***P.170: Potential detection of oral transmission of H type atypical BSE
in cattle using in vitro conversion
Sandor Dudas, John G Gray, Renee Clark, and Stefanie Czub Canadian Food
Inspection Agency; Lethbridge, AB Canada
Keywords: Atypical BSE, oral transmission, RT-QuIC
The detection of bovine spongiform encephalopathy (BSE) has had a
significant negative impact on the cattle industry worldwide. In response,
governments took actions to prevent transmission and additional threats to
animal health and food safety. While these measures seem to be effective for
controlling classical BSE, the more recently discovered atypical BSE has
presented a new challenge. To generate data for risk assessment and control
measures, we have challenged cattle orally with atypical BSE to determine
transmissibility and mis-folded prion (PrPSc) tissue distribution. Upon
presentation of clinical symptoms, animals were euthanized and tested for
characteristic histopathological changes as well as PrPSc deposition.
The H-type challenged animal displayed vacuolation exclusively in rostral
brain areas but the L-type challenged animal showed no evidence thereof. To our
surprise, neither of the animals euthanized, which were displaying clinical
signs indicative of BSE, showed conclusive mis-folded prion accumulation in the
brain or gut using standard molecular or immunohistochemical assays. To confirm
presence or absence of prion infectivity, we employed an optimized real-time
quaking induced conversion (RT-QuIC) assay developed at the Rocky Mountain
Laboratory, Hamilton, USA.
Detection of PrPSc was unsuccessful for brain samples tests from the orally
inoculated L type animal using the RT-QuIC. It is possible that these negative
results were related to the tissue sampling locations or that type specific
optimization is needed to detect PrPSc in this animal. We were however able to
consistently detect the presence of mis-folded prions in the brain of the H-type
inoculated animal. Considering the negative and inconclusive results with other
PrPSc detection methods, positive results using the optimized RT-QuIC suggests
the method is extremely sensitive for H-type BSE detection. This may be evidence
of the first successful oral transmission of H type atypical BSE in cattle and
additional investigation of samples from these animals are ongoing.
P.126: Successful transmission of chronic wasting disease (CWD) into mice
over-expressing bovine prion protein (TgSB3985)
Larisa Cervenakova,1 Christina J Sigurdson,2 Pedro Piccardo,3 Oksana
Yakovleva,1 Irina Vasilyeva,1 Jorge de Castro,1 Paula Saá,1 and Anton Cervenak1
1American Red Cross, Holland Laboratory; Rockville, MD USA; 2University of
California; San Diego, CA USA; 3Lab TSE/OBRR /CBER/FDA; Rockville, MD USA
Keywords: chronic wasting disease, transmission, transgenic mouse, bovine
prion protein
Background. CWD is a disease affecting wild and farmraised cervids in North
America. Epidemiological studies provide no evidence of CWD transmission to
humans. Multiple attempts have failed to infect transgenic mice expressing human
PRNP gene with CWD. The extremely low efficiency of PrPCWD to convert normal
human PrPC in vitro provides additional evidence that transmission of CWD to
humans cannot be easily achieved. However, a concern about the risk of CWD
transmission to humans still exists. This study aimed to establish and
characterize an experimental model of CWD in TgSB3985 mice with the following
attempt of transmission to TgHu mice.
Materials and Methods. TgSB3985 mice and wild-type FVB/ NCrl mice were
intracranially injected with 1% brain homogenate from a CWD-infected Tga20 mouse
(CWD/Tga20). TgSB3985 and TgRM (over-expressing human PrP) were similarly
injected with 5% brain homogenates from CWD-infected white-tailed deer (CWD/WTD)
or elk (CWD/Elk). Animals were observed for clinical signs of neurological
disease and were euthanized when moribund. Brains and spleens were removed from
all mice for PrPCWD detection by Western blotting (WB). A histological analysis
of brains from selected animals was performed: brains were scored for the
severity of spongiform change, astrogliosis, and PrPCWD deposition in ten brain
regions.
Results. Clinical presentation was consistent with TSE. More than 90% of
TgSB3985 and wild-type mice infected with CWD/Tga20, tested positive for PrPres
in the brain but only mice in the latter group carried PrPCWD in their spleens.
We found evidence for co-existence or divergence of two CWD/ Tga20 strains based
on biochemical and histological profiles. In TgSB3985 mice infected with CWD-elk
or CWD-WTD, no animals tested positive for PrPCWD in the brain or in the spleen
by WB. However, on neuropathological examination we found presence of amyloid
plaques that stained positive for PrPCWD in three CWD/WTD- and two
CWD/Elk-infected TgSB3985 mice. The neuropathologic profiles in CWD/WTD- and
CWD/Elkinfected mice were similar but unique as compared to profiles of BSE,
BSE-H or CWD/Tg20 agents propagated in TgSB3985 mice. None of CWD-infected TgRM
mice tested positive for PrPCWD by WB or by immunohistochemical detection.
Conclusions. To our knowledge, this is the first established experimental
model of CWD in TgSB3985. We found evidence for co-existence or divergence of
two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice.
Finally, we observed phenotypic differences between cervid-derived CWD and
CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway
to characterize these strains.
P.169: PrPSc distribution in brain areas of a natural German H-type BSE
case
Anne Balkema-Buschmann, Grit Priemer, Markus Keller, and Martin H Groschup
Friedrich Loeffler Institut, Institute for Novel and Emerging Infectious
Diseases; Greifswald, Insel Riems, Germany
Keywords: BSE H-type, brain, muscle
Ten years after the initial description of atypical BSE cases of the H-type
and L-type, the distribution of PrPSc in different brain areas and peripheral
tissues of natural cases of these BSE forms is still not fully understood.
Intracerebral challenge experiments have been performed with both atypical BSE
forms in cattle, and the distribution of the abnormal prion protein and
infectivity has been analysed in a variety of tissues, confirming the general
restriction to the central nervous system as it was already generally
acknowledged for classical BSE, but showing a slightly earlier and stronger
involvement of the peripheral nervous system and the skeletal muscle.
www.landesbioscience.com Prion 105
However, data from cattle orally challenged with atypical BSE, which might
mimic the natural situation, are not yet available. Unfortunately, for most
natural cases of atypical BSE, only the obex region is available for further
analysis. The PrPSc distribution in the brains of natural L-type BSE cases in
Italy has been described in some detail, but comparably few such data are yet
available for natural H-type cases. Here we describe the analysis of different
brain areas and muscle samples of a natural H-type BSE case diagnosed in Germany
in 2014, and compare these data with those obtained from the respective samples
collected from cattle challenged intracerebrally with H-type BSE.
P.159: Transgenic mice overexpressing rabbit prion protein are susceptible
to BSE, BASE and scrapie prion strains but resistant to CWD and atypical
scrapie
Natalia Fernández-Borges,1 Enric Vidal,2 Belén Pintado,4 Hasier Eraña,1
Montserrat Ordóñez,3 Mercedes Márquez,5 Francesca Chianini,6 Dolors Fondevila,5
Manuel A Sánchez-MartÃn,7 Olivier Andréoletti,8 Mark P Dagleish,6 MartÃ
Pumarola,5 and JoaquÃn Castilla1,3 1CIC bioGUNE; Parque tecnológico de Bizkaia;
Derio; Bizkaia, Spain; 2Centre de Recerca en Sanitat Animal (CReSA); UAB-IR TA,
Campus de la Universitat Autònoma de Barcelona; Bellaterra; Barcelona,
Catalonia, Spain; 3IKERBASQUE; Basque Foundation for Science; Bilbao, Bizkaia,
Spain; 4Centro Nacional de BiotecnologÃa (CNB), Campus de Cantoblanco;
Cantoblanco; Madrid, Spain; 5Department of Animal Medicine and Surgery;
Veterinary faculty; Universitat Autònoma de Barcelona (UAB); Bellaterra
(Cerdanyola del Vallès); Barcelona, Catalonia, Spain; 6Moredun Research
Institute; Bush Loan, Penicuik, Scotland, UK; 7Unidad de Generación de OMGs.
S.E.A. Department of Medicine; University of Salamanca; Salamanca, Spain; 8Ecole
Nationale du Veterinaire; Service de Pathologie du Bétail; Toulouse,
France
Interspecies transmission of prions is a well established phenomenon, both
experimentally and in field conditions. Upon passage through new hosts prion
strains have proven their capacity to change their properties. It is, in fact, a
source of strain diversity which needs to be considered when assessing the
potential risks associated with consumption of prion contaminated protein
sources.
Rabbits were considered for decades a prion resistant species until proven
recently otherwise. To determine the extent of rabbit susceptibility to prions
and to assess their effects on the passage of different prion strains through
this species, a transgenic mouse model overexpressing rabbit PrPC was developed
(TgRab). Intracerebral challenges with prion strains originating from a variety
of species including field isolates (SSBP1 scrapie, Nor98-like scrapie, BSE,
BASE and CWD), experimental murine strains (ME7 and RML), experimentally
obtained strains (sheepBSE) and strains obtained by in vitro crossing of the
species barrier using saPMCA (BSE-RabPrPres, SSBP1-RabPrPres and CWD-RabPrPres)
have been performed.
Interestingly, on first passage, TgRab were susceptible to the majority of
prions tested with the exception of SSBP1 scrapie, CWD and Nor98 scrapie.
Furthermore TgRab were capable of propagating strain-specific features such as
differences in incubation periods, brain lesion and PrPd deposition profiles and
PK resistant western blotting band patterns. Our results confirm previous
studies shattering the myth that rabbits are resistant to prion infection and
this should be taken into account when choosing protein sources to feed rabbits.
P.168: Evolution of the biological properties of L-BSE after passage in
sheep with susceptible and resistant PrP genotypes
Michele A Di Bari, Umberto Agrimi, Claudia D’Agostino, Geraldina Riccardi,
Stefano Marcon, Elena Esposito, Paolo Frassanito, Flavio Torriani, Shimon
Simson, and Romolo Nonno Istituto Superiore di Sanità (ISS) Department of
Veterinary Public Health and Food Safety; Rome, Italy
Background. Cattle L-BSE was efficiently transmitted to sheep with
susceptible (QQ171) and resistant (QR171) PrP genotypes. 1 Notably, the PrPSc
signature of L-BSE was preserved in QQ171 sheep but not in QR171 sheep.2
Notwithstanding, bioassay in transgenic mice expressing bovine or ovine (ARQ)
PrPC showed that L-BSE strain was preserved in both, QQ171 and QR171
sheep-passaged L-BSE.3
Here we studied the biological properties of sheep-passaged L-BSE by
bioassay in bank voles and transgenic mice expressing the ovine VRQ PrP (tg338),
both characterized by a comparatively low susceptibility to cattle L-BSE.
Material and Methods. Voles and tg338 mice were intracerebrally inoculated
with cattle L-BSE and sheep-passaged (QQ171 and QR171) L-BSE isolates. Survival
time, lesion profiles, Pet-blot and WB analysis were used for strain typing.
