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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Thursday, March 26, 2015

National Scrapie Eradication Program Monthly Report - February 2015

National Scrapie Eradication Program Monthly Report - February 2015

 

 The monthly report for the National Scrapie Eradication Program for February 2015 is now available. The monthly reports are available in both PowerPoint and PDF formats.

 

PowerPoint Monthly Report PDF Monthly Report Highlights of the February 2015 Report

 

One new scrapie infected flock and two new scrapie source flocks have been designated in FY 2015. Since the beginning of FY 2015, 29 sheep have tested positive for scrapie; 26 of these positives were from the same source flock. Two goats have tested for positive—both from the same herd.

 

The most recent positive case was confirmed on February 18, 2015; the animal was a black-faced sheep tested at slaughter. A trace investigation is underway.

 

For FY 2015, as of February 28, 12,683 sheep and 3,325 goats have been tested for scrapie.

 

Scrapie/Scrapie Program Monthly Tidbit

 

In November 2014, the first positive goat found through VS’ Regulatory Scrapie Slaughter Surveillance (RSSS) program was identified. Based on the goats sampled at slaughter to date, the prevalence of scrapie in U.S. cull goats is 0.004 percent with an upper 95 percent confidence limit of 0.013 percent.

 

Resources

 

To report a sheep or goat with clinical signs of scrapie, please contact your local VS office. To learn more about scrapie, the disease, and the national scrapie eradication program visit the APHIS VS Scrapie Website and www.eradicatescrapie.org.

 

 

SNIP...

 

As of February 28, 2015, 2 black-faced sheep and 1 goat have tested positive for scrapie in FY 2015; this is the first positive goat case found through RSSS. The weighted percentage of samples from sheep that have tested positive for each face color from FY 2003 through FY 2015 is depicted in Chart 3**; percent positive goats are shown in Chart 3a. In November 2013, administrative units within APHIS Veterinary Services reorganized from 2 Regions to 6 Districts (Figure 1). The distribution of sheep and goat populations by District is depicted in Chart 4a. The number of animals collected for FY 2015 by District where collected is shown in Chart 4b. A monthly comparison of RSSS collections by fiscal year is displayed in Chart 5. Chart 6 is a retrospective 6-month rolling average of the percent positive, black-faced sheep sampled at RSSS collection sites.

 

* RSSS positives are reported based on collection date and may have been confirmed after February 28, 2015.

 

** White, black and mottled face color sheep are weighted based on population. White faced sheep have the highest weight, so when the rare white face positive sheep is found it causes this statistic to markedly increase. Goats and other face colored sheep are not included in this calculation.

 

Introduction – Surveillance (Part 1)

 

Introduction – Surveillance (Part 1)

 

INTRODUCTION - Surveillance (Part 2) On-Farm Surveillance Testing sheep and goats on-farm is an essential part of scrapie surveillance. It includes both regulatory testing of scrapie exposed and potentially exposed sheep and goats and testing sheep and goats on farm for routine surveillance. As the National Scrapie Eradication Program moves closer towards meeting the goal of identifying the last remaining cases of classical scrapie, finding and testing all sheep and goats meeting targeted sampling criteria is even more important. As of February 28, 2015, 511 sheep and 214 goats have been tested on-farm for FY 2015. Twenty-eight animals (27 sheep and 1 goat) have tested positive; 26 of the positive sheep were from the same flock. The number of animals tested on-farm by month and by species for FY 2015 is shown in Chart 7. Total Animals Sampled for Scrapie Testing As of February 28, 2015, 16,188 animals have been sampled for scrapie testing in FY 2015:

 


15,463 RSSS samples and 725 on-farm samples (Chart 8);

 


Of which 12,863 were sheep and 3,325 were goats. Distribution of sampling by type (RSSS or on-farm) and by species is shown in Chart 9.

