From: Terry S. Singeltary Sr.
Sent: Thursday, December 17, 2015 12:13 PM
Cc: Bernhard.Url@efsa.europa.eu ; CJD-L@LISTS.AEGEE.ORG ; cjdvoice@yahoogroups.com ; bloodcjd@yahoogroups.com ; EFSA
Press ; sue.davies@which.co.uk ; Carlos.Moedas@ec.europa.eu ; Vytenis.Andriukaitis@ec.europa.eu
; jaana.husu-kallio@cec.eu.int ; pg.facelli@sanita.it ; p.piattelli@sanita.it ; am.taddei@sanit.it
; e.serpetti@sanita.it ; jordbruksverket@jordbruksverket.se
Subject: Re: Annual report of the Scientific Network on BSE-TSE 2015
EFSA-Q-2015-00738 10 December 2015
p.s. I forgot a few grams of suspect mad cow feed still in commerce in the
USA since August 1997 ban that was nothing but ink on paper...see at the
bottom...kindest regards, terry
From: Terry S. Singeltary Sr.
Sent: Thursday, December 17, 2015 12:04 PM
Cc: Bernhard.Url@efsa.europa.eu ; CJD-L@LISTS.AEGEE.ORG ; cjdvoice@yahoogroups.com ; bloodcjd@yahoogroups.com ; EFSA
Press ; sue.davies@which.co.uk ; Carlos.Moedas@ec.europa.eu ; Vytenis.Andriukaitis@ec.europa.eu
; jaana.husu-kallio@cec.eu.int ; pg.facelli@sanita.it ; p.piattelli@sanita.it ; am.taddei@sanit.it
; e.serpetti@sanita.it ; jordbruksverket@jordbruksverket.se
Subject: Annual report of the Scientific Network on BSE-TSE 2015
EFSA-Q-2015-00738 10 December 2015
Annual report of the Scientific Network on BSE-TSE 2015
Question Number:
EFSA-Q-2015-00738
Issued:
10 December 2015
Download Report (427.8 KB)
Abstract:
The EFSA Scientific Network on bovine spongiform encephalopathies and other
transmissible spongiform encephalopathies (BSE-TSE) held its 10th meeting on 7
and 8 October 2015 in Parma. The meeting served as an opportunity to exchange
scientific information on BSE-TSE related issues among EU Member States,
countries from the European Free Trade Association (EFTA), EFSA, the European
Commission and ad hoc participants. In this occasion, ad hoc representation
included the World Animal Health Organisation (OIE), a scientific expert on
genetic breeding for resistance to Classical scrapie in goats, and two
scientific experts on human variant Creutzfeldt-Jakob disease (vCJD). The topics
discussed included an update on BSE-TSE-related activities at national and
international level and results from recent TSE-related research projects and
risk assessments in several EU countries. In addition, the investigations of
recent Classical BSE cases, the monitoring and control of Classical scrapie in
small ruminants, and the distribution and trends of vCJD cases in humans were
also discussed.
Summary:
Establishing a system of Networks of organisations operating in the fields
within EFSA’s mission is among the tasks of EFSA, according to its founding
regulation (Regulation (EC) No 178/2002), in order to facilitate a scientific
cooperation framework by the coordination of activities, the exchange of
information, the development and implementation of joint projects, the exchange
of expertise and best practices. Additionally, the EFSA Science Strategy
2012–2016 set the objective of developing, together with Member States’
competent authorities, multi-annual work programmes focused on filling data gaps
and setting priorities for data collections. To implement the above provisions
various Networks were established. The Scientific Network on bovine spongiform
encephalopathies and other transmissible spongiform encephalopathies (BSE-TSE)
was launched in 2006. The BSE-TSE Network had its first meeting in 2006, and,
following this, one meeting per year has been held.
The BSE-TSE Network is currently composed as follows: Network Members
representing 27 EU MSs (Malta has not appointed a representative to this
Network) and countries from the European Free Trade Association (EFTA). The
European Commission Directorate-General of Health and Consumers and of Research
and Innovation are also Observers of the Network.
The 10th meeting of the Network was held on 7–8 October 2015 in Parma. In
this occasion, ad hoc representation included the World Animal Health
Organisation (OIE), a scientific expert on genetic breeding for resistance to
Classical scrapie in goats, and two scientific experts on human variant
Creutzfeldt-Jakob disease (vCJD).
The topics discussed included an update on BSE-TSE-related activities at
national and international level, results from recent TSE-related research
projects and risk assessments in several EU countries, the investigations of
recent Classical BSE cases, the monitoring and control of Classical scrapie in
small ruminants, and the distribution and trends of vCJD cases in humans.
Published:
11 December 2015
snip...
1. Introduction
Background and Terms of Reference as provided by EFSA 1.1.
Art. 23 (g) of the EFSA founding regulation1 stipulates that EFSA shall
establish a system of Networks of organisations operating in the fields within
its mission and be responsible for their operation. Furthermore, Art. 23 (e) and
Art. 33 provide for collection, collation, analysis and reporting on scientific
and technical data in the fields within the Authority’s mission. The aim of such
networking, as defined in Art. 36, is to facilitate a scientific cooperation
framework by the coordination of activities, the exchange of information, the
development and implementation of joint projects, the exchange of expertise and
best practices. Additionally, since ‘in order to obtain data of adequate quality
it is essential that data collections are planned over the medium to longer
term’, the EFSA Science Strategy 2012–20162 set the objective of developing,
together with the Member States’ competent authorities, multi-annual work
programmes focused on filling data gaps and setting priorities for data
collections.
To implement the above provisions of the founding regulation various
Networks were established.
In 2006 the Network on Microbiological Risk Assessment and the Network on
bovine spongiform encephalopathies and other transmissible spongiform
encephalopathies (BSE-TSE) convened, strengthening over time the scientific
cooperation on issues of concern, anticipating and reducing the duplication of
activities and hence avoiding divergence of opinions.
All these ongoing initiatives were supported by consultative processes,
such as the review3 of the EFSA’s Strategy for cooperation and networking with
Member States.4 In result EFSA identified four priority areas for the
cooperation, notably: (i) exchange of scientific data and information, (ii)
sharing of the risk assessment practices, (iii) harmonisation of risk assessment
methodologies, and (iv) cooperation and coherence in communication. Meanwhile,
the Management Board adopted a Decision5 governing the establishment and
operation of EFSA Networks.
In 2013 the Network mandates expired. Owing to a need for a strong specific
cooperation platform between EFSA and the Member States to provide advice, steer
the process and help establishing a common vision, EFSA Advisory Forum at their
meeting in December 2013 made recommendations with regard to the terms of
reference of the Networks. Therefore it is now opportune to renew the mandates
of the Networks operating in the remits of the Risk Assessment and Scientific
Assistance (RASA) units.
