-------- Original Message --------
Subject: Re: Mysterious BSE-like disease found in sheep (UK sheep goat export]
Date: Sat, 10 Apr 2004 09:52:54 -0500
From: "Terry S. Singeltary Sr." <flounder@wt.net>
Reply-To: Bovine Spongiform Encephalopathy <BSE-L@uni-karlsruhe.de>
To: BSE-L@uni-karlsruhe.de
References: <4076C1BD.7050602@wt.net>

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


I thought for those that have not seen these figures, for some, they
might be interesting to keep for your files for later use...



Terry S. Singeltary Sr. wrote:

> Mysterious BSE-like disease found in sheep
> 16:58 08 April 04
> NewScientist.com news service
> A massive research programme to find out whether BSE is circulating in
> British sheep has turned up its first suspicious result. But while
> scientists say the sheep did not have conventional BSE, they cannot rule
> out the possibility that it could have had a new form of mad cow disease
> that has adapted to sheep.
> Britain's Department for Environment, Food and Rural Affairs has
> announced that the Veterinary Laboratories Agency in Weybridge, England,
> had found "a type of scrapie not previously seen in the UK". Scrapie is
> a sheep disease similar to BSE which is not generally thought to harm
> people.
> DEFRA said the disease-causing prion detected in the sheep's brain "had
> some characteristics similar to experimental BSE in sheep", but that on
> other tests it resembled neither BSE nor "previously recognised types of
> scrapie".
> The UK's Food Standards Agency said in a statement: "Uncertainties still
> remain on this issue. However, based on the best scientific evidence to
> date, we are not advising against eating lamb and sheep meat."
> Meat and bone
> There have long been fears that sheep which ate cattle-derived meat and
> bone meal during Britain's BSE epidemic in the 1980s might have acquired
> BSE, although they have never been confirmed.
> Unlike BSE in cattle, prion diseases spread directly from sheep to
> sheep. So any BSE in sheep could still be circulating despite subsequent
> bans on animal-derived feed.
> Furthermore, sheep experimentally fed BSE develop a disease
> indistinguishable from ordinary scrapie, making detection very
> difficult. Yet the prion from such animals still behaves like BSE, and
> could cause the fatal human disease vCJD.
> Worse, sheep carry prions in more tissues than cattle, including the
> muscle that people eat, so BSE-infected sheep could cause more human
> disease than mad cows.
> A previous attempt to determine whether British sheep had acquired BSE
> went spectacularly wrong in 2001 when sheep and cattle brains were mixed
> up in the lab. But since then, the VLA has tested the brains of all 1019
> newly reported cases of scrapie, as well as 1125 scrapie brains dating
> back to 1998, with tests designed to distinguish scrapie from BSE.
> Blot test
> The new result announced on Wednesday, from a sheep recently reported
> with scrapie symptoms, is the first to give results that resembled BSE.
> Danny Matthews of the VLA told New Scientist that in a prion test called
> a western blot, the sheep's brain did not bind an antibody called P4. P4
> also does not bind prions from sheep experimentally infected with BSE,
> but does bind all but one forms of scrapie tested with it.
> Also like BSE, the form of the prion without a sugar attached to it had
> a lower molecular weight than the form found in scrapie. But the ratio
> of prions with different numbers of sugars on them looked like scrapie,
> not BSE, says Matthews.
> Most conclusively, immunohistochemistry (IHC), in which thin slices of
> the sheep's brain were stained with various antibodies, showed prions
> had accumulated in different parts of the brain and different kinds of
> cells from BSE - or any known form of scrapie.
> Passing change
> IHC seems to be a reliable indicator of BSE, as it has given a constant
> pattern in the 100 sheep of different genetic varieties experimentally
> infected with BSE and tested so far. But so little scrapie has been
> tested, says Matthews, it is not known if one strain might give these
> results on the tests.
> More on this story
> Subscribe
> to
> New Scientist for more news and features
> Related Stories
> vCJD deaths will rise if UK sheep have BSE
> 9 January 2002
> Bungled BSE experiments due to refrigerator mix-up
> 30 November 2001
> BSE experiment farce deepens
> 23 October 2001
> For more related stories
> search the print edition Archive
> Weblinks
> UK Department of Environment, Food and Rural Affairs
> Veterinary Laboratories Agency
> UK Food Standards Agency
> The IHC pattern reliably indicates BSE, says Matthews, having been
> constant in the 100 experimentally infected sheep of different genetic
> varieties tested so far. But so little scrapie has been tested, he says,
> it is not known if one strain might give these results on the tests.
> One possibility, he says, is that the sheep might have been carrying a
> prion initially derived from BSE. Passage into new species is well known
> to change prions.
