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.
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_report.pdf
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
posted by Terry S. Singeltary Sr. | 12:57 PM
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