The main overall goals of the Scientific Network on BSE-TSE are: to improve
dialogue among participants; to build mutual understanding of risk assessment
principles; to enhance knowledge on and confidence in the scientific assessments
carried out in the EU; and to provide increased transparency in the current
process among Member States and EFSA. The Network also aims to raise the
harmonisation level of the risk assessments developed in the European Union
(EU). The Scientific Network strengthens the scientific cooperation on BSE-TSE.
It aims at anticipating and reducing the duplication of activities and hence
avoiding divergence of opinions. The Network is a privileged environment to
share data and methodologies facilitating harmonisation of assessment practices
and to assist in anticipating emerging risks in the EU.
1 Regulation (EC) No 178/2002 of the European Parliament and the Council of
28 January 2002 laying down the general principles and requirements of food law,
establishing the European Food Safety Authority and laying down procedures in
matters of food safety. OJ L 31, 1.2.2002, p.1–24.
2 EFSA Science Strategy 2012-2016. Available online: http://www.efsa.europa.eu/en/corporate/pub/sciencestrategy12.htm
3 Interim Review of the Strategy for Cooperation and Networking between EU
Member States and EFSA. Available online: http://www.efsa.europa.eu/en/keydocs/docs/msstrategyreview.pdf
4 MB 19.12.2006 – 6a Strategy for cooperation and networking. Available
online: http://www.efsa.europa.eu/en/keydocs/docs/networksoperation.pdf
5 MB 18 03 10 – item 7 doc 6 Management Board Decision concerning the
establishment and operation of European Networks of scientific organisations
operating in the fields within the Authority’s mission. Available online: http://www.efsa.europa.eu/en/scdocs/doc/panelnetworksrop.pdf
Annual report of the Scientific Network on BSE-TSE
www.efsa.europa.eu/publications 6 EFSA Supporting publication
2015:EN-915
The specific objectives of the Scientific Network on BSE-TSE are:
a. identifying common themes and areas for mutual collaboration;
b. identifying and avoiding duplication and divergence of opinion;
c. identification of experts in specific areas and on special issues;
d. sharing of data availability and quality;
e. strengthening cooperation amongst risk assessors and risk
managers;
f. exchanging information between EFSA, Member States and other
stakeholder;
g. strengthening communication between EFSA and the EU Member States and
among risk assessors, risk managers and stakeholders;
h. focusing attention on and streamlining of common research needs;
i. identifying potential emerging risks when addressing current
issues.
EFSA may entrust to the Network certain tasks, in particular preparatory
work for scientific opinions, scientific and technical assistance, and
collection of data.
2. Annual meeting 2015
The Scientific Network on BSE-TSE was launched in 2006. The BSE-TSE Network
had its first meeting in 2006, and, following this, one meeting per year has
been held.
The 10th meeting of the EFSA Scientific Network on BSE-TSE was held on 7–8
October 2015 in Parma (‘annual meeting 2015’).
The annual meeting 2015 was attended by representatives of 22 EU Member
States (Austria, Belgium, Bulgaria, Croatia, Cyprus, the Czech Republic,
Estonia, Finland, France, Germany, Greece, Hungary, Ireland, Italy, Luxembourg,
the Netherlands, Poland, Romania, Slovakia, Slovenia, Spain and the United
Kingdom) and by the representative of one EFTA country (Norway). The European
Commission (Directorate-General Health and Consumers (DG SANTE)) was also
present at the meeting. Further ad hoc attendees to this meeting included: Dr.
Derek Belton (World Organisation for Animal Health (OIE)), Dr. Jan Langeveld,
Dr. Maurizio Pocchiari and Dr. Maria Puopolo. Apologies were received from
Network Members from Denmark, Latvia, Lithuania, Portugal, Sweden and
Switzerland. Malta has not appointed a representative to this EFSA
Network.
Topics discussed during the meeting are summarised in the following
sections.
Follow-up from annual meeting 2014 2.1.
During the 2014 Network meeting, the Network expressed an interest to
strengthen the collaboration of the Network with the OIE and other international
organisations/agencies, and to consider the invitation of representatives from
Third Countries in order to have insights on BSE-TSE related issues from a
non-EU perspective (EFSA, 2014). In reply to these suggestions, OIE
participation was confirmed for the 2015 Network meeting, and EFSA has taken
contacts with the European Centre for Disease Prevention and Control (ECDC),
inviting representatives from the ECDC former EuroCJD Network for participation
in the 2015 Network meeting. Invitation of representatives from Third Countries
will be considered in future meetings.
Update on Network Members’ and EFSA’s activities in the field of 2.2.TSE
since the annual meeting 2014
The Members of the Network provided an update on the situation of classical
and atypical TSE in their countries and summarised to the group the scientific
activities in the field of TSE, including risk assessment activities, which had
been undertaken in their respective countries since the previous Network
meeting. Austria, the Czech Republic and France reported that the EFSA TSE
infectivity (TSEi) Model presented during the 2014 Network Meeting, and provided
by EFSA to the three countries upon request, was asked and possibly used for
study/research purposes.
Annual report of the Scientific Network on BSE-TSE
www.efsa.europa.eu/publications 7 EFSA Supporting publication
2015:EN-915
The Secretariat of the EFSA Unit on Biological Hazards and Contaminants
(BIOCONTAM) presented the EFSA activities in the field of TSE already completed
and ongoing since the 2014 Network meeting. Completed activities include one
Scientific Opinion of the Panel on Biological Hazards (BIOHAZ) on the zoonotic
potential of ovine scrapie prions (EFSA BIOHAZ Panel, 2015). Ongoing activities
include two mandates for scientific and technical assistance and a procurement
activity: a) the evaluation of applications of Denmark, Finland and Sweden to be
recognised as having a negligible risk status for classical scrapie; b) the
revision of the BSE monitoring regime in Croatia; c) procurement activity on an
experimental study on the infectivity of sheep embryos.
Results from search and safeguarding of TSE-resistant goats 2.3.
Dr. Jan Langeveld presented to the Network an overview on the results of
several research projects carried out in the EU over 13 years, focusing on the
work conducted in the last few years by the Goat-TSE-FREE consortium in relation
to genetic resistance to TSE in goats. He presented the different goat genotypes
identified in several EU countries and the association of genotype distribution
and occurrence of scrapie. He presented results from experimental challenges of
goats of different genotypes with different TSE agents and from the use of
several mouse models to study TSE infectivity in goats. Dr. Jan Langeveld
concluded that, based on current scientific evidence, the K222 allele and some
other polymorphisms of the prion protein gene have shown to confer resistance to
TSE in goats. The representative of Cyprus indicated that the studies on genetic
resistance to classical scrapie in goats in Cyprus, presented to the 2014
Network meeting, have been progressing and that final results will be reported
soon to the European Commission.
Update on UK/EU-RL activities and research projects 2.4.
The representative of the United Kingdom (UK) presented to the Network an
update of the results of 2015 TSE surveillance in the UK, also including a
recent case of classical BSE in a cow, currently under investigation. She
presented results of studies on atypical BSE transmission to sheep and of
studies investigating the presence of prion accumulation in human appendices.