> BSE from experimentally infected sheep has so far been passed to just
> one more round of sheep, with no apparent change. "But we don't know if
> passage through many sheep, of different genetic types, might change it
> so it no longer gives the same pattern in IHC or western blots," says
> Matthews. "Those experiments are underway now."
> Any such new incarnation of BSE in sheep may - or may not - have lost
> its ability to harm humans.
> Debora MacKenzie
> http://www.newscientist.com/news/news.jsp?id=ns99994869
> IF THERE is one categorical pronouncement you
> can safely make about prion diseases like BSE
> or CJD, it is that one should not make
> categorical pronouncements. "British beef is
> safe" and "there is no BSE in Germany" come
> to mind. Now there are two more: "scrapie is
> safe", and "people don't catch sporadic CJD".
> Scrapie is the most widespread prion
> disease, infecting untold numbers of
> sheep worldwide. Sporadic CJD is the
> old-fashioned pre-BSE kind that is supposed
> to happen spontaneously in unlucky people.
> But a surprise observation in France suggests
> some sCJD cases--though by no means all--may
> be linked to scrapie after all (see p 4).
> For years, British authorities asserted that
> BSE was harmless because it was a form of
> scrapie. In fact, the only evidence scrapie
> is safe is some broad-brush epidemiology, good
> as far as it goes but unable to reveal
> occasional risks for some people from some
> sheep. Alarm bells should have rung in 1980
> when researchers gave monkeys scrapie by
> feeding them infected brains. But that
> research, like so much other work on
> prion diseases, was never followed up.
> We still have little idea what BSE does
> in pigs and chickens. The Queniborough
> vCJD outbreak (see p 5) would be easier
> to understand if we knew how much brain
> we must eat to be infected. As for scrapie,
> it shouldn't take a chance finding to
> tell us that there may be dangerous sheep
> out there.
> Suspect symptoms
> What if you can catch old-fashioned CJD by
> eating meat from a sheep infected with
> scrapie?
> Exclusive from New Scientist magazine
> Four years ago, Terry Singeltary watched his
> mother die horribly from a degenerative brain disease.................
> full text url follows
> By Debora MacKenzie
> Suspect Symptoms
> http://www.newscientist.com/hottopics/bse/suspectsymptoms.jsp
> if url dead, go here for 'SUSPECT SYMPTOMS'
> you can access article here also;
> http://www.organicconsumers.org/meat/scrapiecjd.cfm
> http://www.vegancowboy.org/TSS-SuspectSymptoms.html
> Then follow up with PNAS studies from which
> new scientist article written from;
> Published online before print March 20, 2001
> Proc. Natl. Acad. Sci. USA, 10.1073/pnas.041490898
> Abstract of this Article
> Reprint (PDF) Version of this Article
> Similar articles found in:
> PNAS Online
> PubMed
> PubMed Citation
> Search Medline for articles by:
> Lasmézas, C. I. || Deslys, J.-P.
> Alert me when:
> new articles cite this article
> Download to Citation Manager
> Neurobiology
> Adaptation of the bovine spongiform encephalopathy agent to primates and
> comparison with Creutzfeldt- Jakob disease: Implications for human health
> Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Virginie Nouvel*,
> Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger
> ] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique
> Dormont*, and Jean-Philippe Deslys*
> * Commissariat à l'Energie Atomique, Service de Neurovirologie,
> Direction des Sciences du Vivant/Département de Recherche Medicale,
> Centre de Recherches du Service de Santé des Armées 60-68, Avenue du
> Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; [Dagger
> ] Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003
> Lyon, France; § Laboratoire de Neuropathologie, Hôpital de la
> Salpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶
> Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital,
> Crewe Road, Edinburgh EH4 2XU, United Kingdom; and [||] Institute for
> Animal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9
> 3JF, United Kingdom
> Edited by D. Carleton Gajdusek, Centre National de la Recherche
> Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000
> (received for review October 16, 2000)
> Abstract
> Top
> Abstract
> Introduction
> Materials and Methods
> Results
> Discussion
> Conclusions
> References
> There is substantial scientific evidence to support the notion that
> bovine spongiform encephalopathy (BSE) has contaminated human beings,
> causing variant Creutzfeldt-Jakob disease (vCJD). This disease has
> raised concerns about the possibility of an iatrogenic secondary
> transmission to humans, because the biological properties of the
> primate-adapted BSE agent are unknown. We show that (i) BSE can be
> transmitted from primate to primate by intravenous route in 25 months,
> and (ii) an iatrogenic transmission of vCJD to humans could be readily
> recognized pathologically, whether it occurs by the central or
> peripheral route. Strain typing in mice demonstrates that the BSE agent
> adapts to macaques in the same way as it does to humans and confirms
