Publications

preclinical detection of infectivity and disease-specific prp in blood throughout the incubation period of prion disease.	variant creutzfeldt-jakob disease (vcjd) is a fatal neurodegenerative disorder characterised by accumulation of pathological isoforms of the prion protein, prp. although cases of clinical vcjd are rare, there is evidence there may be tens of thousands of infectious carriers in the united kingdom alone. this raises concern about the potential for perpetuation of infection via medical procedures, in particular transfusion of contaminated blood products. accurate biochemical detection of prion infe ...	2015	26631638
overview and evaluation of 15 years of creutzfeldt-jakob disease surveillance in belgium, 1998-2012.	in 1998, following the detection of variant creutzfeldt-jakob disease (vcjd) in the uk, belgium installed a surveillance system for creutzfeldt-jakob disease (cjd). the objectives of this system were to identify vcjd cases and detect increases in cjd incidence. diagnostic confirmation of cjd is based on autopsy after referral by neurologists. reference centres perform autopsies and report to the surveillance system. the aim of this study was to assess whether the system met its objectives and to ...	2015	26630984
live kidney donation from a person with haemophilia.	there are many documented cases of a person with haemophilia successfully receiving a solid organ transplant, including liver and kidney. however, there is no literature reporting live organ donation by a person with haemophilia. presumably, this is because the associated risks of excessive bleeding, inhibitor development after a period of intensive treatment with factor replacement and the possibility of variant creutzfeldt-jakob disease transmission in those previously treated with blood produ ...	2015	26628308
the end of the bse saga: do we still need surveillance for human prion diseases?	the epidemics of classical bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd) related to bse-infected food are coming to an end. the decline in concern about these diseases may invite complacency and questions whether surveillance for human prion diseases is still necessary. this article reviews the main points of surveillance and why it is still needed: animal sources for human prion infection other than bse cannot be excluded; the potentially increasing circula ...	2015	26715203
bank vole prion protein as an apparently universal substrate for rt-quic-based detection and discrimination of prion strains.	prions propagate as multiple strains in a wide variety of mammalian species. the detection of all such strains by a single ultrasensitive assay such as real time quaking-induced conversion (rt-quic) would facilitate prion disease diagnosis, surveillance and research. previous studies have shown that bank voles, and transgenic mice expressing bank vole prion protein, are susceptible to most, if not all, types of prions. here we show that bacterially expressed recombinant bank vole prion protein ( ...	2015	26086786
a prion reduction filter does not completely remove endogenous prion infectivity from sheep blood.	variant creutzfeldt-jakob disease (vcjd) is a transmissible spongiform encephalopathy affecting humans, acquired initially through infection with bovine spongiform encephalopathy (bse). a small number of vcjd cases have been acquired through the transfusion of blood from asymptomatic donors who subsequently developed vcjd. filter devices that selectively bind the infectious agent associated with prion disease have been developed for removal of infection from blood. this study independently asses ...	2015	26032915
comparative quantitative study of 'signature' pathological lesions in the hippocampus and adjacent gyri of 12 neurodegenerative disorders.	the hippocampus (hc) and adjacent gyri are implicated in dementia in several neurodegenerative disorders. to compare hc pathology among disorders, densities of 'signature' pathological lesions were measured at a standard location in eight brain regions of 12 disorders. principal components analysis of the data suggested that the disorders could be divided into three groups: (1) alzheimer's disease (ad), down's syndrome (ds), sporadic creutzfeldt-jakob disease, and variant creutzfeldt-jakob disea ...	2015	25929331
foodborne-transmitted prions from the brain of cows with bovine spongiform encephalopathy ascend in afferent neurons to the simian central nervous system and spread to tonsils and spleen at a late stage of the incubation period.	protease-resistant prion protein (prp(res)) accumulation in lymphoreticular tissues indicates prion infection. to date, tonsillectomy and appendectomy samples have been used in population prevalence surveys to detect clinically silent carriers of variant creutzfeldt-jakob disease (vcjd). however, the temporal sequence of prion spread in the human body is still not known. we therefore traced the temporal-spatial pattern of prp(res) accumulation in the body of a simian vcjd model.	2015	25895987
removal of transmissible spongiform encephalopathy prion from large volumes of cell culture media supplemented with fetal bovine serum by using hollow fiber anion-exchange membrane chromatography.	cases of variant creutzfeldt-jakob disease in people who had consumed contaminated meat products from cattle with bovine spongiform encephalopathy emphasize the need for measures aimed at preventing the transmission of the pathogenic prion protein (prpsc) from materials derived from cattle. highly stringent scrutiny is required for fetal bovine serum (fbs), a growth-medium supplement used in the production of parenteral vaccines and therapeutic recombinant proteins and in the ex vivo expansion o ...	2015	25874629
