Sunday, November 17, 2013

L-BSE in Genetically Susceptible and Resistant Sheep: Changes in Prion Strain or Phenotypic Plasticity of the Disease-Associated Prion Protein?

L-BSE in Genetically Susceptible and Resistant Sheep: Changes in Prion Strain or Phenotypic Plasticity of the Disease-Associated Prion Protein?
 
Simon Nicot1, Anna Bencsik1, Sergio Migliore2, Dominique Canal1, Mikael Leboidre1, Umberto Agrimi2, Romolo Nonno2 and Thierry Baron1,* + Author Affiliations
 
1Agence nationale de sécurité sanitaire de l'alimentation, de l'environnement et du travail (Anses), Unité Maladies Neuro-Dégénératives, Lyon Cedex 07, France
 
2Istituto Superiore di Sanità (ISS). Department of Veterinary Public Health and Food Safety, Rome, Italy ↵*Corresponding author: Thierry Baron, Anses. Laboratoire de Lyon. 31, avenue Tony Garnier, 69364 Lyon cedex 07, France. Phone: (+33) 4 78 72 65 43. Fax: (+33) 4 78 61 91 45. thierry.baron@anses.fr
 
Abstract
 
Background. Sheep with prion protein (PrP) gene polymorphisms QQ171 and RQ171 were shown to be susceptible to the prion causing L-type bovine spongiform encephalopathy (L-BSE), although RQ171 sheep specifically propagated a distinctive prion molecular phenotype in their brains, characterized by a high molecular mass protease-resistant PrP fragment (HMM PrPres), distinct from L-BSE in QQ171 sheep.
 
Methods. The resulting infectious and biological properties of QQ171 and RQ171 ovine L-BSE prions were investigated in transgenic mice expressing either bovine or ovine PrP.
 
Results. In both mouse lines, ovine L-BSE transmitted similarly to cattle-derived L-BSE, with respect to survival periods, histopathology, biochemical features of PrPres in the brain, as well as splenotropism, clearly differing from ovine classical BSE or from scrapie strain CH1641. Nevertheless and unexpectedly, HMM PrPres was found in the spleen of ovine PrP transgenic mice infected with L-BSE from RQ171 sheep at first passage, reminiscent, in lymphoid tissues only, of the distinct PrPres features found in RQ171 sheep brains.
 
Conclusions. The L-BSE agent differs from both ovine C-BSE or CH1641 scrapie maintaining its specific strain properties after passage in sheep, although striking PrPres molecular changes could be found in RQ171 sheep and in the spleen of ovine PrP transgenic mice. Received April 24, 2013. Revision received September 2, 2013. Accepted September 20, 2013. © The Author 2013. Published by Oxford University Press on behalf of the Infectious Diseases Society of America. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.
 
 
 
 
re-BSE in goats can be mistaken for scrapie
 
 
 
Wednesday, January 18, 2012
 
BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE
 
February 1, 2012
 
 
 
Wednesday, January 18, 2012
 
Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie
 
Journal of Neuropathology & Experimental Neurology:
 
February 2012 - Volume 71 - Issue 2 - p 140–147
 
 
 
Thursday, July 14, 2011
 
Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)
 
 
 
SHEEP AND BSE
 
PERSONAL AND CONFIDENTIAL
 
SHEEP AND BSE
 
A. The experimental transmission of BSE to sheep.
 
Studies have shown that the ''negative'' line NPU flock of Cheviots can be experimentally infected with BSE by intracerebral (ic) or oral challenge (the latter being equivalent to 0.5 gram of a pool of four cow brains from animals confirmed to have BSE).
 
 
 
RB264
 
BSE - TRANSMISSION STUDIES
 
 
 
Wednesday, January 18, 2012
 
Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie
 
Journal of Neuropathology & Experimental Neurology:
 
February 2012 - Volume 71 - Issue 2 - p 140–147
 
 
 
Saturday, November 02, 2013
 
OREGON DETECTS SCRAPIE
 
 
 
Tuesday, February 01, 2011

Sparse PrP-Sc accumulation in the placentas of goats with naturally acquired scrapie

Research article
http://scrapie-usa.blogspot.com/2011/02/sparse-prp-sc-accumulation-in-placentas.html
Thursday, June 2, 2011

USDA scrapie report for April 2011 NEW ATYPICAL NOR-98 SCRAPIE CASES Pennsylvania AND California
http://nor-98.blogspot.com/2011/06/usda-scrapie-report-for-april-2011-new.html
UPDATE PLEASE NOTE ;
AS of June 30, 2011,

snip...

INCLUDING 10 POSITIVE GOATS FROM THE SAME HERD (FIGURE 7).

snip...

see updated APHIS scrapie report ;
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
Tuesday, February 01, 2011

Sparse PrP-Sc accumulation in the placentas of goats with naturally acquired scrapie

Research article

snip...