> that the BSE agent is responsible for vCJD not only in the United
> Kingdom but also in France. The agent responsible for French iatrogenic
> growth hormone-linked CJD taken as a control is very different from vCJD
> but is similar to that found in one case of sporadic CJD and one sheep
> scrapie isolate. These data will be key in identifying the origin of
> human cases of prion disease, including accidental vCJD transmission,
> and could provide bases for vCJD risk assessment.
> Introduction
> Top
> Abstract
> Introduction
> Materials and Methods
> Results
> Discussion
> Conclusions
> References
> The recognition of a variant of the human transmissible spongiform
> encephalopathy (TSE) Creutzfeldt-Jakob Disease (vCJD) in the U.K. in
> 1996 raised the major concern that it would correspond to human
> infection with the agent responsible for bovine spongiform
> encephalopathy (BSE; ref. 1). Transmission of BSE to macaques provided
> the first experimental evidence as it produced a disease close to vCJD
> in humans (2). Strain typing in inbred mice (consisting of measuring the
> incubation period and establishing lesion profiles corresponding to the
> strain-specific distribution of brain vacuolation) allows reliable
> identification of TSE strains (3). This method, together with
> biochemical methods, has revealed a single phenotype for the agents of
> BSE and the British cases of vCJD (4-6). Mice expressing only the bovine
> prion protein (PrP) were highly susceptible to vCJD and BSE, which
> induced the same disease (7). Thus, it is now well established that BSE
> has caused vCJD, probably by alimentary contamination. In this respect,
> the finding of abnormal PrP labeling in the gastrointestinal tract and
> lymphatic tissues of orally BSE-contaminated lemurs shows that the BSE
> agent can infect primates by the oral route (8). About 1 million
> contaminated cattle may have entered the human food chain, and the
> future number of vCJD cases could range from 63 to 136,000 depending on
> the incubation period of BSE in humans (9). Unlike sporadic CJD (sCJD)
> and iatrogenic CJD (iCJD) linked to the administration of contaminated
> growth hormone extracted from human hypophyses, in vCJD, the infectious
> agent seems to be widely distributed in lymphoid organs, as pathological
> PrP (PrPres) can be detected in tonsils, lymph nodes, spleen, and
> appendix even in the preclinical phase of the disease (10, 11). This
> raises a public health issue with regard to the risk of iatrogenic
> transmission of vCJD through surgical instruments, grafts, blood
> transfusion, or parenteral administration of biological products of
> human origin. However, this risk is difficult to assess, because it
> largely depends on factors such as the virulence of the BSE agent
> adapted to primates and the efficiency of secondary transmission to
> humans by a peripheral route such as the i.v. one. A further issue is
> whether vCJD accidentally acquired from humans would be recognized. The
> latter poses the question of a phenotypic variation of the BSE agent
> after successive transmissions in humans: does it retain its strain
> characteristics, and does it induce a pathology similar to that observed
> in the previous host? A 9-year history of transmission of BSE to
> primates and mice enables us today to clarify a number of these
> important points.
> Although BSE has mainly affected the U.K., two definite cases and one
> probable case of vCJD have now been reported in France in people who
> have never resided in the U.K. (12, 13). We strain-typed the first of
> these cases to establish its origin. Strain typing in C57BL/6 mice of
> BSE, French, and British vCJD was compared with that of BSE passaged in
> nonhuman primates, thus allowing us to study the effect of serial
> passages in primates. Comparisons were also made with French cases of
> sCJD and iCJD and two strains of scrapie (one of French and one of U.S.
> origin). Our findings provide experimental demonstration that the same
> agent, namely that responsible for the cattle disease BSE, has caused
> vCJD both in France and in the U.K., in line with biochemical data and
> with the fact that, until 1996, about 10% of the beef consumed in France
> was imported from the U.K. We found that the BSE agent in nonhuman
> primates is similar to that causing vCJD in humans and tends to evolve
> rapidly toward a primate-adapted variant. Furthermore, we showed that
> the strain responsible for iCJD is closely related to that of one
> patient with sCJD, and, more unexpectedly, that these agents were
> similar to the French scrapie strain studied (but different from the
> U.S. scrapie strain). This finding requires a cautious interpretation
> for several reasons, not least because of the inevitably limited number
> of TSE strains that can be studied by such a cumbersome method as strain
> typing. Nonetheless, it also prompts reconsideration of the possibility
> that, in some instances, sheep and human TSEs can share a common origin.
> snip...
> http://www.pnas.org/cgi/content/full/041490898v1