She also updated the Network on the activities of the EU Reference Laboratory
(EU-RL) for TSE, including results from studies on the diagnostic sensitivity of
rapid tests for the detection of TSE in goats.
Investigation on the origin of a case of Classical BSE in Ireland
2.5.
The representative of Ireland updated the Network on the classical BSE case
detected in a 5-year-old cow in June 2015 in Ireland. He presented the results
of the epidemiological investigation aimed at detecting the origin of the case,
which has not produced so far evidence to support horizontal transmission of
classical BSE to the cow following to environmental exposure or feed, vertical
transmission or iatrogenic transmission.
Investigation on the existence of spontaneous cases of Classical
2.6.BSE
The representatives of Poland and Germany presented to the Network the
results of joint studies on classical BSE isolates from cattle and comparison
with atypical BSE cases using biochemical characterisation and mouse bioassay.
Recent ANSES activities on TSE 2.7.
The representative of France updated the Network on the most recent TSE
risk assessments carried out by The French Agency for Food, Environmental and
Occupational Health & Safety (ANSES). In particular, he presented the
results of an assessment of the surveillance programmes for TSE in small
ruminants in France, of two assessments of the human TSE transmission risks
linked to a possible change of the list of specified risk material, and of one
assessment of the risks linked to the use of ruminant fat in animal feed.
Annual report of the Scientific Network on BSE-TSE
www.efsa.europa.eu/publications 8 EFSA Supporting publication
2015:EN-915
Practical problems and challenges with the design of TSE 2.8.surveillance
in small ruminants
The representative of Croatia updated the Network on the TSE surveillance
in small ruminants in his country. Surveillance in small ruminants started in
Croatia in 2002 and the number of animals tested increased only in 2010–2015.
Despite almost 11,000 tests in sheep and 3,000 tests in goats, classical scrapie
has never been detected, and only two cases of atypical scrapie (in 2013 and
2015) have been detected. Also considering the epidemiological situation in
neighbouring countries, he concluded that it seems surprising that classical
scrapie has not been detected in Croatia so far. A discussion followed on
relevant issues that Croatia may consider when studying the situation and
designing and implementing a surveillance programme aimed at maximising the
chances to detect classical scrapie if present, including: a) investigation on
genotypes of the small ruminant population; b) implementation of risk-based
active surveillance (e.g. focused on fallen stock); c) use of incentives to
encourage reporting and presentation of animals for testing; d) consideration to
breeding for genetic resistance.
Distribution and trends of vCJD and associated risk factors 2.9.
Dr. Maurizio Pocchiari, representative of the former EuroCJD Network,
managed by the ECDC, presented to the Network a recent analysis on the
distribution and trends of human vCJD cases. He discussed vCJD case definition,
clinical and diagnostic features, including differences between EU countries,
and risk factors for vCJD development. Dr. Pocchiari recommended continued
surveillance of all forms of CJD in the EU, also to allow a better study and
comparison of human and animal TSE epidemiological data.
Update on the OIE activities in the field of TSE 2.10.
Dr. Derek Belton, representative of the OIE, explained to the Network the
procedure for setting OIE standards and provided an update on the recent
activities carried out at the OIE in relation to TSE. In 2015 the OIE
Terrestrial Animal Health Code was updated to specifically exclude atypical BSE
from the OIE process of official BSE risk status recognition ‘as a condition
believed to occur spontaneously in all cattle populations at a very low rate’.
Update on the activities of the European Commission in the field of
2.11.TSE
The representative of the European Commission (DG SANTE) updated the
Network on the recent TSE-related risk management activities in the European
Commission, as a follow-up of the TSE Road Map 2, in relation to feed ban,
specified risk material, TSE surveillance in cattle and small ruminants, and
other measures on scrapie eradication and intra-EU trade. The European
Commission is planning to send EFSA new mandates in relation to genetic
resistance to classical scrapie in goats and an update on a past EFSA risk
assessment of the BSE risk in processed animal proteins. Representatives of
Member States and of the European Commission discussed recent developments of
the TSE surveillance and control measures implemented in the EU and their
possible impact.
Future EFSA involvement in drafting the Annual report on TSE
2.12.monitoring in the EU
The EFSA BIOCONTAM Secretariat updated the Network on the future transfer
from the European Commission to EFSA of the responsibility for the preparation
of the annual report on animal TSE surveillance in the EU (as of 2016, with
respect to the analysis of 2015 data), and related data collection (as of 2017,
with respect to 2016 data). In relation to 2015, no changes are expected for
Member States on the collection and transmission of data to the European
Commission, while in future years EFSA will reflect on the opportunity to adjust
the requirements and the system for data collection.
Annual report of the Scientific Network on BSE-TSE
www.efsa.europa.eu/publications 9 EFSA Supporting publication
2015:EN-915
Information on a Workshop on TSE in Italy 2.13.
The representatives of Italy provided to the Network information with
respect to a Workshop on TSE that is going to take place on 10 December 2015 in
Turin (Italy), organised by the Italian National Reference Centre for TSE, and
intended for EU Candidate and Potential Candidate Countries.
3. Planned Network activities for 2016
Network members were invited to provide, also after the meeting,
suggestions for improvement of the functioning of the Network and possible
issues for future discussion in the Network.
The next meeting of the EFSA Network on BSE-TSE will be organised during
the third quarter of 2016.
snip...
Maximizing profits is all that is going on now, thanks to the OIE BSE MRR
policy, the legal trading of all strains of TSE prion disease globally. ...Terry
S. Singeltary Sr.
atypical BSE spontaneous sporadic ???
Saturday, May 26, 2012
Are USDA assurances on mad cow case 'gross oversimplification'?
SNIP...
*** What irks many scientists is the USDA’s April 25 statement that the
rare disease is “not generally associated with an animal consuming infected
feed.”
*** The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul
Brown, one of the world’s experts on this type of disease who retired recently
from the National Institutes of Health.
*** "(The agency) has no foundation on which to base that statement.”
*** “We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an
official with the USDA during the Clinton Administration now at Mississippi
State.
*** In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows
the origins of atypical cases of BSE,” she said
*** The argument about feed is critical because if feed is the cause, not a
spontaneous mutation, the California cow could be part of a larger outbreak.
SNIP...
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
Singeltary et al
31 Jan 2015 at 20:14 GMT
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics
of BSE in Canada Singeltary reply ;
*** It also suggests a similar cause or source for atypical BSE in these
countries. ***
Discussion: The C, L and H type BSE cases in Canada exhibit molecular
characteristics similar to those described for classical and atypical BSE cases
from Europe and Japan.
