News and Reports
TSES
Case of classical BSE confirmed in Wales
THE Welsh Government reported on October 1 that a single case of classical BSE had been detected on a farm in Wales. This was the first case recorded in Wales since 2013.
In a written statement on October 1, Rebecca Evans, the Welsh deputy minister for farming and food, said that the case had been identified as a result of control measures in place, including the routine testing of …
http://veterinaryrecord.bmj.com/content/177/14/353.1.extract?etoc
Written Statement - Isolated case of Classical Bovine spongiform encephalopathy detected in deceased bovine
TSES
Case of classical BSE confirmed in Wales
THE Welsh Government reported on October 1 that a single case of classical BSE had been detected on a farm in Wales. This was the first case recorded in Wales since 2013.
In a written statement on October 1, Rebecca Evans, the Welsh deputy minister for farming and food, said that the case had been identified as a result of control measures in place, including the routine testing of …
http://veterinaryrecord.bmj.com/content/177/14/353.1.extract?etoc
Written Statement - Isolated case of Classical Bovine spongiform encephalopathy detected in deceased bovine
Last updated 01 October 2015
Rebecca Evans, Deputy Minister for Farming and Food The Welsh Government
and the Animal and Plant Health Agency have confirmed a case of classical BSE in
a single deceased bovine on a farm in Wales.
The case was identified as a result of the strict control measures we have
in place. It did not enter the human food chain and the Food Standards Agency
and Public Health Wales have confirmed there is no risk to human health as a
result of this isolated case.
There have been a number of sporadic cases of BSE identified across the UK
in recent years. The last case recorded in Wales was in 2013.
My officials are working closely with DEFRA and the APHA to investigate the
circumstances of this case.
All animals over four years of age that die on a farm are routinely tested
for the disease under our comprehensive surveillance system. Whilst the disease
is not directly transmitted from animal to animal, its cohorts, including
offspring, have been traced and isolated, and will be destroyed in line with EU
requirements.
In addition to the measures we have in place for fallen stock and animal
feed, there is a strict control regime to protect consumers. This includes the
removal of ‘specified risk material’ such as the spinal column, brain and skull
from carcasses.
Identification of this case demonstrates that the controls we have in place
are working well. Beef across the UK continues to be produced in compliance with
the World Organisation for Animal Health rules.
Thursday, June 25, 2015
Ireland Department of Agriculture, Food and the Marine Confirms recent mad
cow case as typical c-type BSE Bovine Spongiform Encephalopathy
Wednesday, September 16, 2015
Ljubljana Slovenia First Mad Cow Disease Case in Eight Years Confirmed
FRANCE HAVE AN EPIDEMIC OF SPONTANEOUS ATYPICAL BSE ‘’LOL’’
spontaneous atypical BSE ???
if that's the case, then France is having one hell of an epidemic of
atypical BSE, probably why they stopped testing for BSE, problem solved $$$
As of December 2011, around 60 atypical BSE cases have currently been
reported in 13 countries, *** with over one third in France.
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
spontaneous TSE prion, that's wishful thinking. on the other hand, if
spontaneous did ever happen (never once documented in the field), it would be
our worst nightmare, due to feed. just saying.
*** We describe the transmission of spongiform encephalopathy in a
non-human primate inoculated 10 years earlier with a strain of sheep c-scrapie.
Because of this extended incubation period in a facility in which other prion
diseases are under study, we are obliged to consider two alternative
possibilities that might explain its occurrence. We first considered the
possibility of a sporadic origin (like CJD in humans). Such an event is
extremely improbable because the inoculated animal was 14 years old when the
clinical signs appeared, i.e. about 40% through the expected natural lifetime of
this species, compared to a peak age incidence of 60–65 years in human sporadic
CJD, or about 80% through their expected lifetimes.
***Moreover, sporadic disease has never been observed in breeding colonies
or primate research laboratories, most notably among hundreds of animals over
several decades of study at the National Institutes of Health25, and in nearly
twenty older animals continuously housed in our own facility.***
>>> Moreover, sporadic disease has never been observed in breeding
colonies or primate research laboratories, most notably among hundreds of
animals over several decades of study at the National Institutes of Health25,
and in nearly twenty older animals continuously housed in our own facility.