########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############

-------- Original Message --------
Subject: DEFRA INVESTIGATES AN UNUSUAL SCRAPIE CASE (similar to exp. BSE in sheep)
Date: Wed, 7 Apr 2004 08:56:36 -0500
From: "Terry S. Singeltary Sr." <flounder@wt.net>
Reply-To: Bovine Spongiform Encephalopathy <BSE-L@uni-karlsruhe.de>
To: BSE-L@uni-karlsruhe.de

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

Date: April 07, 2004 Time: 13:45


The Veterinary Laboratories Agency (VLA) have informed Defra, the
Devolved Administrations and the Food Standards Agency of a type of
scrapie not previously seen in the UK.

The VLA and other European laboratories with expertise in scrapie-like
diseases have now applied several rapid diagnostic methods to tissue
samples from a sheep with suspected scrapie. Some of the methods have
indicated that the case does not appear to resemble previously
recognised cases of scrapie and, although there were differences, it had
some characteristics similar to experimental BSE in sheep and also to an
experimental strain of sheep scrapie. More importantly, though,
microscopic analysis of brain material showed that the case neither
resembled previously recognised types of scrapie or experimental BSE in

A meeting of the scientific experts who performed these analyses, held
on the 30th March, concluded that this case could not be considered to
be BSE in sheep, although it does not behave like known types of scrapie
either. Further investigation will be needed before more can be said
about how this unusual result should be described.

Defra's Chief Scientific Adviser, Professor Howard Dalton, said "The UK,
and especially the VLA, have played an important part in improving the
diagnostic methods available for identifying TSEs in sheep. As we
continue to assess more samples with these improved methods it is likely
that we will continue to find samples, such as this, which fall outside
our current knowledge of the disease. Defra, as it does with all
research, will continue to consult scientific experts to ensure that we
are investigating these cases using the best available techniques and

The National Scrapie Plan remains unaffected by this new result and SEAC
will be consulted in the near future.

Notes to editors

1. Scrapie is a fatal neurological sheep disease belonging to a group of
diseases called transmissible spongiform encephalopathies (TSEs),
including BSE in cattle and CJD in humans. It has been present in the
national flock for over 250 years. It is not considered to be
transmissible to humans.

2. There is a theoretical risk that BSE could be present in sheep,
masked by scrapie, but it has not been found naturally occurring in sheep.

3. There is as yet no definitive diagnostic method that can rapidly
distinguish between different TSEs for example scrapie from BSE.
Consequently, from time to time the scrapie surveillance programmes in
EU member states throw up unusual results that merit further
investigations (Defra press release 371/03 refers

4. The VLA have applied several different methods to the sample to
compare it to a wide range of previously detected scrapie cases,
experimental BSE in sheep and an experimental strain of scrapie, termed
CH1461. Two main methods have been used in this analysis:-

a. Western blot (WB)
This involves taking a sample of the brain and treating it with an
enzyme proteinase k to destroy the normal prion protein (PrPC). The
diseased form of the protein (PrPSc) is able to withstand this treatment
and is then separated from other cellular material on a gel. A blot is
taken of the gel and the PrPSc is visualised using specific antibodies.

b. Immunohistochemistry (IHC)
This involves taking thin slices of the brain, and by using special
(antibody) markers to detect the PrPSc it is possible to see disease
specific patterns of PrPSc distribution in the brain under a microscope.
The Western blot method found that the sample did not appear to resemble
previously recognised cases of scrapie and, although there were some
differences, some characteristics were similar to experimental BSE in
sheep and also the experimental strain of sheep scrapie, CH1461. IHC
found that it neither resembled previously recognised types of scrapie
or experimental BSE in sheep

5. The tissue sample has now been analysed using a total of 5 different
diagnostic methods claiming to be able to differentiate between scrapie
and experimental BSE in sheep. Two were performed at the VLA and three
were performed in other European laboratories.