*** This supports the theory that the importation of BSE contaminated
feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these
countries. ***
see page 176 of 201 pages...tss
spontaneous atypical BSE ???
don’t let anyone fool you. spontaneous TSE prion disease is a hoax in
natural cases, never proven.
all one has to do is look at France. France is having one hell of an
epidemic of atypical BSE, probably why they stopped testing for BSE, problem
solved $$$ same as the USA, that’s why they stopped testing for BSE mad cow
disease in numbers they could find any with, after those atypical BSE cases
started showing up. shut down the testing to numbers set up by OIE that are so
low, you could only by accident find a case of BSE aka mad cow disease. and this
brilliant idea by the WHO et al, to change the name of mad cow disease, thinking
that might change things is preposterous. it’s all about money now folks, when
the OIE, USDA and everyone else went along and made the TSE prion disease aka
mad cow type disease a legal trading commodity by the BSE MRR policy, I would
say everyone bit off more then they can chew, and they will just have to digest
those TSE Prions coming from North America, and like it, and just prey you don’t
get a mad cow type disease i.e. Transmissible Spongiform Encephalopathy TSE
prion disease in the decades to come, and or pass it to some other poor soul via
the iatrogenic medical surgical tissue friendly fire mode of transmission i.e.
second hand transmission. it’s real folks, just not documented much, due to lack
of trace back efforts. all iatrogenic cjd is, is sporadic cjd, until the
iatrogenic event is tracked down and documented, and put into the academic and
public domain, which very seldom happens. ...
As of December 2011, around 60 atypical BSE cases have currently been
reported in 13 countries, *** with over one third in France.
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Transmission of scrapie prions to primate after an extended silent
incubation period
Authors
item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire,
Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item
Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item
Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron,
Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item
Deslys, Jean-Philippe -
Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal
Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015
Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E.,
Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C.,
Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J.
2015. Transmission of scrapie prions to primate after an extended silent
incubation period. Scientific Reports. 5:11573.
Interpretive Summary:
The transmissible spongiform encephalopathies (also called prion diseases)
are fatal neurodegenerative diseases that affect animals and humans. The agent
of prion diseases is a misfolded form of the prion protein that is resistant to
breakdown by the host cells. Since all mammals express prion protein on the
surface of various cells such as neurons, all mammals are, in theory, capable of
replicating prion diseases. One example of a prion disease, bovine spongiform
encephalopathy (BSE; also called mad cow disease), has been shown to infect
cattle, sheep, exotic undulates, cats, non-human primates, and humans when the
new host is exposed to feeds or foods contaminated with the disease agent. The
purpose of this study was to test whether non-human primates (cynomologous
macaque) are susceptible to the agent of sheep scrapie. After an incubation
period of approximately 10 years a macaque developed progressive clinical signs
suggestive of neurologic disease. Upon postmortem examination and microscopic
examination of tissues, there was a widespread distribution of lesions
consistent with a transmissible spongiform encephalopathy. This information will
have a scientific impact since it is the first study that demonstrates the
transmission of scrapie to a non-human primate with a close genetic relationship
to humans. This information is especially useful to regulatory officials and
those involved with risk assessment of the potential transmission of animal
prion diseases to humans.
Technical Abstract:
Classical bovine spongiform encephalopathy (c-BSE) is an animal prion
disease that also causes variant Creutzfeldt-Jakob disease in humans. Over the
past decades, c-BSE's zoonotic potential has been the driving force in
establishing extensive protective measures for animal and human health. In
complement to the recent demonstration that humanized mice are susceptible to
scrapie, we report here the first observation of direct transmission of a
natural classical scrapie isolate to a macaque after a 10-year incubation
period. Neuropathologic examination revealed all of the features of a prion
disease: spongiform change, neuronal loss, and accumulation of PrPres throughout
the CNS.
***This observation strengthens the questioning of the harmlessness of
scrapie to humans, at a time when protective measures for human and animal
health are being dismantled and reduced as c-BSE is considered controlled and
being eradicated. Our results underscore the importance of precautionary and
protective measures and the necessity for long-term experimental transmission
studies to assess the zoonotic potential of other animal prion strains.
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats
SUMMARY: We are reopening the comment period for our proposed rule that
would revise completely the scrapie regulations, which concern the risk groups
and categories established for individual animals and for flocks, the use of
genetic testing as a means of assigning risk levels to animals, movement
restrictions for animals found to be genetically less susceptible or resistant
to scrapie, and recordkeeping requirements. This action will allow interested
persons additional time to prepare and submit comments.
DATES: The comment period for the proposed rule published on September 10,
2015 (80 FR 54660-54692) is reopened. We will consider all comments that we
receive on or before December 9, 2015. ...
COMMENT SUBMISSION TERRY S. SINGELTARY SR.
WITH regards to Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats, I
kindly submit the following ;
>>>The last major revision of the scrapie regulations occurred on
August 21, 2001, when we published in theFederal Register(66 FR 43964, Docket
No. 97-093-5) a final rule amending part 79 by imposing additional restrictions
on the interstate movement of sheep and goats.<<<
Indeed, much science has changed about the Scrapie TSE prion, including
more science linking Scrapie to humans. sadly, politics, industry, and trade,
have not changed, and those usually trump sound science, as is the case with all
Transmissible Spongiform Encephalopathy TSE Prion disease in livestock producing
animals and the OIE. we can look no further at the legal trading of the Scrapie
TSE prion both typical and atypical of all strains, and CWD all stains. With as
much science of old, and now more new science to back this up, Scrapie of all
types i.e. atypical and typical, BSE all strains, and CWD all strains, should be
regulated in trade as BSE TSE PRION. In fact, I urge APHIS et al and the OIE,
and all trading partners to take heed to the latest science on the TSE prion
disease, all of them, and seriously reconsider the blatant disregards for human
and animal health, all in the name of trade, with the continued relaxing of TSE
Prion trade regulations through the ‘NEGLIGIBLE BSE RISK’ PROGRAM, which was set
up to fail in the first place. If the world does not go back to the ‘BSE RISK
ASSESSMENTS’, enhance, and or change that assessment process to include all TSE
prion disease, i.e. ‘TSE RISK ASSESSMENT’, if we do not do this and if we
continue this farce with OIE and the USDA et al, and the ‘NEGLIGIBLE BSE RISK’
PROGRAM, we will never eradicate the TSE prion aka mad cow type disease, they
will continue to mutate and spread among species of human and animal origin, and
they will continue to kill. ...
please see ;
O.05: Transmission of prions to primates after extended silent incubation
periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni,
Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys
Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies
reputed to be transmissible under field conditions since decades. The
transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that
an animal PD might be zoonotic under appropriate conditions. Contrarily, in the
absence of obvious (epidemiological or experimental) elements supporting a
transmission or genetic predispositions, PD, like the other proteinopathies, are
reputed to occur spontaneously (atpical animal prion strains, sporadic CJD
summing 80% of human prion cases). Non-human primate models provided the first
evidences supporting the transmissibiity of human prion strains and the zoonotic
potential of BSE. Among them, cynomolgus macaques brought major information for
BSE risk assessment for human health (Chen, 2014), according to their
phylogenetic proximity to humans and extended lifetime. We used this model to
assess the zoonotic potential of other animal PD from bovine, ovine and cervid
origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical
scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD,
albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked
in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases. We will present an
updated panorama of our different transmission studies and discuss the
implications of such extended incubation periods on risk assessment of animal PD
for human health.