Results. Cattle L-BSE transmitted quite inefficiently to tg338 mice, with
survival time >400 days post-infection (d.p.i.), while sheep-passaged inocula
were much more efficient and all gave terminal disease by ~140 d.p.i. However,
after sub-passage all inocula converged to a survival time of ~145 d.p.i.. and
showed overlapping pathological phenotypes.
In voles, cattle L-BSE transmitted with very long survival times (~800
d.p.i.) and was accompanied by an upward shift of the PrPSc type. Again, all
sheep-passaged L-BSE isolates transmitted much more efficiently, with similar
survival times of ~360 d.p.i.. Upon second passage, three different strains were
isolated in vole, characterized by distinct pathological phenotypes. This
divergence is epitomized by the different survival times of vole-adapted L-BSE
strains, which were ~400 d.p.i. for cattle L-BSE, ~130 d.p.i. for QQ171-passaged
L-BSE and ~225 d.p.i. for QR171-passaged L-BSE.
Conclusions. These findings, along with previously published data,3 show
that the original L-BSE strain was recovered after passage in sheep when
bioassay was performed in animal models expressing bovine or ovine PrPC. In
contrast, strain changes were observed in both, QQ171- and QR171-passaged L-BSE
by bioassay in vole, a species with divergent PrP sequence compared to
ruminants. Importantly, QQ171- and QR171-passaged L-BSE were characterised by
different PrPSc types and, accordingly, showed different biological properties
when transmitted to voles, but not when transmitted to other animal models.
Overall, our work support the hypothesis that prion isolates are likely
composed of multiple prion components, emphasizes the role of host PrP
polymorphisms on strain selection and mutation, and highlights the risk for new
potentially zoonotic strains that could emerge from prion evolution in animal
reservoirs.
P.172: BSE exposure risk from bovine intestine and mesentery
Fulvio Barizzone,1 Herbert Budka,2 Christine Fast,3 John N Griffin,4
Giuseppe Ru,5 Pietro Stella1 and Olivier Andréoletti6 1European Food Safety
Authority; Parma, Italy; 2Institute of Neuropathology; University Hospital
Zurich; Zurich, Switzerland; 3Friedrich-Loeffler-Institut; Institute of Novel
and Emerging Infectious Diseases; Isle of Riems, Germany; 4Department of
Agriculture, Food and the Marine; Backweston, Celbridge, Co. Kildare, Ireland;
5Istituto Zooprofilattico Sperimentale del Piemonte; Liguria e Valle d’Aosta;
Biostatistics Epidemiology and Analysis of Risk (BEAR) unit; Turin, Italy; 6UMR
Interactions Hôtes Agents Pathogènes; Ecole Nationale Vétérinaire INR A; ENVT;
Toulouse, France
Keywords: Bovine Spongiform Encephalopathy (BSE), cattle, intestine,
mesentery, specified risk material (SRM), quantitative risk assessment
(QRA)
Bovine intestines and mesenteries in the European Union (EU) are considered
among the tissues potentially containing the highest level of BSE infectivity
and have to be removed from the food and feed chain. A quantitative assessment
of the BSE infectious load potentially entering the food and feed chain yearly
in the European Union (EU) was developed. The evolution of the BSE infectious
titre and of the weight of the structures accumulating infectivity was
considered. The number of BSE infected cattle entering undetected in the food
and feed chain yearly was estimated. A model (TSEi) was developed to estimates
the evolution of the BSE infectious load in animals and the total yearly
infectious load that could enter the food and feed chain. In a BSE infected
bovine, the distribution of infectivity in intestines and mesentery varies with
the age. Up to 36 months of age the infectivity is mainly associated (on average
more than 90%) with the last 4 metres of small intestine and the caecum, over 36
and under 60 months of age, there is an inter-individual variability, from 60
months of age the infectivity is mainly associated (on average more than 90%)
with the mesenteric nerves and the celiac and mesenteric ganglion complex. The
total amount of infectivity peaks, about 15 BoID50, in animals younger than 18
months, it declines to 8-9 BoID50 (24–48 months of age) and it drops to 0.7
BoID50 in animals older than 60 months. The ileocaecal plate is the most
infectious part of the intestine and it can be used to estimate the potential
maximum level of exposure for an individual consumer.
In the EU, between 2007 and 2012, the yearly amount of BSE infectivity
associated with intestine and mesentery from animals entering the food and feed
chain was reduced by a factor of 10 (from about 23,000 to about 2,000 BoID50).
However, the maximum level of exposure to the BSE agent from intestine
remained stable (on average about 1.5-1.6 BoID50 per meter).
In case of re-emergence of BSE in the EU there would be an increase of the
potential maximum level of exposure to BSE from intestine. According to the TSEi
model the removal of the last four metres of the small intestine and of the
caecum from the food and feed chain would result in a major reduction of the BSE
exposure risk associated with intestine and mesentery in cattle.
P.131: Transmission of sheep-bovine spongiform encephalopathy in
pigs
Carlos Hedman,1 Belén MarÃn,1 Fabian Corbière,3 Hicham Filali,1 Francisco
Vázquez, José Luis Pitarch,1 William Jirón,1 Rodrigo S Hernandez,1 Bernardino
Moreno,1 Martà Pumarola,2 Olivier Andréoletti,3 Juan José Badiola,1 and Rosa
Bolea1 1University of Zaragoza; Zaragoza, Spain; 2University of Barcelona;
Barcelona, Spain; 3Institut National de la Recherche (INR A); Toulouse,
France
Introduction. The transmissible spongiform encephalopathies (TSE) don´t
occur in swine in natural conditions. However, the bovine spongiform
encephalopathy (BSE) agent, inoculated by 3 simultaneous routes in pigs, is able
to reproduce a neurological disease in these animals. On the other hand, the BSE
agent after passage in sheep under experimental conditions (sheep- BSE) exhibits
altered pathobiologic properties. This new agent is able to cross the cattle-pig
transmission barrier more efficiently than BSE. The potential propagation of TSE
in animals from the human food chain, including pigs, needs to be assessed
regarding the risk for human infection by animals other than TSE-infected
ruminants. The aim of this work was to determine the susceptibility of pigs to
the Sheep-BSE agent and describe the pathological findings and PrPSc deposition
in different tissues.
Material and Methods. Seven minipigs were challenged intracerebrally with
sheep-BSE agent. Clinical observation and postmortem histopathology,
immunohistochemistry (antibody 2G11) and Western blotting were performed on
central nervous system (CNS), peripheral nervous system (PNS) and other
tissues.
Results. One pig was culled in an early incubation stage, and remaining six
were culled at the presence of clinical sings. Pigs developed a clinical disease
with locomotor disorders in an average time of 23 months post inoculation,
showing clinical findings in most of them earlier than those described in the
BSE in pigs experimental infection. TSE wasn´t confirmed in the preclinical pig.
In clinical pigs, the entire cerebral cortex showed severe neuropil vacuolation,
extensive and severe vacuolar changes affecting the thalamus, hippocampus and
cerebellum. PrPSc was found in CNS of all clinical pigs (6/6). Intracellular
(intraneuronal and intraglial) and neuropil-associated PrPSc deposition was
consistently observed in the brainstem, thalamus, and deeper layers of the
cerebral cortex. Also, PrPSc was observed in PNS, mainly in the myenteric plexus
and also in nerves belonging to the skeleton muscle. Moreover, the glycosylation
profile showed a 3 band pattern with a predominant monoglycosylated band in
positive pig samples.
This features concern on the potential risk of utilization of meat and
bound meal of small ruminants in feeding pigs.
P.177: Elements modulating the prion species barrier and its passage
consequences
Juan-Carlos Espinosa,1 Patricia Aguilar-Calvo,1 Ana Villa-Diaz,1 Olivier
Andréoletti,2 and Juan MarÃa Torres1 1Centro de Investigación en Sanidad Animal
(CISA-INI A); Valdeolmos, Madrid, Spain; 2UMR INR A-ENVT 1225; Interactions Hôte
Agent Pathogène; École Nationale Vétérinaire de Toulouse; Toulouse, France
The phenotypic features of Transmissible Spongiform Encephalopathy (TSE)
strains may be modified during passage across a species barrier. In this study
we investigated the biochemical and biological characteristics of Bovine
Spongiform Encephalopathy (BSE) infectious agent after transmission in both
natural host species (cattle, sheep, pigs, and mice) and in transgenic mice
overexpressing the corresponding cellular prion protein (PrPC) in comparison
with other non-BSE related prions from the same species. After these passages,
most characteristics of the BSE agent remained unchanged. BSE-derived agents
only showed slight modifications in the biochemical properties of the
accumulated PrPSc, which were demonstrated to be reversible upon re-inoculation
into transgenic mice expressing bovine-PrPC. Transmission experiments in
transgenic mice expressing bovine, porcine or human-PrP revealed that all
BSE-derived agents were transmitted with no or a weak transmission barrier. In
contrast, a high species barrier was observed for the non-BSE related prions
that harboured an identical PrP amino acid sequence such as sheep-scrapie, mouse
RML or human sCJD isolates, supporting the theory that the prion transmission
barrier is modulated by strain properties (presumably conformation-dependent)
rather than by PrP amino acid sequence differences between host and donor.
As identical results were observed with prions propagated either in natural
hosts or in transgenic mouse models, we postulate that the species barrier and
its passage consequences are uniquely governed by the host PrPC sequence and not
influenced by the PrPC expression level or genetic factors other than the PrPC
amino acid sequence. All these findings unequivocally demonstrate that the
species barrier and its passage consequences are uniquely driven by the PrPC
sequence, and not by other host genetic factors, demonstrating the validity of
transgenic PrP animals as models for studies of the species barrier.
The results presented herein reinforce the idea that the BSE agent is
highly promiscuous, infecting other species, maintaining its properties in the
new species, and even increasing its capabilities to jump to other species
including humans. These data are essential for the development of an accurate
risk assessment for BSE.
P.140: BSE monitoring in the Russian Federation in 2011-2013
Alexander Yegorov, Sergey Rybakov, Asya Borisova, and Andrey Pavlov FGBI
Federal Centre for Animal Health; Vladimir, Russia
Keywords: BSE, monitoring, BSE risk status of the cattle population
Background. The goal of the study was to improve monitoring of bovine
spongiform encephalopathy (BSE) in the Russian Federation (RF) aimed at the
solution of the main tasks:
• confirmation of efficacy of imposed in 1989 restrictions on import of
feeds, live cattle and beef from some countries, where the level of BSE spread
was significant;
• confirmation of efficacy of the introduced in 1990 ban on feeding of
ruminant protein to ruminants, as well as brought into force in 1996-2001
measures focused on the improvement of import control and feed production for
ruminants.
The solution of these tasks is the area of responsibility of the Federal
Service for Veterinary and Phytosanitary Surveillance (FSVPS).