<<<
Monday, September 07, 2015
ITALY REPORTING INCREASE IN CASES OF MAD COW TYPE TSE PRION DISEASE IN
HUMANS CJD
Thursday, January 29, 2015
Identification of H-type BSE in Portugal
Thursday, January 29, 2015
Atypical H-TYPE BSE Case Confirmed in Norway
Monday, February 23, 2015
20th BSE Case Raises New Concerns about Canada's Feeding Practices and
Voluntary Testing Program; Highlights Importance of COOL
Thursday, May 28, 2015
OIE cuts six European countries' mad cow risk level, while increasing risk
factors for humans to the BSE TSE PRION DISEASE around the globe
Monday, December 1, 2014
Germany Bovine Spongiform Encephalopathy BSE CJD TSE Prion disease A Review
December 1, 2014
H-type bovine spongiform encephalopathy associated with E211K prion protein
polymorphism: clinical and pathologic features in wild-type and E211K cattle
following intracranial inoculation
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: H-type bovine spongiform encephalopathy associated with E211K prion
protein polymorphism: clinical and pathologic features in wild-type and E211K
cattle following intracranial inoculation
Authors
item Moore, Sarah - item West Greenlee, Mary - item Smith, Jodi item
Nicholson, Eric item Vrentas, Catherine item Greenlee, Justin
Submitted to: Prion
Publication Type: Abstract Only
Publication Acceptance Date: August 12, 2015
Publication Date: May 25, 2015
Citation: Moore, S.J., West Greenlee, M.H., Smith, J., Nicholson, E.,
Vrentas, C., Greenlee, J. 2015. H-type bovine spongiform encephalopathy
associated with E211K prion protein polymorphism: clinical and pathologic
features in wild-type and E211K cattle following intracranial inoculation. Prion
2015. p. S5.
Technical Abstract: In 2006 an H-type bovine spongiform encephalopathy
(BSE) case was reported in an animal with an unusual polymorphism (E211K) in the
prion protein gene. Although the prevalence of this polymorphism is low, cattle
carrying the K211 allele are predisposed to rapid onset of H-type BSE when
exposed. The purpose of this study was to investigate the phenotype of this BSE
strain in wild-type (E211E) and E211K heterozygous cattle. One calf carrying the
wild-type allele and one E211K calf were inoculated intracranially with H-type
BSE brain homogenate from the US 2006 case that also carried one K211 allelle.
In addition, one wild-type calf and one E211K calf were inoculated
intracranially with brain homogenate from a US 2003 classical BSE case. All
animals succumbed to clinical disease. Survival times for E211K H-type BSE
inoculated catttle (10 and 18 months) were shorter than the classical BSE
inoculated cattle (both 26 months). Significant changes in retinal function were
observed in H-type BSE challenged cattle only. Animals challenged with the same
inoculum showed similar severity and neuroanatomical distribution of vacuolation
and disease-associated prion protein deposition in the brain, though differences
in neuropathology were observed between E211K H-type BSE and classical BSE
inoculated animals. Western blot results for brain tissue from challenged
animals were consistent with the inoculum strains. This study demonstrates that
the phenotype of E211K H-type BSE remains stable when transmitted to cattle
without the E211K polymorphism, and exhibits a number of features that differ
from classical BSE in both wild-type and E211K cattle.
*** All animals succumbed to clinical disease. Survival times for E211K
H-type BSE inoculated catttle (10 and 18 months) were shorter than the classical
BSE inoculated cattle (both 26 months). ***
-------- Original Message --------
Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD
Date: Thu, 28 Nov 2002 10:23:43 -0000
From: "Asante, Emmanuel A" e.asante@ic.ac.uk
To: "'flounder@wt.net'" flounder@wt.net
Dear Terry,
I have been asked by Professor Collinge to respond to your request. I am a
Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have
attached a pdf copy of the paper for your attention.
Thank you for your interest in the paper.
In respect of your first question, the simple answer is, ***yes. As you
will find in the paper, we have managed to associate the alternate phenotype to
type 2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim
any further sub-classification in respect of Heidenhain variant CJD or Vicky
Rimmer's version. It will take further studies, which are on-going, to establish
if there are sub-types to our initial finding which we are now reporting. The
main point of the paper is that, as well as leading to the expected new variant
CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an
alternate phenotype which is indistinguishable from type 2 PrPSc.