Date: Tuesday, February 01, 2011 5:03 PM

To: Mr Terry Singeltary

Subject: Your comment on BMC Veterinary Research 2011, 7:7

Dear Mr Singeltary

Thank you for contributing to the discussion of BMC Veterinary Research 2011, 7:7 .

Your comment will be posted within 2 working days, as long as it contributes to the topic under discussion and does not breach patients' confidentiality or libel anyone. You will receive a further notification by email when the posting appears on the site or if it is rejected by the moderator.

Your posting will read:

Mr Terry Singeltary,

retired

Scrapie cases Goats from same herd USA Michigan

Comment: " In spite of the poorly defined effects of PRNP genetics, scrapie strain, dose, route and source of infection, the caprine placenta may represent a source of infection to progeny and herd mates as well as a source of persistent environmental contamination. "

Could this route of infection be the cause of the many cases of Goat scrapie from the same herd in Michigan USA ?

Has this been investigated ?

(Figure 6) including five goat cases in FY 2008 that originated from the same herd in Michigan. This is highly unusual for goats, and I strenuously urge that there should be an independent investigation into finding the common denominator for these 5 goats in the same herd in Michigan with Scrapie. ...

Kind Regards, Terry
Thursday, January 07, 2010

Scrapie and Nor-98 Scrapie November 2009 Monthly Report Fiscal Year 2010 and FISCAL YEAR 2008
http://scrapie-usa.blogspot.com/2010/01/scrapie-and-nor-98-scrapie-november.html
In FY 2010, 72 cases of classical Scrapie and 5 cases of Nor-98 like Scrapie were confirmed...
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/yearly_report.ppsx
Scrapie Nor-98 like case in California FY 2011 AS of December 31, 2010.

Scrapie cases in goats FY 2002 - 2011 AS of December 31, 2010 Total goat cases = 21 Scrapie cases, 0 Nor-98 like Scrapie cases (21 field cases, 0 RSSS cases)

Last herd with infected goats disignated in FY 2008 Michigan 8 cases
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
Tuesday, February 01, 2011

Sparse PrP-Sc accumulation in the placentas of goats with naturally acquired scrapie

Research article
http://www.biomedcentral.com/1746-6148/7/7/abstract
http://www.biomedcentral.com/content/pdf/1746-6148-7-7.pdf
"In spite of the poorly defined effects of PRNP genetics, scrapie strain, dose, route and source of infection, the caprine placenta may represent a source of infection to progeny and herd mates as well as a source of persistent environmental contamination."

Could this route of infection be the cause of the many cases of Goat scrapie from the same herd in Michigan USA ?

Has this been investigated ?

(Figure 6) including five goat cases in FY 2008 that originated from the same herd in Michigan. This is highly unusual for goats, and I strenuously urge that there should be an independent investigation into finding the common denominator for these 5 goats in the same herd in Michigan with Scrapie. ...

Kind Regards, Terry
SNIP...
Scrapie Nor-98 like case in California FY 2011 AS of December 31, 2010.

Scrapie cases in goats FY 2002 - 2011 AS of December 31, 2010 Total goat cases = 21 Scrapie cases, 0 Nor-98 like Scrapie cases (21 field cases, 0 RSSS cases)

Last herd with infected goats disignated in FY 2008 Michigan 8 cases
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
UPDATED RESPONSE ON MY CONCERNS OF GOAT SCRAPIE IN MICHIGAN ;

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

From: "BioMed Central Comments"

To:

Sent: Wednesday, February 16, 2011 4:13 AM

Subject: Your comment on BMC Veterinary Research 2011, 7:7

Your discussion posting "Scrapie cases Goats from same herd USA Michigan" has been rejected by the moderator as not being appropriate for inclusion on the site.

Dear Mr Singeltary,

Thank you for submitting your comment on BMC Veterinary Research article (2011, 7:7). We have read your comment with interest but we feel that only the authors of the article can answer your question about further investigation of the route of infection of the five goats in Michigan. We advise that you contact the authors directly rather than post a comment on the article.

With best wishes,

Maria

Maria Kowalczuk, PhD Deputy Biology Editor BMC-series Journals

BioMed Central 236 Gray's Inn Road London, WC1X 8HB

+44 20 3192 2000 (tel) +44 20 3192 2010 (fax)

W: www.biomedcentral.com E: Maria.Kowalczuk@biomedcentral.com

Any queries about this decision should be sent to comments@biomedcentral.com

Regards

BMC Veterinary Research

SNIP...PLEASE SEE FULL TEXT ;

Tuesday, February 01, 2011

Sparse PrP-Sc accumulation in the placentas of goats with naturally acquired scrapie

Research article
http://scrapie-usa.blogspot.com/2011/02/sparse-prp-sc-accumulation-in-placentas.html
Thursday, March 29, 2012

atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012

NIAA Annual Conference April 11-14, 2011San Antonio, Texas
http://nor-98.blogspot.com/2012/03/atypical-nor-98-scrapie-has-spread-from.html


