. Nonetheless, it also prompts reconsideration of the possibility that, in some instances, sheep and human TSEs can share a common origin.
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http://www.pnas.org/cgi/content/full/041490898v1
12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note CHAIRMAN: PROFESSOR PETER WILDY
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A The Present Position with respect to ScrapieA]
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 is 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 duringthe 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 ofAgriculture 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 one 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 scrapie problem urgent if the sheep industry is not to suffer grievously.
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76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Subject: FSIS NOTICE SAMPLE COLLECTION FROM CATTLE UNDER THE BOVINESPONGIFORM ENCEPHALOPATHY (BSE)ONGOING SURVEILLANCE PROGRAMFrom: "Terry S. Singeltary Sr."Reply-To: Sustainable Agriculture Network Discussion GroupDate: Fri, 2 Feb 2007 17:32:58 -0600
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&P=720
ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007 Date: Mon, 24 Sep 2007 21:31:55 -0500
I suggest that you all read the data out about h-BASE and sporadic CJD, GSS,blood, and some of the other abstracts from the PRION2007. ...
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744
*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!!
THE PRICE OF POKER INDEED GOES UP. ...TSS
USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 200711:52 PM. ...TSS
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779
see full text 143 pages ;
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
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