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From: TSS ()
Subject: UNITED STATES DOCUMENTS FIRST CASE RARE NOR98 SCRAPIE STRAIN
Date: March 17, 2007 at 7:31 pm PST

Rare form of scrapie found in sheep
By Gazette News Services

CHEYENNE - The Wyoming Livestock Board says a sheep from a flock in the northeastern corner of the state has tested positive for a rare form of the disease scrapie, the first time the particular strain has been found in the United States.

The USDA Animal Plant Health Inspection Service notified the state Friday that the ewe tested positive for a form of scrapie consistent with the Nor98 strain, first diagnosed in Norway in 1998.

The livestock board said it doesn't expect the strain of the disease to become a major problem for the Wyoming sheep industry. State and federal officials intend to monitor the remainder of the flock, near the Black Hills, to make sure the disease doesn't become established.

According to a release from the livestock board, the ewe was slaughtered in Michigan as part of the USDA's regular scrapie slaughter surveillance program and traced back to the Wyoming flock.

The release states that the Nor98 strain of scrapie is rare even in Europe, with fewer than 300 cases diagnosed since it was identified in 1998.

Scrapie is a transmissible disease similar to chronic wasting disease found in deer and elk. Scrapie is limited to sheep and goats and takes years to affect an animal after it has been infected.

There are no known human health risks associated with scrapie.

"This provides evidence that the surveillance program is working," said Bryce Reece, executive director of the Wyoming Wool Growers Association. "It also indicates that the program is on the cutting edge of science to detect such a rare disease during standard surveillance."

An epidemiological investigation is ongoing.


Published on Saturday, March 17, 2007.
Last modified on 3/17/2007 at 12:32 am

http://www.billingsgazette.net/articles/2007/03/17/news/wyoming/64-scrapie.txt

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

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

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

Edited by Stanley B. Prusiner, University of California, San Francisco, CA and approved September 12, 2005 (received for review March 21, 2005)

Abstract

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


sheep prion | transgenic mice

snip...

Conclusion

Our study demonstrates that an authentic TSE infectious agent is responsible in sheep and goats of sporadic atypical infections that remained unnoticed until recently. This raises important issues with regard to control of scrapie infection in small ruminants. Of major concern, ARR/ARR sheep can no longer be regarded as free of natural TSE infection. This finding challenges, at least to some extent, the foundation of the selective breeding programs engaged in several European Union member states (47, 48) and may call for a reappraisal of possible consequences of this strategy in the long term. Finally, more information about this newly discovered type of TSE agent, its prevalence in countries free of scrapie or BSE disease, and its potential to across-species transmission would be needed for a comprehensive evaluation of its implications in terms of public health.

http://www.pnas.org/cgi/content/full/102/44/16031

Volume 13, Number 1–January 2007
Research
Similar Biochemical Signatures and Prion Protein Genotypes in Atypical Scrapie and Nor98 Cases, France and Norway
Jean-Noël Arsac,* Olivier Andreoletti,† Jean-Marc Bilheude,‡ Caroline Lacroux,† Sylvie L. Benestad,§ and Thierry Baron*
*Agence Française de Sécurité Sanitaire des Aliments, Lyon, France; †Ecole Nationale Vétérinaire de Toulouse, Toulouse, France; ‡Bio-Rad, Marnes-la-Coquette, France; and §National Veterinary Institute, Oslo, Norway

Suggested citation for this article

Abstract
Isolates of atypical scrapie recently identified in sheep and goats in France were compared with Nor98 isolates reported in Norway. Western blot methods for characterization of the protease-resistant prion protein showed that all these isolates shared a unique biochemical signature: 5 groups of bands, including a characteristic band of apparent low molecular weight (11 kDa). This pattern could originate from the presence of 3 different protease cleavage products, including the 11 kDa most likely cleaved at both N- and C-sides of the protein. Genetic data, which strongly suggested the higher susceptibility of AHQ and AF141RQ animals in French cases, resembled earlier data from Nor98 scrapie.

snip...

Conclusion
Our data provide new information about the recently described atypical cases of TSE. These cases appear to be associated with a novel PrPres biochemical pattern; they shared similarities with some rare prion diseases in humans and were clearly distinct from classic scrapie or BSE. This potential similarity in PrPres formation mechanisms with some other rare prion diseases in humans is intriguing. However, the unusual properties of these atypical cases illustrate our decades of underestimating the biodiversity of TSEs in small ruminants and the consequences. This finding should lead to a general reexamination of our conceptual approach in the control of TSEs in small ruminants.

http://www.cdc.gov/ncidod/EID/13/1/58.htm

12/10/76
AGRICULTURAL RESEARCH COUNCIL
REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note
CHAIRMAN: PROFESSOR PETER WILDY

snip...