===============
***thus questioning the origin of human sporadic cases***
===============
***This information will have a scientific impact since it is the first
study that demonstrates the transmission of scrapie to a non-human primate with
a close genetic relationship to humans. This information is especially useful to
regulatory officials and those involved with risk assessment of the potential
transmission of animal prion diseases to humans.
***This observation strengthens the questioning of the harmlessness of
scrapie to humans, at a time when protective measures for human and animal
health are being dismantled and reduced as c-BSE is considered controlled and
being eradicated. Our results underscore the importance of precautionary and
protective measures and the necessity for long-term experimental transmission
studies to assess the zoonotic potential of other animal prion strains.
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Evaluation of the zoonotic potential of transmissible mink
encephalopathy
Authors
item Comoy, Emmanuel - item Mikol, Jacqueline - item Ruchoux,
Marie-Madeleine - item Durand, Valerie - item Luccantoni-Freire, Sophie - item
Dehen, Capucine - item Correia, Evelyne - item Casalone, Cristina - item Richt,
Juergen item Greenlee, Justin item Torres, Juan Maria - item Brown, Paul - item
Deslys, Jean-Philippe -
Submitted to: Pathogens Publication Type: Peer Reviewed Journal Publication
Acceptance Date: July 30, 2013 Publication Date: July 30, 2013 Citation: Comoy,
E.E., Mikol, J., Ruchoux, M., Durand, V., Luccantoni-Freire, S., Dehen, C.,
Correia, E., Casalone, C., Richt, J.A., Greenlee, J.J., Torres, J.M., Brown, P.,
Deslys, J. 2013. Evaluation of the zoonotic potential of transmissible mink
encephalopathy. Pathogens. 2:(3)520-532.
Interpretive Summary: Cases of bovine spongiform encephalopathy (BSE) or
mad cow disease can be subclassified into at least 3 distinct disease forms with
the predominate form known as classical BSE and the others collectively referred
to as atypical BSE. Atypical BSE can be further subdivided into H-type and
L-type cases that are distinct from classical BSE and from each other. Both of
the atypical BSE subtypes are believed to occur spontaneously, whereas classical
BSE is spread through feeding contaminated meat and bone meal to cattle.
Transmissible mink encephalopathy (TME) is another prion disease that transmits
to cattle and show similarities to L-type BSE when subjected to laboratory
testing. The purpose of this study was to use non-human primates (cynomologous
macaque) and transgenic mice expressing the human prion protein to determine if
TME could represent a potential risk to human health. TME from two sources
(cattle and raccoons) was able to infect non-human primates and transgenic mice
after exposure by the intracranial route. This result suggest that humans may be
able to replicate TME prions after an exposure that allows infectious material
access to brain tissue. At this time, it is unknown whether non-human primates
or transgenic mice would be susceptible to TME prions after oral exposure. The
results obtained in these animal models were similar to those obtained for
L-type BSE. Although rare, the existence of TME and that it transmits to cattle,
non-human primates, and transgenic mice suggest that feed bans preventing the
feeding of mammalian tissues to cattle should stay in place and that regular
prion surveillance during the slaughter should remain in place. Parties with
interest in the cattle and beef industries and regulatory officials responsible
for safe feeding practices of cattle will be interested in this work. Technical
Abstract: Successful transmission of Transmissible Mink Encephalopathy (TME) to
cattle supports the bovine hypothesis to the still controversial origin of TME
outbreaks. Human and primate susceptibility to classical Bovine Spongiform
Encephalopathy (c-BSE) and the transmissibility of L-type BSE to macaques assume
a low cattle-to-primate species barrier: we therefore evaluated the zoonotic
potential of cattle-adapted TME. In less than two years, this strain induced in
cynomolgus macaques a neurological disease similar to L-BSE and distinct from
c-BSE. TME derived from another donor species (raccoon) induced a similar
disease with shorter incubation periods.
*** L-BSE and cattle-adapted TME were also transmissible to transgenic mice
expressing human PrP. Interestingly, secondary transmissions to transgenic mice
expressing bovine PrP showed the maintenance of prion strain features for the
three tested bovine prion strains (cattle TME, c-BSE and L-BSE) regardless of
intermediate host.
*** Thus, TME is the third animal prion strain transmissible to both
macaques and humanized transgenic mice, suggesting zoonotic potentials that
should be considered in the risk analysis of animal prion diseases for human
health.
*** Moreover, the similarities between TME and L-BSE are highly suggestive
of a link between those strains, and of the presence of L-BSE decades prior to
its identification in USA and Europe.
Research Project: Transmission, Differentiation, and Pathobiology of
Transmissible Spongiform Encephalopathies 2014 Annual Report
1a.Objectives (from AD-416): 1. Investigate the pathobiology of atypical
transmissible spongiform encephalopathies (TSEs) in natural hosts. A.
Investigate the pathobiology of atypical scrapie. B. Investigate the
pathobiology of atypical bovine spongiform encephalopathy (BSE). 2. Investigate
the horizontal transmission of TSEs. A. Assess the horizontal transmission of
sheep scrapie in the absence of lambing. B. Determine routes of transmission in
chronic wasting disease (CWD) infected premises. C. Assess oral transmission of
CWD in reindeer. 3. Investigate determinants of CWD persistence. A. Determine
CWD host range using natural routes of transmission. B. Investigate the
pathobiology of CWD.
1b.Approach (from AD-416): The studies will focus on three animal
transmissible spongiform encephalopathy (TSE) agents found in the United States:
bovine spongiform encephalopathy (BSE); scrapie of sheep and goats; and chronic
wasting disease (CWD) of deer, elk, and moose. The research will address sites
of accumulation, routes of infection, environmental persistence, and ante mortem
diagnostics with an emphasis on controlled conditions and natural routes of
infection. Techniques used will include clinical exams, histopathology,
immunohistochemistry and biochemical analysis of proteins. The enhanced
knowledge gained from this work will help mitigate the potential for
unrecognized epidemic expansions of these diseases in populations of animals
that could either directly or indirectly affect food animals.
