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Atypical Scrapie naturally infect sheep with resistant PrP

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


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: [email protected]

www.pnas.org/cgi/doi/10.1073/pnas.0502296102


http://www.pnas.org/cgi/content/abstract/0502296102v1


TSS

#################### https://lists.aegee.org/bse-l.html ####################


Infected and Source Flocks

As of August 31, 2005, there were 115 scrapie infected and source flocks
(figure 3). There were 3 new infected and source flocks reported in August
(Figure 4) with a total of 148 flocks reported for FY 2005 (Figure 5). The
total infected and source flocks that have been released in FY 2005 are 102
(Figure 6), with 5 flocks released in August. The ratio of infected and
source flocks released to newly infected and source flocks for FY 2005 =
0.69 :
1. In addition, as of August 31, 2005, 574 scrapie cases have been confirmed
and reported by the National Veterinary Services Laboratories (NVSL), of
which 122 were RSSS cases (Figure 7). This includes 55 newly confirmed cases
in August 2005 (Figure 8). Fifteen cases of scrapie in goats have been
reported since 1990 (Figure 9). The last goat case was reported in May 2005.

snip...

full text ;

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


SCRAPIE USA JULY 2005 UPDATE

AS of July 31, 2005, there were 120 scrapie infected soure flocks (figure
3). There were 16 new infected and source flocks reorted in July (Figure 4)
with a total of 143 flocks reported for FY 2005 (Figure 5). The total
infected and source flocks that have been released in FY 2005 are 89 (Figure
6), with 8 flocks released in July. The ratio of infected and source flocks
released to newly infected and source flocks for FY = 0.62 : 1. IN addition,
as of July 31, 2005, 524 scrapie cases have been confirmed and reported by
the National Veterinary Services Laboratories (NVSL), of which 116 were RSSS
cases (Figure 7). This includes 76 newly confirmed cases in July 2005
(Figure 8). Fifteen cases of scrapie in goats have been reported since 1990
(Figure 9). The last goat case was reported in May 2005. ...........

snip...

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


SCRAPIE USA JUNE 2005 UPDATE


AS of June 30, 2005, there were 114 scrapie infected and source flocks
(Figure 3). There were 14 new infected and source flocks reported in June
(Figure 4) with a total of 123 flocks reported for FY 2005 (Figure 5).


snip...


In addition, as of June 30, 2005, 448 scrapie cases have been confirmed and
reported by the National Veterinary Services Laboratories (NVSL), of which
106 were RSSS cases (Figure 7). This includes 81 newly confirmed cases in
June 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported
since 1990 (Figure 9). The last goat case was reported in May 2005.


snip...end


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


From: TSS ()
Subject: SCRAPIE USA UPDATE MARCH - JUNE 2005
Date: August 24, 2005 at 7:03 pm PST

SCRAPIE USA MONTHLY REPORT 2005

AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure
3). There were 11 new infected and source flocks reported in March (Figure
4) with a total of 51 flocks reported for FY 2005 (Figure 5). The total
infected and source flocks that have been released in FY 2005 are 39 (Figure
6), with 1 flock released in March. The ratio of infected and source flocks
released to newly infected and source flocks for FY 2005 = 0.76 : 1. IN
addition, as of March 31, 2005, 225 scrapie cases have been confirmed and
reported by the National Veterinary Services Laboratories (NVSL), of which
53 were RSSS cases (Figure 7). This includes 57 newly confirmed cases in
March 2005 (Figure 8). Fourteen cases of scrapie in goats have been reported
since 1990 (Figure 9). The last goat cases was reported in January 2005. New
infected flocks, source flocks, and flocks released or put on clean-up plans
for FY 2005 are depicted in Figure 10. ...

FULL TEXT ;

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


TSS
 

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