I hope reading the paper will enlighten you more on the subject. If I can
be of any further assistance please to not hesitate to ask. Best wishes.
Emmanuel Asante
<>
____________________________________
Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial
College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44
(0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@ic.ac.uk (until
9/12/02) New e-mail: e.asante@prion.ucl.ac.uk (active from now)
_________end...TSS___________________
P.150: Zoonotic potential of L-type BSE prions: A new prion disease in
humans?
Emilie Jaumain,1 Stéphane Haïk,2 Isabelle Quadrio,3 Laetitia Herzog,1
Fabienne Reine,1 Armand Perret-Liaudet,3 Human Rezaei,1 Hubert Laude,1 Jean-Luc
Vilotte,4 and Vincent Béringue1 1INR A (Institut National de la Recherche
Agronomique); UR892; Virologie Immunologie Moléculaires; Jouy-en-Josas, France;
2IN SERM; Equipe maladie d’Alzheimer et maladies à Prions; CRicm; UMRS 1127; CNR
S; UPMC. R.; ICM, Hôpital de la Salpêtrière; Paris, France; 3Neurobiologie, CMRR
, Gériatrie, Hospices Civils de Lyon, Université Lyon 1-CNR S UMR5292-IN SERM
U1028; Lyon, France; 3INR A; UMR1313; Génétique Animale et Biologie Intégrative;
Jouy-en-Josas, France
Two novel prion strains, referred to as BSE-L and BSE-H, have been
recognized in bovines through active prion surveillance programs, both being
distinct from the epizootic, ‘classical’, BSE strain (C-BSE). Both H and L-types
have been detected worldwide as rare cases occurring in aged animals. Like C-BSE
prions, H- and L-types prions can propagate with relative ease in foreign
species or in transgenic mouse lines expressing heterologous PrP sequences. A
prion exhibiting biological properties similar to C-BSE agent sometimes emerged
from these cross-species transmissions. Previously, L-type prions were shown to
transmit to transgenic mice expressing human PrP with methionine at codon 129
with higher efficacy than C-BSE prions. Here, we examined whether L-type prions
propagate without any apparent transmission barrier in these mice and whether
such ‘humanised’ L-type prions share biological properties with CJD prions.
L-type prions and a panel of human CJD cases with various genotypes at codon 129
and electrophoretic PrPres signatures were serially transmitted by intracerebral
route to human PrP mice. The biological phenotypes induced by these agents were
compared by all the standard methods currently used to distinguish between prion
strains. At each passage, L-type prions were also transmitted back to bovine PrP
mice to assess whether the agent has evolved upon passaging on the human PrP
sequence. L-type prions transmitted to human PrP mice at 100% attack rate,
without notable alteration in the mean incubation times over 5 passages. At each
passage, ‘humanized’ L-type prions were able to transmit back to bovine PrP
transgenic mice without apparent transmission barrier, as based on the survival
time and the restoration of a L-type BSE phenotype. Comparison of mean
incubation times on primary and subsequent passages in human PrP mice showed no
overlap between L-type and sporadic CJD agents. While the electrophoretic
signature and regional distribution of PrPres in L-type diseased mouse brains
resembled that seen after transmission of MM2 CJD strain type, both agents
exhibited distinct resistance of the associated PrPres molecules to protease
denaturation.
In summary, L-type prions can be passaged on the human PrP sequence without
any obvious transmission barrier. The phenotype obtained differs from the
classical CJD prion types known so far. *** Careful extrapolation would suggest
that the zoonotic transmission of this agent could establish a new prion disease
type in humans.