3.Progress Report: Research efforts directed toward meeting objective 1 of
our project plan, Investigate the pathobiology of atypical transmissible
spongiform encephalopathies (TSEs) in natural hosts, include work in previous
years starting with the inoculation of animals for studies designed to address
the pathobiology of atypical scrapie, atypical bovine spongiform encephalopathy
(BSE), as well as a genetic version of BSE. Animals inoculated with atypical
scrapie have not yet developed disease. Atypical BSE animals have developed
disease and evaluation of the samples is currently underway. Animals inoculated
with a genetic version of BSE have developed disease and the manuscript has been
published (2012). In addition, we have investigated the possibility that
atypical scrapie was present earlier than previously detected in the national
flock by analyzing archived field isolates using methods that were unavailable
at the time of original diagnosis. Sample quality was sufficiently degraded that
modern methods were not suitable for evaluation. In research pertaining to
objective 2, Investigate the horizontal transmission of TSEs, we have initiated
a study to determine if cohousing non-lambing scrapie inoculated sheep is
sufficient to transmit scrapie to neonatal lambs. At this time, scrapie free
ewes have lambed in the presence of scrapie inoculated animals and the lambs are
cohoused with these inoculated animals.
4.Accomplishments 1. Evaluated enzyme immunoassay for rapid identification
of prion disease in livestock. Scrapie of sheep and bovine spongiform
encephalopathy of cattle are diseases that cause damage to the central nervous
system including the retina in the eye. The infectious agent is an abnormal
protein called a prion that has misfolded from its normal state and is resistant
to breakdown by the host cells. Current diagnostic methods require the testing
of brain material, which can be difficult to collect and may lead to
contamination of the environment and exposure of personnel to the infectious
agent. Eyes can be readily collected without opening the skull. ARS researchers
at Ames, Iowa demonstrated that the enzyme immunoassay results using eyes of
negative controls or samples collected from sheep or cattle with clinical signs
were in agreement with approved confirmatory assays (western blot or
immunohistochemistry). These results indicate the retina is a useful tissue for
rapid diagnosis of prion disease in clinically ill sheep and cattle and could be
considered to greatly increase the number of samples submitted for prion disease
diagnosis with a minimal investment of time and limited exposure of personnel to
prion agents.
2. Evaluated E211K cattle as a model for inherited human prion disease.
Prion diseases cause damage to the central nervous system of animals and humans.
The infectious agent is an abnormal protein called a prion that has misfolded
from its normal state and is resistant to breakdown by the host cells and thus
accumulates and damages those cells. Some forms of prion disease are genetic and
can be inherited. Current models of genetic prion disease in humans rely on
mouse models expressing either the human prion protein (E200K) or a combination
of both mouse and human sequences. In addition to being an entirely artificial
system these mouse models have a short lifespan making them a less than ideal
system to study a naturally occurring genetic disorder with a long incubation
time and late onset of disease. Cattle, however, exhibit a number of
similarities to humans with regard to prion disease and perhaps most notable is
the late onset of genetic prion disease. ARS researchers at Ames, Iowa have
produced cattle containing both 1 and 2 chromosome copies of the cattle prion
gene (E211K) and evaluated many aspects of this prion protein from cattle
including protein stability, protein expression levels and ratios, as well as
evidence of oxidative stress. Taken together, these results highlight the
differences between mouse models of genetic prion disease and a naturally
occurring prion disease system in cattle and suggest that cattle will provide a
more relevant understanding of genetic prion disease in humans than do current
rodent models.
Review Publications Smith, J.D., Greenlee, J.J. 2014. Detection of
misfolded prion protein in retina samples of sheep and cattle by use of a
commercially available enzyme immunoassay. American Journal of Veterinary
Research. 75(3):268-272. Haldar, S., Beveridge, A.J., Wong, J., Singh, A.J.,
Galimberti, D., Borroni, D., Zhu, X., Blevins, J., Greenlee, J., Perry, G.,
Mukhopadhyay, C.K., Schmotzer, C., Singh, N. 2014. A low-molecular-weight
ferroxidase is increased in the CSF of sCJD Cases: CSF ferroxidase and
transferrin as diagnostic biomarkers for sCJD. Antioxidants & Redox
Signaling. 19(14):1662-1675.
http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=421870&fy=2014
http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=421870
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Scrapie transmits to white-tailed deer by the oral route and has a
molecular profile similar to chronic wasting disease
Authors
item Greenlee, Justin item Moore, S - item Smith, Jodi - item Kunkle,
Robert item West Greenlee, M -
Submitted to: American College of Veterinary Pathologists Meeting
Publication Type: Abstract Only Publication Acceptance Date: August 12, 2015
Publication Date: N/A Technical Abstract: The purpose of this work was to
determine susceptibility of white-tailed deer (WTD) to the agent of sheep
scrapie and to compare the resultant PrPSc to that of the original inoculum and
chronic wasting disease (CWD). We inoculated WTD by a natural route of exposure
(concurrent oral and intranasal (IN); n=5) with a US scrapie isolate. All
scrapie-inoculated deer had evidence of PrPSc accumulation. PrPSc was detected
in lymphoid tissues at preclinical time points, and deer necropsied after 28
months post-inoculation had clinical signs, spongiform encephalopathy, and
widespread distribution of PrPSc in neural and lymphoid tissues. Western
blotting (WB) revealed PrPSc with 2 distinct molecular profiles. WB on cerebral
cortex had a profile similar to the original scrapie inoculum, whereas WB of
brainstem, cerebellum, or lymph nodes revealed PrPSc with a higher profile
resembling CWD. Homogenates with the 2 distinct profiles from WTD with clinical
scrapie were further passaged to mice expressing cervid prion protein and
intranasally to sheep and WTD. In cervidized mice, the two inocula have distinct
incubation times. Sheep inoculated intranasally with WTD derived scrapie
developed disease, but only after inoculation with the inoculum that had a
scrapie-like profile. The WTD study is ongoing, but deer in both inoculation
groups are positive for PrPSc by rectal mucosal biopsy. In summary, this work
demonstrates that WTD are susceptible to the agent of scrapie, two distinct
molecular profiles of PrPSc are present in the tissues of affected deer, and
inoculum of either profile readily passes to deer.
Monday, November 16, 2015
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
*** 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
***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.***
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
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were
exposed to the infectious agents only by their nonforced consumption of known
infectious tissues. The asymptomatic incubation period in the one monkey exposed
to the virus of kuru was 36 months; that in the two monkeys exposed to the virus
of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the
two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively.
Careful physical examination of the buccal cavities of all of the monkeys failed
to reveal signs or oral lesions. One additional monkey similarly exposed to kuru
has remained asymptomatic during the 39 months that it has been under
observation.
snip...
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie
by natural feeding to squirrel monkeys that we have reported provides further
grounds for concern that scrapie-infected meat may occasionally give rise in
humans to Creutzfeldt-Jakob disease.
PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
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
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC.
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK
National Institute of Neurological Diseases and Stroke, National Institutes
of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey
(Macaca fascicularis) with an incubation period of more than 5 yr from the time
of intracerebral inoculation of scrapie-infected mouse brain. The animal
developed a chronic central nervous system degeneration, with ataxia, tremor and
myoclonus with associated severe scrapie-like pathology of intensive astroglial
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of
grey matter. The strain of scrapie virus used was the eighth passage in Swiss
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton,
Berkshire).