Material and methods. BSE monitoring in 2011-2012 was carried out in 23 RF
regions where more than a thousand of bovine animals from controlled BSE risk
countries had been imported. In 2013 fifty six RF Subjects were involved in the
implementation of the monitoring program; in those Subjects the adult cattle
population amounted to 8.35 million or 94.6% of the whole adult cattle
population in RF. Bio-Rad diagnostic kits and equipment were used for brain
sample testing.
Results. Until 2011 the BSE monitoring was carried out only in several
regions of the European part of RF by virtue of the fact that animals were
imported into those regions from EU countries, USA and Canada as from 2004.
About 8,600 cattle brain samples were tested for BSE in RF before 2011. Cattle
brain samples at the rate of 5,258 and 2,598 were collected within monitoring
program in 2011 and 2012, respectively. In 2013 11,687 cattle brain tissue
samples were tested for BSE. The age of 90% of animals was 3–8 years.
Conclusion. As a result of conducted in 2011-2013 studies the disease agent
was detected in none of 19.5 thousand tested samples. The obtained result shows
that applied measures aimed at BSE control are effective. It is necessary to
examine 3.33% of cattle adult population from all Subjects of RF in proportion
to their quantity in order to prove with 95% confidence the absence of BSE in
RF. The chief problems in the arrangement of such activities are associated with
insufficient number of laboratories certified for carrying out BSE studies as
well as need for collection and delivery of samples from huge territories of
RF.
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.
P.152: QuIC amplification of BSE infected sheep blood identifies prion
aggregates in peripheral blood cells and plasma
Richard Alejo Blanco,1 Chris de Wolf,1 Boon Chin Tan,1 Allister JA Smith,1
Christina Orrù,2 Byron Caughey,2 Jean C Manson,1 and Sandra McCutcheon1
1Neurobiology Division; The Roslin Institute and R(D)SVS; University of
Edinburgh; Edinburgh, UK; 2Laboratory of Persistent Viral Diseases; Rocky
Mountain Laboratories; National Institute for Allergy and Infectious Diseases;
Hamilton, MT USA
To date there have been 4 cases of vCJD infection in humans which have
likely arisen as the result of blood transfusion. However, the direct
measurement of the infectious agent is technically challenging due to the low
amounts of blood borne prions in preclinical and clinical animals. The
development of in vitro amplification procedures, such as the Real-Time Quaking
Induced Conversion assay (RT-QuIC)1,2 and enhanced QuIC (eQuIC)3,4 is enabling
more rapid and sensitive methods for the ante-mortem detection of prions in
blood.
We have used an established in vivo blood transfusion model in sheep, in
which BSE is transmitted by the transfusion of blood between donors and naïve
recipients, and shown that all types of blood components contain sufficient
levels of prion-associated infectivity to cause disease.5 As part of this study,
we are accumulating a unique source of thousands of blood samples (consisting of
whole blood, plasma, buffy coat and red cell concentrates) collected at serial
time points from BSE-infected animals and negative controls from the initiation
of infection through to clinical endpoint. Critically, we know when during the
sampling regime blood was infectious as demonstrated by the transmission of BSE
following transfusion. We aim to understand better the relationship between
infectivity seen in vivo and the in vitro detection of prion aggregates in
blood.
We report here for the first time, the use of QuIC methodologies to
successfully amplify prions from BSE-infected blood components. Using a new mAb,
BC6,6 for immunocapture of PrP prior to amplification, we detected prion protein
aggregation from 1 million peripheral blood mononuclear cells (PBMCs) from
BSE-infected sheep; 6 out of 7 sheep (including 2 donors whose transfusion of
buffy coats lead to disease in recipient sheep) were known to have
pre-clinically transmitted infectivity to transfused recipients. RT-QuIC in
conjunction with phosphtungstic acid precipitation was also able to amplify
prion aggregates from PBMCs in addition to plasma from clinical stage,
BSE-infected sheep. These plasma samples were from BSE-positive donors some of
which 14/18 were known to have given rise to positive transmission of BSE
following transfusion to recipients. We are extending our current analyses to
blinded blood samples from the same animals collected at pre-clinical time
points.
These results suggest that QuIC assays are robust and may provide a
reliable method for the consistent detection of the abnormal prion protein in
ante-mortem blood samples.
Acknowledgments. This work was funded by The UK Department of Health
(007/0162) and Fondation Alliance Biosecure
References
1. Henderson DM, Manca M, Haley NJ, Denkers ND, Nalls AV, Mathiason CK,
Caughey B, Hoover EA. Rapid antemortem detection of CWD prions in deer saliva.
PLoS One 2013; 8:e74377; PMID:24040235; http://dx.doi.org/10.1371/journal.
pone.0074377
2. Elder AM, Henderson DM, Nalls AV, Wilham JM, Caughey BW, Hoover EA,
Kincaid AE, Bartz JC, Mathiason CK. In vitro detection of prionemia in
TSEinfected cervids and hamsters. PLoS One 2013; 8:e80203; PMID:24224043;
">http://
dx.doi.org/10.1371/journal.pone.0080203
3. Orrú CD, Wilham JM, Raymond LD, Kuhn F, Schroeder B, Raeber AJ, Caughey
B. Prion disease blood test using immunoprecipitation and improved
quaking-induced conversion. mBio 2011; 2:e00078-11; PMID: 21558432; http://dx.doi.org/10.1128/
mBio.00078-11
4. Vascellari S, Orrù CD, Hughson AG, King D, Barron R, Wilham JM, Baron
GS, Race B, Pani A, Caughey B. Prion seeding activities of mouse scrapie strains
with divergent PrPSc protease sensitivities and amyloid plaque content using
RT-QuIC and eQuIC. PLoS One 2012; 7:e48969; PMID:23139828; http://dx.doi.org/10.1371/journal.
pone.0048969
5. McCutcheon S, Alejo Blanco AR, Houston EF, de Wolf C, Tan BC, Smith A,
Groschup MH, Hunter N, Hornsey VS, MacGregor IR, et al. All clinically-relevant
blood components transmit prion disease following a single blood transfusion: a
sheep model of vCJD. PLoS One 2011; 6:e23169; PMID:21858015; http://dx.doi. org/10.1371/journal.pone.0023169
6. McCutcheon S, Langeveld JP, Tan BC, Gill AC, de Wolf C, Martin S,
Gonzalez L, Alibhai J, Blanco AR, Campbell L, et al. Prion Protein-Specific
Antibodies that Detect Multiple TSE Agents with High Sensitivity. PLoS One 2014;
9:e91143; PMID:24608105; http://dx.doi.org/10.1371/journal.pone.0091143
O.11: Fatal myelopathy in primates exposed to prion contaminated blood
products: Unmasking abnormal PrP
Emmanuel E Comoy,1 Nina Jaffré,1 Jacqueline Mikol,1 Valérie Durand,1
Christelle Jas-Duval,1,2 Sophie Luccantoni-Freire,1 Evelyne Correia,1 Nathalie
Lescoutra-Etchegaray,3 Nathalie Streichenberger,4 Stéphane Haïk,5 Chryslain
Sumian,3 Paul Brown,1 and Jean-Philippe Deslys1 1CEA, Institute of Emerging
Diseases and Innovative Therapies (iMETI), Division of Prions and Related
Diseases (SEPIA); Fontenay-aux-Roses, France; 2EFS-Nord de France; Lille,
France; 3MacoPharma; Tourcoing, France; 4Hospices Civils de Lyon, Prion Unit,
Neurobiology department; Bron, France; 5Inserm, U 975 - CNR S, UMR 7225 -
Université Pierre et Marie Curie-Paris 6, Centre de Recherche de l’Institut du
Cerveau et de la Moelle épinière (CRI CM); Paris, France
The recent report1 of 16 appendices positive for abnormal PrP among 32,441
in British patients strongly suggests a high prevalence of silent vCJD carriers
in the UK (almost 200- fold greater than the total number of clinical vCJD cases
reported so far), This high prevalence might even be underestimated as suggested
by the recent report of one case of vCJD with minimal PrPres deposition in
lymphoid tissues.2 There is thus a continuing cause for concern about the
management of blood and blood products and surgical instruments that encourages
further evaluation of BSE primary and vCJD secondary risks in relevant
experimental models. We present here unexpected results of experiments
evaluating blood transmission risk in a non-human primate model.
www.landesbioscience.com Prion 17
We previously described a new fatal neurological myelopathic syndrome
without detectable PrPres in monkeys exposed to various vCJD/BSE-infected blood
components. After optimization of immunochemical methods and enhanced epitope
retrieval, we are now able, after proteinase K treatment, to detect abnormal PrP
labeling in the spleen and in the spinal cords of affected animals. The same
signature is visible in vCJD controls, together with classical labeling of
PrPres aggregates, but not in uninfected controls. We will present updated data
on transmission of this new disease to primates and mice and will discuss a
“strain competitionlike” phenomenon between this myelopathic disease and the
classical vCJD/BSE in recipient animals. Notably, 100% of the primates (6/6)
exposed to PrP-depleted blood products (through prion removal filters) remain
asymptomatic, whereas 83% (5/6) of the animals exposed to the corresponding
non-depleted samples developed one of these two fatal neurological diseases
(myelopathy or classical vCJD).
The apparent transmission from human blood samples of a highly atypical
prion disease devoid of PrPres to non human-primates, raise the possibility of
an already existing but undetected equivalent of this disease in humans.
References
1. Gill ON, Spencer Y, Richard-Loendt A, Kelly C, Dabaghian R, Boyes L,
Linehan J, Simmons M, Webb P, Bellerby P, et al. Prevalent abnormal prion
protein in human appendixes after bovine spongiform encephalopathy epizootic:
large scale survey. BMJ 2013; 347:f5675; PMID:24129059; http://dx.doi.org/10.1136/bmj.f5675
2. Mead S, Wadsworth JD, Porter MC, Linehan JM, Pietkiewicz W, Jackson GS,
Brandner S, Collinge J. Variant creutzfeldt-jakob disease with extremely low
lymphoreticular deposition of prion protein. JAMA Neurol 2014; 71:340-3;
PMID:24445428; http://dx.doi.org/10.1001/jamaneurol.2013.5378
O.12: Transmissibility and propagation of PrPSc from sCJDMM1-2 in humanized
transgenic mice
Ignazio Cali,1,2 Wenquan Zou,1,4 Laura Cracco,1 Tetsuyuki Kitamoto,3
Qingzhong Kong,1 and Pierluigi Gambetti1 1National Prion Disease Pathology
Surveillance Center (NPDPSC); Department of Pathology; Case Western Reserve
University; School of Medicine; Cleveland, OH USA; 2Department of Clinical and
Experimental Medicine, Second University of Naples; Naples, Italy; 3Graduate
School of Medicine, Tohoku University; Sendai, Japan; 4National Prion Disease
Pathology Surveillance Center (NPDPSC), Department of Neurology, Case Western
Reserve University; School of Medicine; Cleveland, OH USA
Introduction. In sporadic Creutzfeldt-Jakob disease with methionine (M)
homozygous genotype at codon 129 of the PrP gene (sCJDMM), scrapie prion protein
(PrPSc) types 1 and 2 coexist in ~40% of cases.1 We investigated
transmissibility, disease phenotype prevalence, reproducibility and brain
propagation of PrPSc from sCJDMM1-2 subjects.