6. The VLA is the European Reference Laboratory for TSEs and is
responsible for co-ordinating such investigations into unusual cases.
Their findings will be considered by the European Food Safety
Authority's committee of TSE experts and in the UK by the Spongiform
Encephalopathy Advisory Committee (SEAC).

7. The genotype of the suspect sheep was ARQ/ARQ which is known to be
susceptible to some strains of scrapie and, in experiments, to BSE.
Background information on scrapie, scrapie genotyping, and the National
Scrapie Plan is published on the Defra internet at www.defra.gov.uk/nsp.

8. For information and advice on BSE in sheep from the FSA please
consult their web site at www.foodstandards.gov.uk

Public enquiries 08459 335577;
Press notices are available on our website
Defra's aim is sustainable development


Nobel House
17 Smith Square
London SW1P 3JR
Website www.defra.gov.uk



########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############

TSE in Sheep Contingency Planning Assessment of Risk due to BSE
Infectivity from Disposal of Sheep A report for DEFRA November 2001

Management Summary It has been recognised for a considerable time that
sheep in the United Kingdom may have been infected with BSE. To date no
evidence has been found to demonstrate that the national flock is
actually infected with the disease. DEFRA have prepared a draft
contingency plan in the event that BSE were to be identified in UK
sheep. The worst case scenario under this plan is the disposal of the
entire UK flock, some 40 million sheep and lambs. This study has
estimated the potential exposure of the UK population to BSE infectivity
present in sheep in the event that this plan had to be put into effect.


but who would have guessed that such an important experiment/study would
have gotton so screwed up, by not being able to tell a sheep brain from
a cow brain;

© DEFRA 2002 Item 3- Scrapie Brain pool experiments- Update on current
position and audits of samples 3.1 Members were updated on experiments
conducted at the Institute of Animal Health (IAH) to examine a pool of
scrapie brains collected in the early 1990s for evidence of BSE. SEAC
had previously recommended that the material should be examined by DNA
analysis to assess whether the pooled brain material may have been
contaminated with bovine tissue. The Laboratory of the Government
Chemist (LGC) had been asked to perform the work. Their results were
completely unexpected as the analysis detected only bovine material in
the sample. SEAC had intended to meet on the 19 October to Agreed
version consider the experiment in detail. However, in view of the
result, the meeting was cancelled.


Executive Summary An audit of the sample handling procedures at IAH-E
was carried out on 24 October 2001 at the request of the Department of
the Environment, Food and Rural Affairs (DEFRA), by a team of two UKAS
auditors. The scope of the audit was limited to the traceability of cow
and sheep brain samples used in several experiments relating to
transmissible spongiform encephalopathy (TSE) agents. In particular, the
team focused on the audit trail of samples that had been sent to LGC,
Teddington, the audit trail of brains collected in 1990/92 by Veterinary
Investigation Centres and the audit trail for archived material held by
IAH-E. In addition the audit team evaluated the IAH-E procedures against
the specific requirements for sampling handling of international
standard, ISO 17025 and identified opportunities for improvement. The
audit established that there was no formal documented quality system
covering this work at IAH-E and that record keeping was inadequate to
give confidence in the chain of custody of samples used in the various
rendering, genotyping and strain typing experiments audited. It was not
possible to establish clear traceability between the samples that had
been used in the individual experiments carried out by IAH-E or IAH-C
with those analysed at LGC or with those that had been collected in
1990/92. The sample handling procedures covered by this audit at IAH-E
did not meet the requirements of ISO 17025.


explaining the brain mixup blunder;

An Investigation of the Substitution of Scrapie Brain Pool Samples A
report for DEFRA November 2001