Singeltary Comment
*** Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy;
Importation of Animals and Animal Products Singeltary Submission ;
Monday, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH
CODE
Thursday, December 20, 2012
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED
Friday, February 04, 2011
NMLB and USDA allow scrapie prion infected mutton to enter food chain on
the Navajo Reservation in New Mexico
***Our study demonstrates susceptibility of adult cattle to oral
transmission of classical BSE. ***
***our findings suggest that possible transmission risk of H-type BSE to
sheep and human. ***
P.86: Estimating the risk of transmission of BSE and scrapie to ruminants
and humans by protein misfolding cyclic amplification
Morikazu Imamura, Naoko Tabeta, Yoshifumi Iwamaru, and Yuichi Murayama
National Institute of Animal Health; Tsukuba, Japan
To assess the risk of the transmission of ruminant prions to ruminants and
humans at the molecular level, we investigated the ability of abnormal prion
protein (PrPSc) of typical and atypical BSEs (L-type and H-type) and typical
scrapie to convert normal prion protein (PrPC) from bovine, ovine, and human to
proteinase K-resistant PrPSc-like form (PrPres) using serial protein misfolding
cyclic amplification (PMCA).
Six rounds of serial PMCA was performed using 10% brain homogenates from
transgenic mice expressing bovine, ovine or human PrPC in combination with PrPSc
seed from typical and atypical BSE- or typical scrapie-infected brain
homogenates from native host species. In the conventional PMCA, the conversion
of PrPC to PrPres was observed only when the species of PrPC source and PrPSc
seed matched. However, in the PMCA with supplements (digitonin, synthetic polyA
and heparin), both bovine and ovine PrPC were converted by PrPSc from all tested
prion strains. On the other hand, human PrPC was converted by PrPSc from typical
and H-type BSE in this PMCA condition.
Although these results were not compatible with the previous reports
describing the lack of transmissibility of H-type BSE to ovine and human
transgenic mice, ***our findings suggest that possible transmission risk of
H-type BSE to sheep and human. Bioassay will be required to determine whether
the PMCA products are infectious to these animals.
================
==========================================
***our findings suggest that possible transmission risk of H-type BSE to
sheep and human. Bioassay will be required to determine whether the PMCA
products are infectious to these animals.
==========================================
PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS
*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE ***
O18
Zoonotic Potential of CWD Prions
Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1,
Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve
University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy,
3Encore Health Resources, Houston, Texas, USA
*** These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.
==================
***These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.***
==================
P.105: RT-QuIC models trans-species prion transmission
Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover
Prion Research Center; Colorado State University; Fort Collins, CO USA
Conversely, FSE maintained sufficient BSE characteristics to more
efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was
competent for conversion by CWD and fCWD.
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.
================
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.***
================
*** BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION REPORT DECEMBER 14, 2015
***
*** CHRONIC WASTING DISEASE CWD TSE PRION REPORT DECEMBER 14, 2015
***
*** CREUTZFELDT JAKOB DISEASE CJD TSE PRION REPORT DECEMBER 14, 2015
***
*** NOTICE: Environmental Impact Statement on Large Livestock Carcasses TSE
Prion REPORT December 14, 2015 ***
Tuesday, December 15, 2015
Chronic Wasting Disease will cause a Wyoming deer herd to go virtually
extinct in 41 years, a five-year study predicts
Study: Chronic Wasting Disease kills 19% of deer herd annually
Wednesday, December 16, 2015
Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge
Spencers Lane BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE
R. G. WILL
1984
*** The association between venison eating and risk of CJD shows similar
pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK
OF CJD (p = 0.04). (SEE LINK IN REPORT HERE...TSS) PLUS, THE CDC DID NOT PUT
THIS WARNING OUT FOR THE WELL BEING OF THE DEER AND ELK ;
snip...
IN CONFIDENCE
PERCEPTIONS OF UNCONVENTIONAL SLOW VIRUS DISEASES IN THE USA
GAH WELLS
REPORT OF A VISIT TO THE USA APRIL-MAY 1989
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed.
Dr. A Thiermann showed the picture in the ''Independent'' with cattle being
incinerated and thought this was a fanatical incident to be avoided in the US at
all costs.
SNIP...
”The occurrence of CWD must be viewed against the contest of the locations
in which it occurred. It was an incidental and unwelcome complication of the
respective wildlife research programmes. Despite it’s subsequent recognition as
a new disease of cervids, therefore justifying direct investigation, no specific
research funding was forthcoming. The USDA veiwed it as a wildlife problem and
consequently not their province!” page 26.
***atypical spontaneous BSE in France LOL***
FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many
spontaneous events of mad cow disease $$$
***so 20 cases of atypical BSE in France, compared to the remaining 40
cases in the remaining 12 Countries, divided by the remaining 12 Countries,
about 3+ cases per country, besides Frances 20 cases. you cannot explain this
away with any spontaneous BSe. ...TSS
Sunday, October 5, 2014
France stops BSE testing for Mad Cow Disease
***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67
PrPsc was not detected using rapid tests for BSE.
***Subsequent testing resulted in the detection of pathologic lesion in
unusual brain location and PrPsc detection by PMCA only.
IBNC Tauopathy or TSE Prion disease, it appears, no one is sure
Posted by flounder on 03 Jul 2015 at 16:53 GMT
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26
April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated
online 11 September 2015 Erratum (October, 2015)
*** Evidence for human transmission of amyloid-β pathology and cerebral
amyloid angiopathy
07 02:27 AM
re-Evidence for human transmission of amyloid-? pathology and cerebral
amyloid angiopathy
*** Terry S. Singeltary Sr. said:
I would kindly like to comment on the Nature Paper, the Lancet reply, and
the newspaper articles.
snip...see full text ;
Subject: 1992 IN CONFIDENCE TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO
PRIMATES POSSIBILITY ON A TRANSMISSIBLE PRION REMAINS OPEN
BSE101/1 0136
IN CONFIDENCE
CMO
From: . Dr J S Metiers DCMO
4 November 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
snip...
4. The other dimension to consider is the public reaction. To some extent
the GSS case demonstrates little more than the transmission of BSE to a pig by
intra-cerebral injection. If other prion diseases can be transmitted in this way
it is little surprise that some pathological findings observed in GSS were also
transmissible to a marmoset. But the transmission of features of Alzheimer's
pathology is a different matter, given the much greater frequency of this
disease and raises the unanswered question whether some cases are the result of
a transmissible prion. The only tenable public line will be that "more research
is required’’ before that hypothesis could be evaluated. The possibility on a
transmissible prion remains open. In the meantime MRC needs carefully to
consider the range and sequence of studies needed to follow through from the
preliminary observations in these two cases. Not a particularly comfortable
message, but until we know more about the causation of Alzheimer's disease the
total reassurance is not practical.
J S METTERS Room 509 Richmond House Pager No: 081-884 3344 Callsign: DOH
832 llllYc!eS 2 92/11.4/1.2
>>> The only tenable public line will be that "more research is
required’’ <<<
>>> possibility on a transmissible prion remains
open<<<
O.K., so it’s about 23 years later, so somebody please tell me, when is
"more research is required’’ enough time for evaluation ?