Materials and Methods. Brain homogenates (BH): 1) from sCJDMM1-2 harboring
PK-resistant PrPSc (resPrPSc) types 1 and 2 co-existing with ratio 50%/50% in
the same region, 2) in vitro mixed type 1 and type 2 with ratio 35%/65%
individually harvested from separate regions of sCJDMM1-2 and 3) in vitro mixed
PrPSc from sCJDMM1 and sCJDMM2 with ratio 10%/90%, were inoculated
intracerebrally into transgenic mice expressing human PrPC-129M. Controls
sCJDMM1 and sCJDMM2 were also used. Mice were sacrificed after 95, 131, 187 days
post inoculation (dpi). The brain hemisphere used for PrPSc studies
(contralateral to the inoculation site) was dissected into four anatomical
regions: 1) cerebral cortex, 2) central brain, 3) brain stem and 4)
cerebellum.
Results. All mice sacrificed at 95, 131 and 187 dpi showed only resPrPSc
type 1 regardless of the type ratios in the inoculum. At 187 dpi, incubation
time, lesion profiles and resPrPSc features in PrPSc type 1-2 inoculated mice
were indistinguishable from those of mice inoculated with sCJDMM1 (183±22 dpi)
but differed from those of sCJDMM2-challenged mice (609±139 dpi). Mice
sacrificed at 95 dpi had low amounts of resPrPSc type 1 in the central brain,
which correlated with spongiform degeneration (SD) and PrP immunostaining in the
thalamus. In mice sacrificed at 131 dpi, resPrPSc type 1 increased in amount in
the central brain and spread to the cerebral cortex and brain stem but not to
the cerebellum. The lesion profile reflected the distribution of resPrPSc. At
187 dpi, PrPres type 1 and SD affected the whole brain with the cerebral cortex
being the most, and cerebellum the least, affected regions.
Conclusions. Regardless of the ratio of PrPSc types 1 and 2 in the
inoculum, all mice reproduce PrPSc type 1 and the sCJDMM1 phenotype indicating
that PrPSc type 2 is much slower in replicating than type 1 and that type 2
replication rate is not accelerated by the presence of type 1.
(1) In sCJDMM1-2, type 1 apparently maintains the same infectivity,
propagation and phenotype determining characteristics of type 1 of
sCJDMM1.
(2) Timing and topography of PrPSc propagation are highly
reproducible.
Acknowledgments. Supported by P01AG 14359, CDC UR8/ CCU515004, NIH NS062787
and Charles S. Britton Fund.
References
1. Cali I, Castellani R, Alshekhlee A, Cohen Y, Blevins J, Yuan J,
Langeveld JP, Parchi P, Safar JG, Zou WQ, et al. Co-existence of scrapie prion
protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the
phenotype and prion-type characteristics. Brain 2009; 132:2643-58;
PMID:19734292; http://dx.doi.org/10.1093/ brain/awp196
O.24: Prion detection in urine of patients with variant Creutzfeldt-Jakob
disease: An update
Fabio Moda,1,2 Pierluigi Gambetti,3 Marcella Catania,2 Luis Concha
Marambio,1 Emanuela Maderna,2 Silvio Notari,3 Kyung-Won Park,1 Ihsin Pan,2
Stéphane Haïk,4 Jean-Philippe Brandel,4 James W Ironside,5 Richard S Knight,5
Silvia Suardi,2 Fabrizio Tagliavini,2 and Claudio Soto1 1Mitchell Center for
Alzheimer’s Disease and Related Brain Disorders; University of Texas Houston
Medical School; Houston, USA; 2 IR CCS Foundation Carlo Besta Neurological
Institute; Milan, Italy; 3Case Western Reserve University; Cleveland, Ohio, USA;
4Cellule Nationale de référence des maladies de Creutzfeldt-Jakob, groupe
hospitalier Pitié-Salpêtrière; Paris, France; 5National CJD Surveillance Unit;
Western General Hospital; University of Edinburgh, UK
Background. Prions are proteinaceous infectious agents responsible for
transmissible spongiform encephalopathies (TSEs). The unique mechanism of
transmission and the appearance of a new form of Creutzfeldt-Jakob disease
(vCJD), which has been linked to the consumption of prion-contaminated cattle
meat, have raised concerns for public health. Compelling evidences suggest that
vCJD has been transmitted from human-to-human by blood transfusion, suggesting
that prions are circulating in biological fluids of people silently incubating
the disease.
Materials and Methods. In this study we investigated whether PrPSc can be
detected in urine of patients affected by vCJD. For this purpose we used the
Protein Misfolding Cyclic Amplification (PMCA) technology. We analyzed urine
from 14 patients with vCJD, 50 patients with sporadic CJD, 5 patients with
genetic forms of TSEs, patients affected by other degenerative (n=50) or
non-degenerative (n=50) neurological disorders, and 50 healthy individuals. All
tested individuals were homozygous for methionine at PRNP codon 129.
Results. Of all urine samples analyzed, only those affected by vCJD were
found to contain PrPSc that could be amplified to obtain a signal that has the
typical electrophoretic profile of PrPSc associated to vCJD. PrPSc was detected
in vCJD urine with 93% sensitivity and 100% specificity. The PrPSc concentration
in urine estimated by quantitative PMCA was around 1x10-16 g/ml, or 3x10-21
moles/ml, which extrapolates to around 40-100 molecules of PrPSc oligomers per
ml of urine.
Conclusions. This is the first report showing the presence of prions in
human urine. The detection of prions in urine may be used as a non-invasive
diagnostic test for vCJD, and also uncovers possible risks related to the use of
urinary-derived products as well as the collection and disposal of urine from
vCJD patients
O.25: In vitro amplification method to detect PrPTSE in blood of macaques
infected with vCJD
Luisa Gregori,1 Nabanita Nag,1 Kristy McDowell,1 Christina M Carlson,2,3
Jay R Scheider,2 Dennis M Heisey,2 Christopher J Johnson,2 Emmanuel E Comoy,4
Jean-Philippe Deslys,4 and David M Asher1 1US Food and Drug Administration,
Center for Biologics Evaluation and Research; Rockville, USA; 2US Geological
Survey, National Wildlife Health Center; Madison, WI USA; 3Program in Cellular
and Molecular Biology, University of Wisconsin; Madison, USA; 4Division of
Prions and Related Diseases, Institute of Emerging Diseases and Innovative
Therapies (iMETI), CEA; Fontenay-aux-Roses, France
Transmission of variant Creutzfeldt-Jakob disease (vCJD) by blood
transfusion is a concern for blood safety. Five probable vCJD transmissions by
blood components were reported in the UK. However, the true vCJD
transfusion-transmission risk is unknown as vCJD prevalence in the UK might be
as high as 1:2,000 individuals. Rapid antemortem tests to detect individuals
with transmissible spongiform encephalopathies (TSE) would contribute to public
health. Such tests are under development, but relevant vCJD blood reference
materials to validate and characterized those candidate tests are not readily
available. As a surrogate for human vCJD-infected blood, we collected blood from
cynomolgus macaques experimentally infected with macaqueadapted vCJD agent.
Macaque vCJD is a relevant experimental model that recapitulates most salient
features of vCJD in humans including infectivity in the blood. Blood was
collected from macaques throughout the incubation period and clinical illness,
thus covering all stages of the disease. These materials are being characterized
for infectivity and for the presence of PrPTSE. Infectivity titrations using
transgenic mice are underway and preliminary results will be reported.
We used an in vitro technique known as protein misfolding cyclic
amplification (PMCA) to amplify abnormal prion protein (PrPTSE) from highly
diluted vCJD-infected human and macaque brain homogenates, seeking to improve
the rapid detection of PrPTSE in tissues and blood. The results showed that,
using red-backed vole (170 S/S) brain homogenates as the substrate,
amplification of 10-12 dilution of 10% brain vCJD homogenates (human and
macaque) was achieved after four rounds of serial PMCA. We concluded that, based
on our estimates and extrapolations, this level of sensitivity might be
sufficient to detect PrPTSE in vCJD-agent-infected human blood.
More recently, we applied optimized PMCA conditions to plasma samples from
terminally ill macaques. Before conducting PMCA, plasma was processed to remove
proteins that inhibited PMCA. Processed vCJD macaque plasma was serially
amplified as described above. In parallel and as a procedural control, 10-9 to
10-12 dilutions of 10% vCJD-infected macaque brain homogenates in normal human
plasma were amplified. The controls for amplification were PMCA with normal
plasma from human and macaque samples and both were negative. The results
confirm our early estimates and suggest that plasma from clinically ill macaques
contains sufficient PrPTSE to be detected by western 24 Prion Volume 8
Supplement
Protein Structure and Biology
Prion and Prion-like Diseases in Humans
P.179: Sporadic Creutzfeldt-Jakob disease in Canada
Zheng Wang,1 Gerard Jansen,1,2 Stacy Sabourin,1 Rolande D’Amour,1 Tim
Connolly,1 Jennifer Kruse,1 David J Knox,3 Neil R Cashman,4 and Michael B
Coulthart1 1The Canadian Creutzfeldt-Jakob Disease Surveillance System; Public
Health Agency of Canada; Ottawa, ON Canada; 2Department of Pathology; Ottawa
Hospital; Ottawa, ON Canada; 3National Microbiology Laboratory; Public Health
Agency of Canada; Winnipeg, MB Canada; 4Brain Research Centre; University of
British Columbia; Vancouver, BC Canada
Background. Sporadic Creutzfeldt-Jakob Disease (sCJD) is a fatal,
transmissible neurodegenerative disease. Systematic surveillance has repeatedly
shown annual mortality in the range 1 to 2 per million population, has
elucidated key characteristics of sCJD, and led to recognition of a new form of
CJD, variant CJD (vCJD), which is associated with BSE. In 1998, Canada launched
comprehensive national CJD surveillance to assess the characteristics of CJD in
Canada, identify any acquired cases of CJD (such as vCJD, of which 2 imported
cases have been identified in Canada to date), and mitigate public health risks.
This study describes the epidemiology of sCJD in Canada from 1998 to 2012.
Methods. Case ascertainment was based on internationally accepted criteria.