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

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

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

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

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

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

snip...

76/10.12/4.6

http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf

Like lambs to the slaughter
31 March 2001
Debora MacKenzie
Magazine issue 2284
What if you can catch old-fashioned CJD by eating meat from a sheep infected
with scrapie?
FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but
Singeltary was suspicious. The diagnosis didn't fit her violent symptoms,
and he demanded an autopsy. It showed she had died of sporadic
Creutzfeldt-Jakob disease.

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

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

The complete article is 889 words long.

full text;

http://www.newscientist.com/article.ns?id=mg16922840.300

Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates and
comparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*,
Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James
Ironside¶, Moira Bruce, Dominique Dormont*, 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 du Général Leclerc,
BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre
Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de
Neuropathologie, Hôpital de la Salpêtrière, 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 for Animal Health, Neuropathogenesis Unit, West Mains Road,
Edinburgh EH9 3JF, United Kingdom

Edited by D. Carleton Gajdusek, Centre National de la Recherche
Scientifique, 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.

http://www.pnas.org/cgi/content/full/041490898v1

Published online before print October 20, 2005

Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102
Medical 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 et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway


Edited by Stanley B. Prusiner, University of California, San Francisco, CA, 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 a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.


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

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

Subject: SCRAPIE UPDATE USA As of December 31, 2006 with OHIO topping out the list again
Date: March 11, 2007 at 6:14 pm PST
Infected and Source Flocks

As of December 31, 2006, there were 78 scrapie infected and source flocks (Figure 3). There were 2 new infected and source flocks reported in December (Figure 4) with a total of 15 flocks reported for FY 2007 (Figure 5). The total number of infected and source flocks that have been released in FY 2007 is 14 (Figure 6), with 4 flocks released in December. The ratio of infected and source flocks released to newly infected and source flocks for FY 2007 = 0.93:1. In addition, as of December 31, 2006, 80 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 8 were RSSS cases (Figure 7). This total includes 22 newly confirmed cases in December 2006 (Figure 8). Eighteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in March 2006. New infected flocks, source flocks, and flocks released for FY 2007 are depicted in Chart 3. New infected and source statuses from 1997 to 2006 are depicted in Chart 4.


http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html


Scrapie Program FY 2006
Revised February 5, 2007


http://www.aphis.usda.gov/vs/nahps/scrapie/yearly_report/yearly_report_2006.ppt


PLEASE note the high rate of scrapie in Ohio as at December, 31, 2006. I have been concerned about this for years, and questioned it several times over the years, Scrapie and CJD in Ohio ???

Item # Date Time Lines Subject
011956 2002-11-06 09:17 765 Re: SCRAPIE/CJD OHIO ???


https://lists.aegee.org/cgi-bin/wa?A2=ind0211&L=BSE-L&P=R3874&X=448C1D33204F216F58&Y=flounder9%40verizon.net


010952 2001-11-15 15:49 651 Re: U.S.A.--NEW SCRAPIE ERADICATION RULES $ OHIO $ sporadic CJDs ?

https://lists.aegee.org/cgi-bin/wa?A2=ind0111&L=BSE-L&P=R2745&X=448C1D33204F216F58&Y=flounder9%40verizon.net

010951 2001-11-15 11:48 582 U.S.A.--NEW SCRAPIE ERADICATION RULES $ OHIO $ sporadic CJDs ?


https://lists.aegee.org/cgi-bin/wa?A2=ind0111&L=BSE-L&P=R2162&X=448C1D33204F216F58&Y=flounder9%40verizon.net

010701 2001-08-02 14:10 193 Global Compliance of TSE regulations? & sCJD's, scrapie and feed mils in OHIO ?

https://lists.aegee.org/cgi-bin/wa?A2=ind0108&L=BSE-L&P=R346&X=448C1D33204F216F58&Y=flounder9%40verizon.net

Subject: Re: SCRAPIE/CJD OHIO ???
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
Date: Wed, 6 Nov 2002 09:17:33 -0600
Content-Type: text/plain

######## Bovine Spongiform Encephalopathy #########

Date: Thu, 2 Aug 2001 14:10:25 -0700
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: "Terry S. Singeltary Sr."
Subject: Global Compliance of TSE regulations? & sCJD's,
scrapie and feed mils in OHIO ?

snip...end.....TSS




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