Risk Solutions Page 19 Why did the experimenters not notice that they
were working with cow brains not sheep brains? The simple answer is
because for the most part they were working with brain pool macerate
(minced brain material) not brains. It is not credible that staff
collecting brains at VICs would have uniformly supplied cow brains or
cow brain parts in mistake for sheep. We have interviewed staff at VICs
and we understand from the VLA that records do not support the
possibility that significant numbers of cow brains were sent to PDM in
place of sheep brains. It is also very unlikely that the people
preparing the scrapie brain pool would not have noticed if they were for
the most part handling cow brains or cow brain parts in place of sheep
brains. We cannot rule out the possibility that some cow brain material
entered the brain pool at this stage but it is not feasible that the
majority of the material was bovine. The substitution, if substitution
occurred, must have involved brain pool macerate or rendered
products. Why cant the results of the experiments tell us what
material was used? The experiments had a number of features that make
the results of the mouse bioassay difficult to interpret unambiguously
and lead to the possibility that substitution of the samples would be
difficult to detect by examining the results of the experiments: 1. The
original experiments were not designed to determine whether BSE was
present in sheep. Reasonable efforts were taken to ensure that the brain
pool remained free from D5055 02 Issue 1 Risk Solutions Page 20
contamination during preparation but the level of control applied during
the earlier experiments (272R and 372R) was not to the standard applied
later. 2. Mouse bioassay as a method of diagnosing TSEs is not based on
a full understanding of biochemical and physical processes. It is an
empirical technique that has been widely applied, for example to show
v-CJD is similar to BSE and different from scrapie. It is a complex
process and the results need to be interpreted by experts. It can take
several years to generate a firm result. The principal data collected in
the experiments are lesion profiles (patterns of lesions in the mice
brains) and incubation period (time from injection of mice to onset of
clinical symptoms. The type of TSE is identified by comparing the
results with those of known provenance. There is no good agreed test
of sameness of lesion profile, so in marginal cases we are reduced to
using subjective observations of the form somewhat similar and
interpretation is difficult. The incubation times in principle give a
more objective signal, but the effect of concentration has to be
controlled. The mouse bioassay data that we understand has been
collected and analysed at each stage of the experiments is summarised in
Table 4.1. Several features of these experiments are not commonly
encountered in mouse bioassay of TSEs and this makes determining the
origin of the original material from the experimental results extremely
difficult. They include: a. Mouse bioassay is generally carried out on
individual brains; experience of working with brain pools is very
limited. b. The BBP exhibited a low titre of infectivity, which can
confound interpretation of results. c. The BBP comprised bovine brains
with the hindbrains removed. By contrast most of the BSE strain typing
has been carried out on the hindbrains, which may give a different
pattern of results. d. The 272R titrations used a different strain of
mice than the 372R titrations, so direct comparison of the resulting
lesion profiles cannot be made. e. The 246 experiments used brain pool
which was in an unsatisfactorily autolysed state. f. The strain typing
data collected (incubation time and lesion profiles) are very sparse.
Judging the sameness or difference of samples is a less challenging task
for strain typing than identifying a strain and it may be possible to
compare data from the 246 experiments with both the 272R and 372R
experiments to determine whether the samples are similar or clearly
different. However, the data are sparse and the result is unlikely to be
clear cut. Much of this work is currently unpublished.



The Institute is concerned, therefore, that the authors of this UKAS
report may have based their findings on an unrepresentative and limited
examination of procedures in place at IAH-E.




Transmission of prion diseases by blood transfusion

Nora Hunter,1 James Foster,1 Angela Chong,1 Sandra McCutcheon,2 David

Parnham,1 Samantha Eaton,1 Calum MacKenzie1 and Fiona Houston2





b) Fibrillar material closely similar to SAF, found in BSE/Scrapie, was
observed in 19 (4.3%) cases, all of which were hounds > 7 years of age.
14/19 of these suspected SAF results correlated with cases in the
unresolveable histopathological catergory...


HOUND SURVEY (about 72 pages)


Also, at paragraph 17, it is noted that BSE had transmitted to the NPU
negative line sheep (please not that as at January 1996, only one of six
challenged sheep was clinically affected after oral challenge, four
others have since died, and one remains alive. Following intracerebral
challenge, three out of six were clinically affected, two confirmed only
on pathology, while one was negative.)