Demographic and medical information were collected by standardized questionnaire
and medical chart review. Poisson regression and descriptive analysis were
employed. Results. A total of 563 sCJD deaths (definite: 462, probable: 101) in
Canadian residents were registered from 1998 to 2012. Average annual sCJD
mortality was 1.2 per million population, increasing gradually from 0.9 in 1999
to 1.7 in 2012 (P = 0.0004). All provinces saw average annual mortalities
ranging from 1.0 to 1.6 (P = 0.25), except three territories where population is
small (~25,000 to ~45,000) and no cases were identified. sCJD occurred at
similar rates in males (1.1) and females (1.2) (P = 0.21). sCJD was rare under
50 years of age with only 11 cases identified (2.7%). Mortality increased after
50 and peaked at 7.4 per million in the 70–74 age group. Median age at death was
69 and median duration of illness was 4 months. Genotype at codon 129 (N = 358)
revealed that the MM subgroup accounted for 223 (62%, median age at death: 69,
duration: 4), the MV subgroup was 82 (23%, median age at death: 68, duration:
9), and the VV subgroup was 53 (23%, median age at death: 66, duration: 5).
Results of molecular typing (Parchi Scheme) for 256 cases are; MM1: 140, MM2:
11, MV1: 28, MV2: 18, VV1: 5, VV2: 25, Mixture: 29.
Conclusion. Characteristics of sCJD in Canada are consistent with those
observed in other countries. The increase in sCJD mortality can be partly
attributed to increased awareness of CJD among Canadian clinicians.
These findings support the conclusion that Canadian CJD surveillance system
is sufficiently sensitive to accurately characterize the epidemiology of sCJD in
Canada, and to detect potential additional cases of acquired CJD such as vCJD or
human chronic wasting disease.
P.200: Clinical expression of BSE in mouse models is unrelated to hallmarks
of prion diseases
Christelle Jas-Duval,1,2 Jacqueline Mikol,1 Sophie Luccantoni-Freire,1
Christine Defer,2 Jean-Jacques Huart,2 Paul Brown,1 Jean-Philippe Deslys,1 and
Emmanuel E Comoy1 1CEA, Institute of Emerging Diseases and Innovative Therapies
(iMETI), Division of Prions and Related Diseases (SEPIA); Fontenay-aux-Roses,
France; 2EFS-Nord de France; Lille, France
Background. In 1997, we demonstrated that intracerebral inoculation of
cattle BSE to C57Bl/6 mice induced neurological disease, even though half of the
recipient animals were devoid of PrPres (Lasmezas et al., 1997). PrPres appeared
with shortening incubation periods after subsequent passages, suggesting that
detectable PrPres should be considered as an indicator of strain virulence
rather than as a specific (and required) marker of prion diseases. Subsequent
blood risk studies showed that cynomolgus macaques exposed to vCJD-contaminated
blood products developed either classical vCJD or a novel neurological disease
without detectable PrPres, In our continuing studies of this phenomenon, we now
report the results of intravenous inoculations of several different wild-type
mouse strains with bovine or primate BSE source material.
Materials and Methods. Brains from one bovine and one cynomolgus monkey
showing clinical signs of BSE were sonicated and ultracentrifuged at 188,000 g
for 1 hour. Resulting pellets and supernatants (equivalent to 2 mg of brain /
mouse) were intravenously injected to PrP+/+ Swiss, PrP+/- and PrP+/+ C57Bl/6N
mice, totalling 12 separate bioassays. PrPres was detected using conventional
ELISA, western blotting and IHC procedures. Pathology was studied on
formalin-fixed brain tissues.
Results. The transmission rate of BSE in those 12 experiments ranged from
0% (PrP+/- C57Bl/6N—cattle BSE—supernatant or pellet: no clinical sign, no
spongiosis and no PrPres) to 100% (Swiss—primate BSE—supernatant or pellet: all
mice exhibited clinical signs, spongiosis and PrPres). Overall, transmission was
more efficient in (1) Swiss than C57Bl/6 mice, (2) PrP+/+ than PrP+/- mice, (3)
primate than cattle BSE and (4) pellets than supernatant preparations. Among the
8 models exhibiting partial transmission ratios, 29 mice showed clinical
neurological signs, of which only one-third (10) had detectable spongiosis and
PrPres. The other animals exhibited only PrPres (7), only spongiosis (5) or
neither (7).
Conclusion. Our results suggest that clinical neurotoxicity, spongiosis,
and accumulation of PrPres are three interconnected but disparate phenomena.
Spongiosis and PrPres are specific but not systematic hallmarks of the onset of
prion diseases, notably upon first passage of non-adapted prion strains in a new
host. These results question the universality of current human diagnostic
criteria and the real prevalence of disease linked to BSE exposure.
P.204: Creutzfeldt-Jakob disease in the aging United States
population
Ryan A Maddox,1 Marissa K Person,1 Arialdi M Minino,2 Janis E Blevins,3
Lawrence B Schonberger,1 and Ermias D Belay1 1National Center for Emerging and
Zoonotic Infectious Diseases; Centers for Disease Control and Prevention (CDC);
Atlanta, GA USA; 2National Center for Health Statistics, CDC; Hyattsville, MD
USA; 3National Prion Disease Pathology Surveillance Center (NPDPSC); Case
Western Reserve University; Cleveland, OH USA
Introduction. Creutzfeldt-Jakob disease (CJD) predominantly occurs among
older individuals. To describe the possible impact of changing demographics in
the US population on the occurrence of CJD, we reviewed data from the US census
and from national prion disease surveillance.
Methods. Prion disease decedents were identified from the US national
multiple cause-of-death data and the National Prion Disease Pathology
Surveillance Center database for 2008-2010. Incidence rates were calculated for
decedents ≥65 years and then applied to US census population estimates for 2030
to obtain projections of the number of CJD deaths in that year, assuming no
advances in treatment or prevention of these diseases.
Results. US census data projects that ≥65-year-olds will increase from
13.1% of the population in 2010 to 20.3% in 2030. The CJD incidence rates for
2008-2010 among decedents in the 65-74, 75-84, and 85+ year age groups were, in
cases per million population, 6.5, 7.2, and 3.1, respectively. Applying these
incidence rates to US census projections, in 2030 there may be 461 CJD decedents
≥65 years of age in the United States, an increase of more than 200 cases
compared to the 2008-2010 average for this age group. Of the 461 cases, 251 are
projected to be aged 65-74 years, 182 to be aged 75-84 years, and 28 to be aged
85 years or older.
Conclusions. Unless effective treatments for CJD are developed, the aging
population in the United States will likely result in an increase in CJD cases
due to its higher incidence among older adults. The increase in cases could
impact infection control policies and health care costs, among other factors.
>>> FSIS and Fruitland American Meat have received no reports of
adverse reactions due to consumption of these products.
ha, ha, ha !
what a hoot.
our fine federal friends think we are all idiots. well, most of the lay
public is oblivious to the science behind the TSE prion aka mad cow type
disease.
SO, every time you here the FSIS, the USDA, the APHIS, or the FDA say that,
‘’NO REPORTED ILLNESS TO DATE’’, they are in fact calling every one of us
idiots.
sadly, most of us go with the flow...and the industry keeps feeding us
SRMs, and the government keeps telling us it’s all sporadic CJD.
and the world is happy $$$
*** In some cases, the incubation period may be as long as 50 years
At a hearing in Parliament last Wednesday, the Science and Technology
Committee was told that vCJD continued to pose a “significant” risk to UK public
health and that more than one in every 2000 people could be silent carriers of
the disease.
*** vCJD can have an incubation period of over 30 years.
Monday, February 03, 2014
CREUTZFELDT-JAKOB DISEASE T.S.E. PRION U.K. UPDATE As at 3rd February 2014
***In addition, non-human primates are specifically susceptible for
atypical BSE as demonstrated by an approximately 50% shortened incubation time
for L-type BSE as compared to C-type. Considering the current scientific
information available, it cannot be assumed that these different BSE types pose
the same human health risks as C-type BSE or that these risks are mitigated by
the same protective measures.
Hence, the data presented here are important for a risk- based SRM
definition.
Competing interests
The authors declare that they have no competing interests.
see much more here ;
Saturday, December 21, 2013
Complementary studies detecting classical bovine spongiform encephalopathy
infectivity in jejunum, ileum and ileocaecal junction in incubating cattle
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE
OAI 2012-2013
OAI (Official Action Indicated) when inspectors find significant
objectionable conditions or practices and believe that regulatory sanctions are
warranted to address the establishment’s lack of compliance with the regulation.
An example of an OAI classification would be findings of manufacturing
procedures insufficient to ensure that ruminant feed is not contaminated with
prohibited material. Inspectors will promptly re-inspect facilities classified
OAI after regulatory sanctions have been applied to determine whether the
corrective actions are adequate to address the objectionable conditions.
ATL-DO 1035703 Newberry Feed & Farm Ctr, Inc. 2431 Vincent St. Newberry
SC 29108-0714 OPR DR, FL, FR, TH HP 9/9/2013 OAI Y
DET-DO 1824979 Hubbard Feeds, Inc. 135 Main, P.O. Box 156 Shipshewana IN
46565-0156 OPR DR, FL, OF DP 8/29/2013 OAI Y
ATL-DO 3001460882 Talley Farms Feed Mill Inc 6309 Talley Rd Stanfield NC
28163-7617 OPR FL, TH NP 7/17/2013 OAI N
NYK-DO 3010260624 Sherry Sammons 612 Stoner Trail Rd Fonda NY 12068-5007
OPR FR, OF NP 7/16/2013 OAI Y
DEN-DO 3008575486 Rocky Ford Pet Foods 21693 Highway 50 East Rocky Ford CO
81067 OPR RE, TH HP 2/27/2013 OAI N
CHI-DO 3007091297 Rancho Cantera 2866 N Sunnyside Rd Kent IL 61044-9605 OPR
FR, OF HP 11/26/2012 OAI Y
*** DEN-DO 1713202 Weld County Bi Products, Inc. 1138 N 11th Ave Greeley CO
80631-9501 OPR RE, TH HP 10/12/2012 OAI N
Ruminant Feed Inspections Firms Inventory (excel format)
PLEASE NOTE, the VAI violations were so numerous, and unorganized in dates
posted, as in numerical order, you will have to sift through them for
yourselves. ...tss
Tuesday, June 11, 2013
*** Weld County Bi-Products dba Fort Morgan Pet Foods 6/1/12 significant
deviations from requirements in FDA regulations that are intended to reduce the
risk of bovine spongiform encephalopathy (BSE) within the United States
Thursday, June 6, 2013
BSE TSE PRION USDA FDA MAD COW FEED COMPLIANCE REPORT and NAI, OAI, and VAI
ratings as at June 5, 2013
Greetings,
since our fine federal friends have decided not to give out any more
reports on the USA breaches of the feed ban and surveillance etc. for the BSE
TSE prion mad cow type disease in the USDA livestock, I thought I might attempt
it. I swear, I just don’t understand the logic of the SSS policy, and that
includes all of it. I assure you, it would be much easier, and probably better
for the FDA and the USDA INC., if they would simply put some kind of report out
for Pete’s sake, instead of me doing it after I get mad, because I am going to
put it all out there. the truth.
PLEASE BE ADVISED, any breach of any of the above classifications OAI, VAI,
RTS, CAN lead to breaches into the feed BSE TSE prion protocols, and CAN lead to
the eventual suspect tainted feed reaching livestock. please, if any USDA
official out there disputes this, please explain then how they could not.
paperwork errors can eventually lead to breaches of the BSE TSE prion mad cow
feed ban reaching livestock, or contamination and exposure there from, as well.