 

 


 

 

Increased Infectivity of Anchorless Mouse Scrapie Prions in Transgenic Mice Overexpressing Human Prion Protein

 

Brent Race1#, Katie Phillips1, Kimberly Meade-White2, James Striebel1 and Bruce Chesebro1

 

+ Author Affiliations 1Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT, 59840 USA 2Rocky Mountain Veterinary Branch, Rocky Mountain Laboratories, National Instituteof Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT, 59840 USA

 

ABSTRACT

 

Prion protein (PrP) is found in all mammals mostly as a glycoprotein anchored to the plasma membrane by a C-terminal glycophosphatidylinositol (GPI) linkage. Following prion infection, host protease-sensitive prion protein (PrPsen or PrPC) is converted into an abnormal, disease-associated, protease-resistant form (PrPres). Biochemical characteristics such as the PrP amino acid sequence and post-translational modifications such as glycosylation and GPI anchoring, can affect the transmissibility of prions as well as the biochemical properties of the PrPres generated. Previous in vivo studies on the effects of GPI anchoring on prion infectivity have not examined cross-species transmission. Here we tested the effect of lack of GPI anchoring on a species barrier model using mice expressing human PrP. In this model, anchorless 22L prions derived from tg44 mice were more infectious than 22L prions derived from C57BL/10 mice when tested in tg66 transgenic mice, which expressed wild-type anchored human PrP at 8-16 fold above normal. Thus the lack of the GPI anchor on the PrPres from tg44 mice appeared to reduce the effect of the mouse-human PrP species barrier. In contrast, neither source of prions induced disease in tgRM transgenic mice which expressed human PrP at 2-4 fold above normal.

 

Importance Prion protein (PrP) is found in all mammals, usually attached to cells by an anchor molecule, called GPI. Following prion infection, PrP is converted into a disease-associated form (PrPres). While most prion diseases are species-specific, this finding is not consistent, and species barriers differ in strength. The amino acid sequence of PrP varies among species, and this variability affects prion species barriers. However, other PrP modifications, including glycosylation and GPI-anchoring, may also influence cross-species infectivity. We studied the effect of PrP GPI-anchoring using a mouse to human species barrier model. Experiments showed that prions produced by mice expressing only anchorless PrP were more infectious than prions produced in mice expressing anchored PrP. Thus, the lack of the GPI anchor on prions reduced the effect of the mouse-human species barrier. Our results suggest that prion diseases that produce higher levels of anchorless PrP may pose an increased risk for cross-species infection.

 

FOOTNOTES

 

↵#Corresponding author. Mailing address: Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 903 South Fourth Street, Hamilton, MT, 59840 USA, Phone: 406-363-9360, Email: raceb@niaid.nih.gov Copyright © 2015, American Society for Microbiology. All Rights Reserved.

 


 

Tuesday, December 16, 2014

 

Evidence for zoonotic potential of ovine scrapie prions

 

Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics

 

Abstract

 

Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

 

Subject terms: Biological sciences• Medical research At a glance

 


 

why do we not want to do TSE transmission studies on chimpanzees $

 

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

 

snip...

 

R. BRADLEY

 


 

Suspect symptoms

 

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

 

28 Mar 01 Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 

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

 

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

 

2001

 

Suspect symptoms

 

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

 

28 Mar 01

 

Like lambs to the slaughter

 

31 March 2001

 

by Debora MacKenzie Magazine issue 2284.

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 


 

Friday, January 30, 2015

 

*** Scrapie: a particularly persistent pathogen ***

 


 

Thursday, March 26, 2015

 

Increased Infectivity of Anchorless Mouse Scrapie Prions in Transgenic Mice Overexpressing Human Prion Protein

 


 

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 ;

 


 

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

 


 

In FY 2010, 72 cases of classical Scrapie and 5 cases of Nor-98 like Scrapie were confirmed...

 


 

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

 


 

Tuesday, February 01, 2011

 

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

 

Research article

 


 


 

"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

 


 

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

 


 

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

 


 

***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

 


 

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

 


 

 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

 


 

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?

 


 

 Wednesday, December 24, 2014

 

National Scrapie Eradication Program November 2014 Monthly Report Fiscal Year 2015

 


 

 TSS

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