4. Meeting 16, on 26/1/94 - the update on research (16/5) confirmed that
BSE had been transmitted to sheep, and that there was clinical evidence
of transmission to mice from the spleen of the affected sheep.









hell, they knew they were screwing up the sheep brains with cow brains
in 1992;

"The sensitivity of the project may be partially compromised by pooling
of brains, but it is considered that the success of transmission to mice
with BSE will prove advantageous."



Personal $ Confidential -- Addressee only TO ALL MEMBERS OF SEAC



a) Summary of transmission studies. b) Update


The only circumstance in which infection with the natural isolate
produces an higher incidence of disease compared to BSE, is in
intracerebrally (and possibly orally) challenged ''positive'' line
sheep. Notwithstanding the possibility of indigenous natural scrapie in
some of these sheep, there are still sufficient numbers of transmission
cases with PrP genotypes which preclude the natural disease developing
i.e. those typed as VA136/RR154/QR171.

As an extension to this study, it has been possible to recover BSE by
passage in mice from brain and spleen taken from ''negative'' line sheep
infected with BSAE by ic and oral challenge (Foster and others 1996).
The close similarity of incubation periods and pathology from the
passage of these tissues in mice to those seen in direct BSE
transmission studies from cattle to mice suggests that passaging BSE in
sheep does not alter its bilogical properties (Bruce and others 1994).
IN FACT, because it has been possible to isolate BSE infectivity from
ovine spleens, when this proved impossible from the spleens of naturally
infected BSE cows (Fraser and Foster 1993), experimentally-induced BSE
in sheep appears to behave more like the natural disease of
scrapie.Whether this putative similarity to natural scrapie extends to
the possibility of maternal transmission of experimentally-induced BSE
in sheep, has till to be elucidated...


we have found a link between BSE and CH1641, a C-group of scrapie.
Disease susceptibility of sheep to these isolates is associated with
different PrP genotypes compared to SSBP/1 scrapie...

Transmission of BSE in sheep, goats and mice.


BSE has been transmitted in two lines of genetically selected sheep
(differeing in their susceptibilities to the SSBP/1 source of scrapie),
and to goats by intracerebral injection AND BY ORAL DOSING.


Also, intermediate passage of BSE in sheep or goats did not alter these
primary transmission properties. Hamsters were susceptible to BSE only
after intervening passage through mice...



Perceptions of unconventional slow virus in the USA

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 fantical incident to be avoided in the USA AT ALL COSTS. BSE was
not reported in the USA...........(some good data on CWD)

> avoided in the USA AT ALL COSTS

and indeed they have and it continues today...TSS




Furthermore, we showed that the strain responsible for iCJD is closely
related to that of one patient with sCJD, and, more unexpectedly, that
these agents were similar to the French scrapie strain studied (but
different from the U.S. scrapie strain). This finding requires a
cautious interpretation for several reasons, not least because of the
inevitably limited number of TSE strains that can be studied by such a
cumbersome method as strain typing. Nonetheless, it also prompts
reconsideration of the possibility that, in some instances, sheep and
human TSEs can share a common origin.







IMPORTS FROM CANADA [takes a few minutes to load]


Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]


Docket Management Docket: 02N-0273 - Substances Prohibited From Use in
Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed
Comment Number: EC -10
Accepted - Volume 2




File Format: PDF/Adobe Acrobat -
Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary
Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ...


Asante/Collinge et al, 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;


Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice
in Manufacturing, Packing, or Holding Dietary Ingredients a
Comment Number: EC -2
Accepted - Volume 7


[PDF] Appendices to PL107-9 Inter-agency Working Group Final Report 1-1
File Format: PDF/Adobe Acrobat - View as HTML
Agent, Weapons of Mass Destruction Operations Unit Federal Bureau of
those who provided comments in response to Docket No. ...
Meager 8/18/01 Terry S. Singeltary Sr ...


# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of
TSS 1/27/03 (0)


Dockets Entered On October 2, 2003 Table of Contents, Docket #,
Title, 1978N-0301,
OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr.Vol #: 1, ...


Daily Dockets Entered on 02/05/03
DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2.... Vol#: 1.
03N-0009 Federal Preemption of State & Local Medical Device Requireme. ...


Docket Management
Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater
Comment Number: EC -1
Accepted - Volume 1