I would sure like to see the full reports of just these ;
4018 CHI-DO 3007091297 Rancho Cantera 2866 N Sunnyside Rd Kent IL
61044-9605 OPR FR, OF HP 11/26/2012 OAI Y
9367 3008575486 Rocky Ford Pet Foods 21693 Highway 50 East Rocky Ford CO
81067 OPR RE, TH HP 2/27/2013 OAI N
9446 DEN-DO 1713202 Weld County Bi Products, Inc. 1138 N 11th Ave Greeley
CO 80631-9501 OPR RE, TH HP 10/12/2012 OAI N
9447 DEN-DO 3002857110 Weld County Bi-Products dba Fort Morgan Pet Foods
13553 County Road 19 Fort Morgan CO 80701-7506 OPR RE HP 12/7/2011 OAI N
see full list of the fda mad cow bse feed follies, toward the bottom, after
a short brief update on the mad cow bse follies, and our good friend Lester
Crawford that was at the FDA.
ALSO, I would kindly like to comment on this FDA BSE/Ruminant Feed
Inspections Firms Inventory (excel format)4 format, for reporting these breaches
of BSE TSE prion protocols, from the extensive mad cow feed ban warning letters
the fda use to put out for each violations. simply put, this excel format sucks,
and the FDA et al intentionally made it this difficult to follow the usda fda
mad cow follies. this is an intentional format to make it as difficult as
possible to follow these breaches of the mad cow TSE prion safety feed
protocols. to have absolutely no chronological or numerical order, and to format
such violations in a way that they are almost impossible to find, says a lot
about just how far the FDA and our fine federal friends will go through to hide
these continued violations of the BSE TSE prion mad cow feed ban, and any
breaches of protocols there from. once again, the wolf guarding the henhouse $$$
NAI = NO ACTION INDICATED
OAI = OFFICIAL ACTION INDICATED
VAI = VOLUNTARY ACTION INDICATED
RTS = REFERRED TO STATE
Inspections conducted by State and FDA investigators are classified to
reflect the compliance status at the time of the inspection, based upon whether
objectionable conditions were documented. Based on the conditions found,
inspection results are recorded in one of three classifications:
OAI (Official Action Indicated) when inspectors find significant
objectionable conditions or practices and believe that regulatory sanctions are
warranted to address the establishment’s lack of compliance with the regulation.
An example of an OAI classification would be findings of manufacturing
procedures insufficient to ensure that ruminant feed is not contaminated with
prohibited material. Inspectors will promptly re-inspect facilities classified
OAI after regulatory sanctions have been applied to determine whether the
corrective actions are adequate to address the objectionable conditions.
VAI (Voluntary Action Indicated) when inspectors find objectionable
conditions or practices that do not meet the threshold of regulatory
significance, but warrant an advisory to inform the establishment that
inspectors found conditions or practices that should be voluntarily corrected.
VAI violations are typically technical violations of the 1997 BSE Feed Rule.
These violations include minor recordkeeping lapses or conditions involving
non-ruminant feeds.
NAI (No Action Indicated) when inspectors find no objectionable conditions
or practices or, if they find objectionable conditions, those conditions are of
a minor nature and do not justify further actions.
when sound science was bought off by junk science, in regards to the BSE
TSE prion mad cow type disease, by the USDA, CFIA, WHO, OIE, et al. $$$
when the infamous, and fraudulently USDA, FSIS, APHIS, FDA, gold card was
taken away that infamous day in December of 2003, all cards were off the table,
it was time to change the science, and change they did. ...tss
snip. ...please see full text ;
Thursday, June 6, 2013
BSE TSE PRION USDA FDA MAD COW FEED COMPLIANCE REPORT and NAI, OAI, and VAI
ratings as at June 5, 2013
IN A NUT SHELL ;
(Adopted by the International Committee of the OIE on 23 May 2006)
11. Information published by the OIE is derived from appropriate
declarations made by the official Veterinary Services of Member Countries. The
OIE is not responsible for inaccurate publication of country disease status
based on inaccurate information or changes in epidemiological status or other
significant events that were not promptly reported to the Central Bureau,
Sunday, December 15, 2013
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE
Saturday, November 10, 2012
Wisconsin Firm Recalls Beef Tongues That May Contain Specified Risk
Materials Nov 9, 2012 WI Firm Recalls Beef Tongues
Saturday, July 23, 2011
CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK
MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE
Sunday, October 18, 2009
Wisconsin Firm Recalls Beef Tongues That Contain Prohibited Materials SRM
WASHINGTON, October 17, 2009
Thursday, October 15, 2009
Nebraska Firm Recalls Beef Tongues That Contain Prohibited Materials SRM
WASHINGTON, Oct 15, 2009
Thursday, June 26, 2008
Texas Firm Recalls Cattle Heads That Contain Prohibited Materials
Tuesday, July 1, 2008
Missouri Firm Recalls Cattle Heads That Contain Prohibited Materials
SRMs
Friday, August 8, 2008
Texas Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs
941,271 pounds with tonsils not completely removed
Saturday, April 5, 2008
SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS
KANSAS
Wednesday, April 30, 2008
Consumption of beef tongue: Human BSE risk associated with exposure to
lymphoid tissue in bovine tongue in consideration of new research findings
Wednesday, April 30, 2008
Consumption of beef tongue: Human BSE risk associated with exposure to
lymphoid tissue in bovine tongue in consideration of new research findings
Friday, October 15, 2010
BSE infectivity in the absence of detectable PrPSc accumulation in the
tongue and nasal mucosa of terminally diseased cattle
SPECIFIED RISK MATERIALS SRMs
2007
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
Saturday, August 4, 2012
Final Feed Investigation Summary - California BSE Case - July 2012
Saturday, August 4, 2012
Update from APHIS Regarding Release of the Final Report on the BSE
Epidemiological Investigation
Wednesday, September 25, 2013
Inspections, Compliance, Enforcement, and Criminal Investigations BSE TSE
PRION 2013
Wednesday, October 30, 2013
SPECIFIED RISK MATERIAL (SRM) CONTROL VERIFICATION TASK FSIS NOTICE 70-13
10/30/13
Owens, Julie
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
Sent: Monday, July 24, 2006 1:09 PM
To: FSIS RegulationsComments
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE) Page 1 of 98
FSIS, USDA, REPLY TO SINGELTARY
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
Friday, April 20, 2012
Ultrastructural findings in pigs experimentally infected with bovine
spongiform encephalopathy agent
Wednesday, July 06, 2011 Swine Are Susceptible to Chronic Wasting Disease
by Intracerebral Inoculation (see tonnage of mad cow feed in commerce USA...tss)
In an experimental study of the transmissibility of BSE to the pig, seven
of 10 pigs, infected at 1-2 weeks of age by multiple-route parenteral
inoculation with a homogenate of bovine brain from natural BSE cases developed
lesions typical of spongiform encephalopathy.
PLEASE NOTE, these old BSE Inquiry links take a while to open with the
wayback machine, so be patient. ...tss
Title: Experimental Intracerebral and Oral Inoculation of Scrapie to Swine:
Preliminary Report
In the United States, feeding of ruminant by-products to ruminants is
prohibited, but feeding of ruminant materials to swine and poultry still occurs.
The potential for swine to have access to scrapie-contaminated feedstuffs
exists, but the potential for swine to serve as a host for
replication/accumulation of the agent of scrapie is unknown. The purpose of this
study was to perform oral and intracerebral inoculation of the U.S. scrapie
agent to determine the potential of swine as a host for the scrapie agent and
their clinical susceptibility.
snip...
IN CONFIDENCE EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY 1. CMO should
be aware that a pig inoculated experimentally (ic, iv, and ip) with BSE brain
suspension has after 15 months developed an illness, now confirmed as a
spongiform encephalopathy. This is the first ever description of such a disease
in a pig, although it seems there ar no previous attempts at experimental
inoculation with animal material. The Southwood group had thought igs would not
be susceptible. Most pigs are slaughtered when a few weeks old but there have
been no reports of relevant neurological illness in breeding sows or other
elderly pigs. ...
see full text ;
IN CONFIDENCE
So it is plausible pigs could be preclinically affected with BSE but since
so few are allowed to reach adulthood this has not been recognised through
clinical disease. ...
snip...
CONFIDENTIAL EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY
snip... see full text ;
Thursday, November 10, 2011
National Meat Association v. Harris Docket No., 10-224 DEADSTOCK DOWNER
PIGS AND PORCINE SPONGIFORM ENCEPHALOPATHY PSE Court Likely to Overturn Calif.
Law on Livestock
PLEASE NOTE, these old BSE Inquiry links take a while to open with the
wayback machine, so be patient. ...tss
Title: Experimental Intracerebral and Oral Inoculation of Scrapie to Swine:
Preliminary Report In the United States, feeding of ruminant by-products to
ruminants is prohibited, but feeding of ruminant materials to swine and poultry
still occurs. The potential for swine to have access to scrapie-contaminated
feedstuffs exists, but the potential for swine to serve as a host for
replication/accumulation of the agent of scrapie is unknown. The purpose of this
study was to perform oral and intracerebral inoculation of the U.S. scrapie
agent to determine the potential of swine as a host for the scrapie agent and
their clinical susceptibility.
snip...
IN CONFIDENCE EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY
1. CMO should be aware that a pig inoculated experimentally (ic, iv, and
ip) with BSE brain suspension has after 15 months developed an illness, now
confirmed as a spongiform encephalopathy. This is the first ever description of
such a disease in a pig, although it seems there ar no previous attempts at
experimental inoculation with animal material. The Southwood group had thought
igs would not be susceptible. Most pigs are slaughtered when a few weeks old but
there have been no reports of relevant neurological illness in breeding sows or
other elderly pigs. ...
see full text ;
IN CONFIDENCE
So it is plausible pigs could be preclinically affected with BSE but since
so few are allowed to reach adulthood this has not been recognised through
clinical disease. ... http://web.archive.org/web/20040904150118/www.bseinquiry.gov.uk/files/yb/1990/08/23002001.pdf
snip...
CONFIDENTIAL EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY While this
clearly is a cause for concern we should not jump to the conclusion that this
means that pigs will necessarily be infected by bone and meat meal fed by the
oral route as is the case with cattle. ...
we cannot rule out the possibility that unrecognised subclinical spongiform
encephalopathy could be present in British pigs though there is no evidence for
this: only with parenteral/implantable pharmaceuticals/devices is the
theoretical risk to humans of sufficient concern to consider any action.
May I, at the outset, reiterate that we should avoid dissemination of
papers relating to this experimental finding to prevent premature release of the
information. ...
3. It is particularly important that this information is not passed outside
the Department, until Ministers have decided how they wish it to be handled. ...
But it would be easier for us if pharmaceuticals/devices are not directly
mentioned at all. ...