========Prion2013==========
***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.
================
P.108: Successful oral challenge of adult cattle with classical BSE
Sandor Dudas1,*, Kristina Santiago-Mateo1, Tammy Pickles1, Catherine
Graham2, and Stefanie Czub1 1Canadian Food Inspection Agency; NCAD Lethbridge;
Lethbridge, Alberta, Canada; 2Nova Scotia Department of Agriculture; Pathology
Laboratory; Truro, Nova Scotia, Canada
Classical Bovine spongiform encephalopathy (C-type BSE) is a feed- and
food-borne fatal neurological disease which can be orally transmitted to cattle
and humans. Due to the presence of contaminated milk replacer, it is generally
assumed that cattle become infected early in life as calves and then succumb to
disease as adults. Here we challenged three 14 months old cattle per-orally with
100 grams of C-type BSE brain to investigate age-related susceptibility or
resistance. During incubation, the animals were sampled monthly for blood and
feces and subjected to standardized testing to identify changes related to
neurological disease. At 53 months post exposure, progressive signs of central
nervous system disease were observed in these 3 animals, and they were
euthanized. Two of the C-BSE animals tested strongly positive using standard BSE
rapid tests, 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. Our study demonstrates susceptibility of adult
cattle to oral transmission of classical BSE. We are further examining
explanations for the unusual disease presentation in the third challenged
animal.
***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
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
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
http://bse-atypical.blogspot.com/2014/07/protocol-for-further-laboratory.html
Saturday, September 19, 2015
*** An interview with Professor John Collinge: VIDEO Director of the MRC
Prion Unit Part of the Hayward Gallery's History Is Now ***
http://transmissiblespongiformencephalopathy.blogspot.com/2015/09/an-interview-with-professor-john.html
Thursday, July 30, 2015
Professor Lacey believes sporadic CJD itself originates from a cattle
infection number of cattle farmers falling victim to Creutzfeld-Jakob Disease is
much too high to be mere chance
Saturday, September 12, 2015
The Canadian Management of Bovine Spongiform Encephalopathy in Historical
and Scientific Perspective, 1990-2014
>>>We propose that Canadian policies largely ignored the implicit
medical nature of BSE, treating it as a purely agricultural and veterinary
issue. In this way, policies to protect Canadians were often delayed and
incomplete, in a manner disturbingly reminiscent of Britain’s failed management
of BSE. Despite assurances to the contrary, it is premature to conclude that BSE
(and with it the risk of variant Creutzfeldt-Jakob disease) is a thing of
Canada’s past: BSE remains very much an issue in Canada’s present.
<<<
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle
Over the next 8-10 weeks, approximately 40% of all the adult mink on the
farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or
dead dairy cattle...
In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - APRIL-MAY 1989 - G A H Wells
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. ...
Wednesday, September 23, 2015
NIH Availability for Licensing AGENCY: [FR Doc. 2015–24117 Filed 9–22–15;
8:45 am] Detection and Discrimination of Classical and Atypical L-Type BSE
Strains by RT-QuIC
http://bovineprp.blogspot.com/2015/09/nih-availability-for-licensing-agency.html
Thursday, October 1, 2015
H-type bovine spongiform encephalopathy associated with E211K prion protein
polymorphism: clinical and pathologic features in wild-type and E211K cattle
following intracranial inoculation
Master Obi-Wan Kenobi, Kemosabe...THIS IS NOT GOOD
GOOSE!...grasshopper...tonto...tss
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...TSS
===============
2014
***Moreover, L-BSE has been transmitted more easily to transgenic mice
overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.
***It has been suggested that some sporadic CJD subtypes in humans may
result from an exposure to the L-BSE agent.
*** Lending support to this hypothesis, pathological and biochemical
similarities have been observed between L-BSE and an sCJD subtype (MV genotype
at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and
another sCJD subtype (MM genotype) [15].
snip...
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far
*** but the possibility that a small proportion of human cases so far
classified as "sporadic" CJD are of zoonotic origin could not be excluded.
*** Moreover, transmission experiments to non-human primates suggest that
some TSE agents in addition to Classical BSE prions in cattle (namely L-type
Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME)
and chronic wasting disease (CWD) agents) might have zoonotic potential.
snip...
Thursday, October 02, 2014
[Docket No. APHIS-2013-0064] Concurrence With OIE Risk Designations for
Bovine Spongiform Encephalopathy
Thursday, September 10, 2015
25th Meeting of the Transmissible Spongiform Encephalopathies Advisory
Committee Food and Drug Administration Silver Spring, Maryland June 1, 2015
Terry S. Singeltary Sr.
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