***SCRAPIE GOATS CALIFORNIA 13 CASES TO DATE ! ***

***SCRAPIE GOATS MICHIGAN 8 CASES TO DATE ! ***

(an unusually high amount of scrapie documented in goats for a happenstance of bad luck, or spontaneous event, THAT DOES NOT HAPPEN IN OTHER STATES ??? )

Sunday, June 2, 2013

Characterisation of an Unusual TSE in a Goat by Transmission in Knock-in Transgenic Mice
http://transmissiblespongiformencephalopathy.blogspot.com/2013/06/characterisation-of-unusual-tse-in-goat.html

Friday, July 26, 2013

Voluntary Scrapie Program USA UPDATE July 26, 2013 increase in FY 2013 is not statistically meaningful due to the sample size
http://scrapie-usa.blogspot.com/2013/07/voluntary-scrapie-program-usa-update.html

 
SUMMARY REPORT CALIFORNIA atypical L-type BASE BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012
 
Summary Report BSE 2012
 
Executive Summary
 
 
 
Saturday, August 4, 2012
 
Final Feed Investigation Summary - California atypical L-type BASE BSE Case - July 2012
 
 
 
Saturday, August 4, 2012
 
Update from APHIS Regarding Release of the Final Report on the atypical L-type BASE BSE Epidemiological Investigation
 
 
 
Saturday, November 2, 2013
 
APHIS Finalizes Bovine Import Regulations in Line with International Animal Health Standards while enhancing the spread of BSE TSE prion mad cow type disease around the Globe
 
 
 
I AGREE WITH MR. BULLARD, it’s all about trade and money, BSE TSE PRION aka mad cow type disease and sound science there from, was thrown out the window by the USDA et al that fateful day in December 23, 2003, when the USDA lost it’s ‘gold card’ of supposedly being BSE FREE, (that was and still is a sad joke though), that’s when mad cow junk science was adopted by the USDA...
 
see why below...kind regards, terry
 
 
Monday, November 4, 2013
 
*** R-CALF Bullard new BSE rule represents the abrogation of USDA’s responsibility to protect U.S. consumers and the U.S. cattle herd from the introduction of foreign animal disease
 
 
 
Saturday, November 2, 2013
 
Exploring the risks of a putative transmission of BSE to new species
 
 
 
Wednesday, October 30, 2013
 
SPECIFIED RISK MATERIAL (SRM) CONTROL VERIFICATION TASK FSIS NOTICE 70-13 10/30/13
 
 
 
Wednesday, September 25, 2013
 
Inspections, Compliance, Enforcement, and Criminal Investigations BSE TSE PRION 2013
 
 
 
Sunday, September 1, 2013
 
Evaluation of the Zoonotic Potential of Transmissible Mink Encephalopathy
 
We previously described the biochemical similarities between PrPres derived from L-BSE infected macaque and cortical MM2 sporadic CJD: those observations suggest a link between these two uncommon prion phenotypes in a primate model (it is to note that such a link has not been observed in other models less relevant from the human situation as hamsters or transgenic mice overexpressing ovine PrP [28]). We speculate that a group of related animal prion strains (L-BSE, c-BSE and TME) would have a zoonotic potential and lead to prion diseases in humans with a type 2 PrPres molecular signature (and more specifically type 2B for vCJD)
 
snip...
 
Together with previous experiments performed in ovinized and bovinized transgenic mice and hamsters [8,9] indicating similarities between TME and L-BSE, the data support the hypothesis that L-BSE could be the origin of the TME outbreaks in North America and Europe during the mid-1900s.
 
 
 
MAD COW TESTING ONLY CATCHES SOME MAD COWS
 
Saturday, October 19, 2013
 
A comparative study of modified confirmatory techniques and additional immuno-based methods for non-conclusive autolytic Bovine spongiform encephalopathy cases
 
 
 
Wednesday, October 09, 2013
 
WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY, £41,078,281 in compensation REVISED
 
 
 
Thursday, October 10, 2013
 
CJD REPORT 1994 increased risk for consumption of veal and venison and lamb
 
 
 
Monday, October 14, 2013
 
Researchers estimate one in 2,000 people in the UK carry variant CJD proteins
 
 
 
WHAT about the sporadic CJD TSE proteins ?
 
WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$
 
 
Sunday, August 11, 2013
 
Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013
 
Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010
 
 
 
Sunday, October 13, 2013
 
CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
 
 
 
Tuesday, September 24, 2013
 
NORDION (US), INC., AND BIOAXONE BIOSCIENCES, INC., Settles $90M Mad Cow TSE prion Contamination Suit Cethrin(R)
 
Case 0:12-cv-60739-RNS Document 1 Entered on FLSD Docket 04/26/2012 Page 1 of 15
 
 

Saturday, November 16, 2013

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

Infect Control Hosp Epidemiol.
http://creutzfeldt-jakob-disease.blogspot.com/2013/11/management-of-neurosurgical-instruments.html
 
 
TSS

posted by Terry S. Singeltary Sr. at 3:55 PM 0 Comments