Our records show that while some use is made of porcine materials in
medicinal products, the only products which would appear to be in a
hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for
which the source material comes from outside the United Kingdom, namely America
China Sweden France and Germany. The products are manufactured by Ferring and
Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon,
makes use of porcine skin - which is not considered to be a ''high risk''
tissue, but one of its uses is described in the data sheet as ''in dural
replacement''. This product is sourced from the United Kingdom.....
snip...
It was not until . . . August 1990, that the result from the pig persuaded
both SEAC and us to change our view and to take out of pig rations any residual
infectivity that might have arisen from the SBOs.
4.303 The minutes of the meeting record that: It was very difficult to draw
conclusions from one experimental result for what may happen in the field.
However it would be prudent to exclude specified bovine offals from the pig
diet. Although any relationship between BSE and the finding of a spongiform
encephalopathy in cats had yet to be demonstrated, the fact that this had
occurred suggested that a cautious view should be taken of those species which
might be susceptible. The 'specified offals' of bovines should therefore be
excluded from the feed of all species. 17
http://web.archive.org/web/20031026084516/http://www.bseinquiry.gov.uk/files/yb/1990/09/07001001.pdf
snip...
7 OF 10 LITTLE PIGGIES WENT ON TO DEVELOP BSE;
1: J Comp Pathol. 2000 Feb-Apr; 122(2-3): 131-43. Related Articles, Links
Click here to read
The neuropathology of experimental bovine spongiform encephalopathy in the
pig.
Ryder SJ, Hawkins SA, Dawson M, Wells GA. Veterinary Laboratories Agency
Weybridge, Woodham Lane, New Haw, Addlestone, Surrey, KT15 3NB, UK.
In an experimental study of the transmissibility of BSE to the pig, seven
of 10 pigs, infected at 1-2 weeks of age by multiple-route parenteral
inoculation with a homogenate of bovine brain from natural BSE cases developed
lesions typical of spongiform encephalopathy. The lesions consisted principally
of severe neuropil vacuolation affecting most areas of the brain, but mainly the
forebrain. In addition, some vacuolar change was identified in the rostral
colliculi and hypothalamic areas of normal control pigs. PrP accumulations were
detected immunocytochemically in the brains of BSE-infected animals. PrP
accumulation was sparse in many areas and its density was not obviously related
to the degree of vacuolation. The patterns of PrP immunolabelling in control
pigs differed strikingly from those in the infected animals. PMID: 10684682
[PubMed - indexed for MEDLINE]
snip...
In the United States, feeding of ruminant by-products to ruminants is
prohibited, but feeding of ruminant materials to swine and poultry still occurs.
The potential for swine to have access to scrapie-contaminated feedstuffs
exists, but the potential for swine to serve as a host for
replication/accumulation of the agent of scrapie is unknown. The purpose of this
study was to perform oral and intracerebral inoculation of the U.S. scrapie
agent to determine the potential of swine as a host for the scrapie agent and
their clinical susceptibility. see full text and more transmission studies here
;
Thursday, June 12, 2014
Missouri Firm Recalls Ribeye and Carcass Products That May Contain
Specified Risk Materials 4,012 pounds of fresh beef products because the dorsal
root ganglia may not have been completely removed
> sCJDMM1-2 should be considered as a separate entity at this time.
> All of the Heidenhain variants were of the methionine/ methionine type
1 molecular subtype.
WHAT ABOUT those old studies at Mission, Texas, where USA scrapie was
transmitted to USA cattle, but the results was not c-BSE. IT was a different
TSE.
WHAT ABOUT atypical Nor-98 Scrapie in the USA, and TSE there from to other
species ???
The key word here is diverse. What does diverse mean?
If USA scrapie transmitted to USA bovine does not produce pathology as the
UK c-BSE, then why would CJD from there look like UK vCJD?"
see ;
Sunday, September 25, 2011 Clinical Heidenhain Variant Of Sporadic
Creutzfeldt-Jakob Disease (CJD) With Co-occurrence Of Prion Protein Types 1 and
2
LET'S take a closer look at this new prionpathy or prionopathy, and then
let's look at the g-h-BSEalabama mad cow.
This new prionopathy in humans? the genetic makeup is IDENTICAL to the
g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like
this, ......wait, it get's better. this new prionpathy is killing young and old
humans, with LONG DURATION from onset of symptoms to death, and the symptoms are
very similar to nvCJD victims, OH, and the plaques are very similar in some
cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets
even better, the new human prionpathy that they claim is a genetic TSE, has no
relation to any gene mutation in that family. daaa, ya think it could be related
to that mad cow with the same genetic make-up ??? there were literally tons and
tons of banned mad cow protein in Alabama in commerce, and none of it
transmitted to cows, and the cows to humans there from ??? r i g h t $$$ ALABAMA
MAD COW g-h-BSEalabama In this study, we identified a novel mutation in the
bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow
from Alabama, United States of America. This mutation is identical to the E200K
pathogenic mutation found in humans with a genetic form of CJD. This finding
represents the first report of a confirmed case of BSE with a potential
pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine
Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old
cow" carrying the E221K mutation.
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and
VPSPr PRIONPATHY
(see mad cow feed in COMMERCE IN ALABAMA...TSS)
***It also suggests a similar cause or source for atypical BSE in these
countries.
P.9.21
Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim
McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre,
Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of
Calgary, Canada
Background: Three BSE types (classical and two atypical) have been
identified on the basis of molecular characteristics of the misfolded protein
associated with the disease. To date, each of these three types have been
detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16
Canadian BSE cases based on the biochemical properties of there associated
PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and
relative proteinase K sensitivity of the PrPres from each of the 16 confirmed
Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type
and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and
changes in glycosylation similar to other atypical BSE cases. PK digestion under
mild and stringent conditions revealed a reduced protease resistance of the
atypical cases compared to the C-type cases. N terminal- specific antibodies
bound to PrPres from H type but not from C or L type. The C-terminal-specific
antibodies resulted in a shift in the glycoform profile and detected a fourth
band in the Canadian H-type BSE.
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.
Saturday, August 4, 2012
*** Final Feed Investigation Summary - California BSE Case - July 2012
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
Saturday, May 26, 2012
Are USDA assurances on mad cow case 'gross oversimplification'?
Singeltary submission to PLOS ;
RE: re-Human Prion Diseases in the United States part 2 flounder replied to
flounder on 02 Jan 2010 at 21:26 GMT I would kindly like to add to my initial
concerns, something I brought up years ago, and I believe that still hold true
today, more so even than when I first stated these concerns in 2003 ;
routine passive mortality CJD surveillance USA ?
THIS has been proven not to be very useful in the U.K.;
THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)
snip...
One reason for this was the _inaccuracy_ in coding of cases correctly
certified as CJD Coding is carried out by staff who are not medically qualified
and it is not surprising that coding errors occur in the processing of large
numbers of certificates. In 1982, 12,000 certificates per week were processed at
the office of population censuses and surveys by 15 coders and 6 checkers
(Alderson et al., 1983). The occurrence of both inter- and intra-observer coding
errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH
certification and coding discovered in this study _support_ the introduction of
a more accurate system of death certificates and a more detailed and specific
coding system...
snip...
Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R.
Will
snip...
IDENTIFICATION OF CASES
Cases of CJD may be identified from death certificates, but this alone is
unlikely to provide adequate monitoring. ERRORS are made in certification and
diagnosis; in the Oxford study death certificates were obtained on a series of
known confirmed cases and CJD was mentioned in only 66% of certificates. In
another series of 175 certified cases, 42 patients were judged not to have
suffered from CJD after examination of case notes (7)...
full text;
http://web.archive.org/web/20050526035006/http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to
other species will invariably present pathology typical of a scrapie-like
disease.
snip...
http://web.archive.org/web/20060307063542/http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
Confucius is confused again? how in 1996 and earlier can the 28 sporadic
CJD victims and the one-in-a-million there from, how can it still be one in a
million in 2008, with the sporadic CJD count rising to 205, still be
one-in-a-million? and the years in-between, steady rise just about every year,
and it still be only one-in-a-million, year after year after years? I suppose
just more of that fuzzy math, which you can see here;
Please see my complete comment to this synopsis here ;
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010
snip...see full text ;
Singeltary submission to PLOS ; RE: re-Human Prion Diseases in the United
States part 2 flounder replied to flounder on 02 Jan 2010 at 21:26 GMT
No competing interests declared.
No competing interests declared.
see full text ;
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?
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. FREE FULL
TEXT
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.
14th ICID International Scientific Exchange Brochure -
Final Abstract Number: ISE.114
Session: International Scientific Exchange
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North
America update October 2009
T. Singeltary
Bacliff, TX, USA
Background:
An update on atypical BSE and other TSE in North America. Please remember,
the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been
documented in North America, along with the typical scrapie's, and atypical
Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these
TSE in different species have been rendered and fed to food producing animals
for humans and animals in North America (TSE in cats and dogs ?), and that the
trading of these TSEs via animals and products via the USA and Canada has been
immense over the years, decades.
Methods:
12 years independent research of available data
Results:
I propose that the current diagnostic criteria for human TSEs only enhances
and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD
only theory in 2009. With all the science to date refuting it, to continue to
validate this old myth, will only spread this TSE agent through a multitude of
potential routes and sources i.e. consumption, medical i.e., surgical, blood,
dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion:
I would like to submit a review of past CJD surveillance in the USA, and
the urgent need to make all human TSE in the USA a reportable disease, in every
state, of every age group, and to make this mandatory immediately without
further delay. The ramifications of not doing so will only allow this agent to
spread further in the medical, dental, surgical arena's. Restricting the
reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO
age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge,
Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al
and many more, that the world of TSE Transmissible Spongiform Encephalopathy is
far from an exact science, but there is enough proven science to date that this
myth should be put to rest once and for all, and that we move forward with a new
classification for human and animal TSE that would properly identify the
infected species, the source species, and then the route.
*** PLOS Singeltary reply ; Molecular, Biochemical and Genetic
Characteristics of BSE in Canada Singeltary reply ;
Creutzfeldt-Jakob Disease Public Health Crisis
Friday, April 25, 2014
Accuracy of administrative diagnostic data for pathologically confirmed
cases of Creutzfeldt-Jakob disease
Article in Press
20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19
year old died from sCJD in France in 1985. There is no evidence of an iatrogenic
cause for those cases....
http://web.archive.org/web/20030330212925/http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf
THE COVER UP OF MAD COW DISEASE IN FARMERS, FARMERS WIVES, AND VICKY
RIMMER, THE DAY MAD COW SCIENCE CHANGED $$$
Monday, May 19, 2008
*** SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND
ABATTOIRS ***
DOES ANYONE BESIDES ME SEE A PATTERN YET ???
Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic
CJD, whatever the hell that is. and there have been 16 year old die from
sporadic CJD in the USA as well.
SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly
are expendable, pets and kids are not.