Self-Propagative Replication of Ab Oligomers Suggests Potential
Transmissibility in Alzheimer Disease
Received July 24, 2014; Accepted September 16, 2014; Published November 3,
2014
*** Singeltary comment PLoS ***
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion
disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
Wednesday, September 2, 2015
Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses
in an Alzheimer’s Disease Database
*** CREUTZFELDT JAKOB DISEASE CJD TSE PRION REPORT DECEMBER 14, 2015
***
USDA Announces Preliminary Concurrence with OIE Risk Designations for BSE
in 16 Countries USDA Animal and Plant Health Inspection Service sent this
bulletin at 12/04/2015 11:15 AM EST
USDA Announces Preliminary Concurrence with World Animal Health
Organization Risk Designations for Bovine Spongiform Encephalopathy in 16
Countries
December 4, 2015—The United States Department of Agriculture’s Animal and
Plant Health Inspection Service (APHIS) is preliminarily concurring with the
World Organization for Animal Health’s (OIE) bovine spongiform encephalopathy
(BSE) risk designations for 16 countries. The OIE recognizes these regions as
being of negligible risk for BSE. APHIS reviewed the information supporting the
OIE’s risk designations for these regions and agrees with the OIE designations.
The 16 countries are: Bulgaria, Cyprus, Czech Republic, Estonia, France,
India, Korea (Republic of), Hungary, Latvia, Liechtenstein, Luxembourg, Malta,
Portugal, Romania, Slovakia, and Switzerland.
The OIE recommendations regarding each of the above countries can be viewed
online.
APHIS considers all countries of the world to fall into in one of three BSE
risk categories: negligible risk, controlled risk, or undetermined risk. Any
region that is not classified by APHIS as presenting either negligible risk or
controlled risk for BSE is considered to present an undetermined risk.
Under the regulations, APHIS may classify a region for BSE in one of two
ways. One way is for countries that have not received a risk classification from
the World Organization for Animal Health (OIE) to request classification by
APHIS. The other way is for APHIS to concur with the classification given to a
country by the OIE.
This notice is available for 60 days for review and comment. APHIS will
consider all comments received on or before February 2, 2016. After reviewing
any comments we receive, we will announce our final determination regarding the
BSE classification of these countries in the Federal Register.
LMAO !!! pot calling kettle black. BSE MRR policy equals the legal trading
of all strains of TSE Prion aka mad cow disease. any consumers death there from
are now acceptable $$$
these blogs are for educational use. I do not advertise or make money from
them.
MOM DOD December 14, 1997 confirmed hvCJD, just made a promise to mom,
never forget, and never let them forget.
carry on with this charade or masquerade God save the industry at all cost
mentality $$$ but be sure, history should be very cruel to those that have been
involved, with no soul or courage to come forward. ...terry
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14,
2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1
reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD)
has been stable since 1985. These estimates, however, are based only on reported
cases, and do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number of
persons with a diagnosis of Alzheimer disease in fact may have CJD, although
only a small number of these patients receive the postmortem examination
necessary to make this diagnosis. Furthermore, only a few states have made CJD
reportable. Human and animal transmissible spongiform encephalopathies should be
reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment
on the CDC's attempts to monitor the occurrence of emerging forms of CJD.
Asante, Collinge et al [1] have reported that BSE transmission to the
129-methionine genotype can lead to an alternate phenotype that is
indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD
and all human TSEs are not reportable nationally. CJD and all human TSEs must be
made reportable in every state and internationally. I hope that the CDC does not
continue to expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in the USA
in both animal and man. CWD in deer/elk is spreading rapidly and CWD does
transmit to mink, ferret, cattle, and squirrel monkey by intracerebral
inoculation. With the known incubation periods in other TSEs, oral transmission
studies of CWD may take much longer. Every victim/family of CJD/TSEs should be
asked about route and source of this agent. To prolong this will only spread the
agent and needlessly expose others. In light of the findings of Asante and
Collinge et al, there should be drastic measures to safeguard the medical and
surgical arena from sporadic CJDs and all human TSEs. I only ponder how many
sporadic CJDs in the USA are type 2 PrPSc?
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003
doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI
Tracking spongiform encephalopathies in North America
Original
Xavier Bosch
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever
since. What I have found is that we have not been told the truth. CWD in deer
and elk is a small portion of a much bigger problem.” 49-year—old Singeltary is
one of a number of people who have remained largely unsatisfied after being told
that a close relative died from a rapidly progressive dementia compatible with
spontaneous Creutzfeldt—Jakob ...
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...
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009
The Pathological Protein:
Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases
Philip Yam
''Answering critics like Terry Singeltary, who feels that the US
undercounts CJD, Schonberger _conceded_ that the current surveillance system has
errors but stated that most of the errors will be confined to the older
population''....end
P.S. I forgot a few grams of banned potential mad cow protein in commerce
here in the USA since the August 1997 mad cow feed ban...just saying, nothing
else matters except trade $$$
P.S.
10 years post mad cow feed ban August 1997
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN
COMMERCE USA 2007
Date: March 21, 2007 at 2:27 pm PST
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II
PRODUCT
Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried,
Recall # V-024-2007
CODE
Cattle feed delivered between 01/12/2007 and 01/26/2007
RECALLING FIRM/MANUFACTURER
Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
Firm initiated recall is ongoing.
REASON
Blood meal used to make cattle feed was recalled because it was cross-
contaminated with prohibited bovine meat and bone meal that had been
manufactured on common equipment and labeling did not bear cautionary BSE
statement.
VOLUME OF PRODUCT IN COMMERCE
42,090 lbs.
DISTRIBUTION
WI
___________________________________
PRODUCT
Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot-
Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M
CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B
DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal,
JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT
Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral,
BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC
LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall #
V-025-2007
CODE
The firm does not utilize a code - only shipping documentation with
commodity and weights identified.
RECALLING FIRM/MANUFACTURER
Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm
initiated recall is complete.
REASON
Products manufactured from bulk feed containing blood meal that was cross
contaminated with prohibited meat and bone meal and the labeling did not bear
cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE
9,997,976 lbs.
DISTRIBUTION
ID and NV
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
16 years post mad cow feed ban August 1997
2013
Sunday, December 15, 2013
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
17 years post mad cow feed ban August 1997
Tuesday, December 23, 2014
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION
Sunday, June 14, 2015
Larry’s Custom Meats Inc. Recalls Beef Tongue Products That May Contain
Specified Risk Materials BSE TSE Prion
*** Monday, October 26, 2015 ***
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE October 2015 ***
Thursday, July 24, 2014
*** Protocol for further laboratory investigations into the distribution of
infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical
BSE investigations
Terry S. Singeltary Sr. Bacliff, Texas USA 77518 flounder9@verizon.net