Science was dictated by 'big buisness' after the Vickey Rimmer case with
the ukbsenvcjd only myth.
and there have been 16 year old die from sporadic CJD in the USA as
well.
snip...
I have interviewed Mrs Rimmer at my constituency surgery
IF there is nothing to hide, why is there so much SECRECY? WHY is the
Government and other Bodies trying to stop any CHANCE OF PEOPLE CONNECTING THE
TWO DISEASES. The B.S.E. problem is obvious, but if the correct measures are
taken, surely the problem could be contained, however, as it stands the lack of
investigation and interest of the possibility of B.S.E. and C.J.D. being linked
is open for speculation and surely someone has to account for peoples lives! WHY
is so much trouble being taken to convice people that B.S.E. and C.J.D. are not
linked? Guilty Conscience perhaps ? - or cover up?
HOUSE OF COMMONS
FROM BARRY JONES, M.P.
22 FEBRUARY 1994
http://web.archive.org/web/20040526010710/http://www.bseinquiry.gov.uk/files/yb/1994/02/22009001.pdf
Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.
(now story changes that biopsy shows she does not have CJD...tss)
http://web.archive.org/web/20030511045541/http://www.bseinquiry.gov.uk/files/yb/1994/06/06004001.pdf
now story changes to ;
Advice
7. The Parliamentary Secretary is invited to note the recent statements
made on __________ and the present position which remains that CJD cannot be
confirmed, in this case at this stage.
http://web.archive.org/web/20030510165315/http://www.bseinquiry.gov.uk/files/yb/1994/06/08004001.pdf
3. The Medical Director at ___________________ Hospital advised the
Department on 6 June that the results of ___________________ brain biopsy had
been received and that it showed NO EVIDENCE OF CJD. ______________ Hospital
subsequently issued a statement to the press to this effect and this was
publicised widely in the press (doc 1). News coverage which followed suggested
that the statement made by ________________ Hospital had been misleading (doc
2). Enquires have been made of the Medical Director at _______________ Hospital
who has CONFIRMED THAT THE STATEMENT ISSUED BY THE HOSPITAL WAS ISSUED IN ERROR.
The facts are that two pathology reports on the same piece of brain tissue were
recieved. The first report indicated that CJD was unlikely, The second report
indicated that CJD was possible, PERHAPS EVEN LIKELY, but that no definitive
diagnosis could be made before a post mortem was undertaken.
http://web.archive.org/web/20030511023028/http://www.bseinquiry.gov.uk/files/yb/1994/06/08006001.pdf
MAD COW MEAL DESTROYED MY DAUGHTERS LIFE
A TEENAGE GIRL may have caught the human form of MAD COW DISEASE by eating
a contaminated burger it was claimed last night.
VICKY RIMMER, 16, has the killer Creutzfeldt-Jakob disease (CJD).
http://web.archive.org/web/20030510205841/http://www.bseinquiry.gov.uk/files/yb/1994/01/25007001.pdf
GIVE ME BACK MY LIFE
THEY BEGGED ME TO HUSH IT UP – GRAN’S AGONY
http://web.archive.org/web/20040521224258/http://www.bseinquiry.gov.uk/files/yb/1994/01/25008001.pdf
HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''
http://web.archive.org/web/20031025182000/http://www.bseinquiry.gov.uk/files/yb/1994/01/25009001.pdf
WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S
BODY
http://web.archive.org/web/20030513071650/http://www.bseinquiry.gov.uk/files/yb/1994/01/25010001.pdf
ONLY PROBLEM IS, VICKY RIMMER, 16, DID NOT DIE FROM nvCJD, SHE DIED FROM
SPORADIC CJD, snip...
full text ;
Wednesday, October 09, 2013
*** WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY,
£41,078,281 in compensation ***
>>> Up until about 6 years ago, the pt worked at Tyson foods where
she worked on the assembly line, slaughtering cattle and preparing them for
packaging. She was exposed to brain and spinal cord matter when she would
euthanize the cattle. <<<
please see full text ;
Monday, March 29, 2010
Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010
in Mesquite Texas
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN
AND SPINAL CORD MATTER
*** WE get them young cases of tse prion disease in Texas, that is not
related to anything $$$ money and politics will buy anything, especially junk
science... sporadic ffi and sporadic gss ;
NOT THIS CASE !!! but another one a while back in Texas...see ;
We report a case of a 33-year-old female who died of a prion disease for
whom the diagnosis of sFI or FFI was not considered clinically. Following death
of this patient, an interview with a close family member indicated the patient's
illness included a major change in her sleep pattern, corroborating the reported
autopsy diagnosis of sFI.
sporadic FFI or nvCJD Texas style ???
Creutzfeldt-Jakob Disease Surveillance in Texas
Sunday, July 11, 2010
CJD or prion disease 2 CASES McLennan County Texas population 230,213 both
cases in their 40s
CJD NE TEXAS CLUSTER
Creutzfeldt-Jakob Disease in Northeast Texas
J.A. Rawlings,*1 K.A. Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2,
Texas Department of Health, 1Austin and 2Tyler, Texas Creutzfeldt-Jacob Disease
(CJD), a transmissible spongiform encephalopathy, is caused by prions composed
of proteinaceous material devoid of nucleic acid. CJD occurs sporadically
(generally 1 case/1,000,000 population per year) in older patients (average age
of 65) and is characterized by rapidly progressive dementia, accompanied by
severe muscle spasms and incoordination. Death usually occurs within 3 to 12
months (average 7 months). CJD activity in Texas, which has a population of
nearly 19 million, appeared to be typical. The statewide death rate for 1995 and
1996 was just under 1/1,000,000. In April of 1997, the Texas Department of
Health became aware of an increased number of possible CJD cases in a 23-county
area of NE Texas with a population of just over one million. After review of
medical and pathology records, four patients were identified with definite
classic CJD and three were identified with probable CJD. Dates of death for the
eight patients were from April, 1996 through mid-July 1997. The patients were
from 46 through 65 years of age; four were male and three were female. A
case-control study to identify risks for CJD in NE Texas has been initiated.
in Texas, they call some sporadic CJD in the very young, sporadic FFi, not
linked to any gene in that family...but sporadic FFi...and other casts of
younger than 55 year olds, just sporadic cjd...right...tss
We report a case of a 33-year-old female who died of a prion disease for
whom the diagnosis of sFI or FFI was not considered clinically. Following death
of this patient, an interview with a close family member indicated the patient's
illness included a major change in her sleep pattern, corroborating the reported
autopsy diagnosis of sFI. Genetic tests identified no prion protein (PrP) gene
mutation, but neuropathological examination and molecular study showed
protease-resistant PrP (PrPres) in several brain regions and severe atrophy of
the anterior-ventral and medial-dorsal thalamic nuclei similar to that described
in FFI. ...
CANADA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic
CJD’s). ...tss
PLEASE NOTE, type determination pending Creutzfeldt Jakob Disease (tdpCJD)
in Canada is also on a steady increase.
please see ;
> 3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is
pending.
CJD Deaths Reported by CJDSS1, 1994-20122
CJD Deaths Reported by CJDSS1, 1994-20122
As of May 31, 2012
SEE DECEMBER 2012 CANADA
USA SEE STEADY INCREASE OF THE SPORADIC CJD’S AND THE VPSPR’S (sporadic
CJD’s). ...tss
National Prion Disease Pathology Surveillance Center
Cases Examined1
(May 18, 2012)
5 Includes 14 cases in which the diagnosis is pending, and 18 inconclusive
cases;
6 Includes 17 (16 from 2012) cases with type determination pending in which
the diagnosis of vCJD has been excluded. The Sporadic cases include 16 cases of
sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive
Prionopathy (VPSPr) and 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).
Rev 5/18/2012
> 6 Includes
> 17 (16 from 2012) cases with type determination pending in which the
diagnosis of vCJD has been excluded.
> The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI)
and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2118 cases
of sporadic Creutzfeldt-Jakob disease (sCJD).
WELL, it seems the USA mad cow strains in humans classified as type
determination pending tdpCJD, VPSPr, sFFI, and sCJD) have steadily increased
over the years, and the same old song and dance continues with sporadic CJD
cases $$$
Creutzfeldt-Jakob Disease CJD cases rising North America updated report
August 2013
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada
seeing an extreme increase of 48% between 2008 and 2010 ***
Sunday, October 13, 2013
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what it ???
Monday, May 19, 2014
Variant CJD: 18 years of research and surveillance
Monday, June 02, 2014
Confirmed Human BSE aka mad cow Variant CJD vCJD or nvCJD Case in Texas
Sunday, April 06, 2014
*** SPORADIC CJD and the potential for zoonotic transmission there from,
either directly or indirectly via friendly fire iatrogenic mode, evidence to
date
Wednesday, December 11, 2013
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease ***
Monday, May 19, 2014
Variant CJD: 18 years of research and surveillance
Saturday, June 14, 2014
Rep. Rosa DeLauro (D-CT) Calls for Briefing on Beef Recalled for Mad Cow
Potential Rep. Rosa DeLauro (D-CT)
Monday, June 9, 2014
TEXAS MAD COW COVER UP (human BSE) AGAIN IN TEXAS, Mr. President Sir, we
need your help please
Governor Rick Perry has done everything he can to cover up mad cow disease
and human TSE prion disease there from in Texas over the last 15 years or so. We
have another nvCJD case here in Texas i.e. human BSE, still no information there
from, another lame excuse, typical though, and more junk science, we need help
Mr. President...
Thursday, June 12, 2014
*** Missouri Firm Recalls Ribeye and Carcass Products That May Contain
Specified Risk Materials 4,012 pounds of fresh beef products because the dorsal
root ganglia may not have been completely removed
Seven main threats for the future linked to prions
***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases
constitute an unforeseen first threat that could sharply modify the European
approach to prion diseases.
Second threat
snip...
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
*** 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...
***In addition, non-human primates are specifically susceptible for
atypical BSE as demonstrated by an approximately 50% shortened incubation time
for L-type BSE as compared to C-type. Considering the current scientific
information available, it cannot be assumed that these different BSE types pose
the same human health risks as C-type BSE or that these risks are mitigated by
the same protective measures.
***Infectivity in skeletal muscle of BASE-infected cattle
***feedstuffs- It also suggests a similar cause or source for atypical BSE
in these countries.
Saturday, August 4, 2012
Final Feed Investigation Summary – California ATYPICAL L-TYPE BASE BSE Case
- July 2012
http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/final-feed-investigation-summary.html
Wednesday, May 30, 2012
PO-028: Oral transmission of L-type bovine spongiform encephalopathy
(L-BSE) in primate model Microcebus murinus
***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans.
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada
seeing an extreme increase of 48% between 2008 and 2010 ***
Sunday, October 13, 2013
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
Tuesday, April 01, 2014
*** Questions linger in U.S. CJD cases 2005, and still do in 2014
Mad cow disease: Could it be here?
Man's stubborn crusade attracts experts' notice By Carol Christian | August
5, 2001
TSS