mri detection of prion protein plaques in variant creutzfeldt-jakob disease.		2015	25846996
rare structural genetic variation in human prion diseases.	prion diseases are a diverse group of neurodegenerative conditions, caused by the templated misfolding of prion protein. aside from the strong genetic risk conferred by multiple variants of the prion protein gene (prnp), several other variants have been suggested to confer risk in the most common type, sporadic creutzfeldt-jakob disease (scjd) or in the acquired prion diseases. large and rare copy number variants (cnvs) are known to confer risk in several related disorders including alzheimer's ...	2015	25726360
evaluation of the protection of primates transfused with variant creutzfeldt-jakob disease-infected blood products filtered with prion removal devices: a 5-year update.	analysis of archived appendix samples reveals that one in 2000 individuals in the united kingdom may carry the infectious prion protein associated with variant creutzfeldt-jakob disease (vcjd), raising questions about the risk of transfusion transmission from apparently healthy carriers. blood leukoreduction shows limited efficiency against prions. therefore, in absence of antemortem diagnostic tests, prion removal filters, including the p-capt filter were designed to improve blood transfusion s ...	2015	25647476
variant creutzfeldt-jakob disease in older patients.		2015	25609647
batch recall of french plasma-derived products due to variant creutzfeldt-jakob disease risk: the psychological impact on haemophilic patients, changes in their therapeutic demands and behaviour and ethical considerations.	the choice of plasma-derived products (pdp) vs. recombinant products (rp) for treating haemophilia is influenced by the infectious and perceived safety of the products. batch recall of pdp due to the risk of variant creutzfeldt-jakob disease (vcjd) may have unfavourable psychological impacts on haemophilia patients and influence their product preferences. this study aimed to assess the psychological impact of batch recalls of pdp in six haemophilia patients and their therapeutic demands, and to ...	2015	25545300
blood reference materials from macaques infected with variant creutzfeldt-jakob disease agent.	variant creutzfeldt-jakob disease (vcjd) is a fatal neurodegenerative infection that can be transmitted by blood and blood products from donors in the latent phase of the disease. currently, there is no validated antemortem vcjd blood screening test. several blood tests are under development. any useful test must be validated with disease-relevant blood reference panels.	2015	25154296
preclinical vcjd after blood transfusion in a prnp codon 129 heterozygous patient.	we report a case of preclinical variant creutzfeldt-jakob disease (vcjd) in a patient who died from a non-neurological disorder 5 years after receiving a blood transfusion from a donor who subsequently developed vcjd. protease-resistant prion protein (prp(res)) was detected by western blot, paraffin-embedded tissue blot, and immunohistochemistry in the spleen, but not in the brain. immunohistochemistry for prion protein was also positive in a cervical lymph node. the patient was a heterozygote a ...	2015	15302196
methods for differentiating prion types in food-producing animals.	prions are an enigma amongst infectious disease agents as they lack a genome yet confer specific pathologies thought to be dictated mainly, if not solely, by the conformation of the disease form of the prion protein (prp(sc)). prion diseases affect humans and animals, the latter including the food-producing ruminant species cattle, sheep, goats and deer. importantly, it has been shown that the disease agent of bovine spongiform encephalopathy (bse) is zoonotic, causing variant creutzfeldt jakob ...	2015	26580664
transmission of scrapie prions to primate after an extended silent incubation period.	classical bovine spongiform encephalopathy (c-bse) is the only animal prion disease reputed to be zoonotic, causing variant creutzfeldt-jakob disease (vcjd) in humans and having guided protective measures for animal and human health against animal prion diseases. recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-bse. we here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly rel ...	2015	26123044
the transmissible spongiform encephalopathies of livestock.	prion diseases or transmissible spongiform encephalopathies (tses) are fatal protein-misfolding neurodegenerative diseases. tses have been described in several species, including bovine spongiform encephalopathy (bse) in cattle, scrapie in sheep and goats, chronic wasting disease (cwd) in cervids, transmissible mink encephalopathy (tme) in mink, and kuru and creutzfeldt-jakob disease (cjd) in humans. these diseases are associated with the accumulation of a protease-resistant, disease-associated ...	2015	25991695
generation of a persistently infected mdbk cell line with natural bovine spongiform encephalopathy (bse).	bovine spongiform encephalopathy (bse) is a zoonotic transmissible spongiform encephalopathy (tse) thought to be caused by the same prion strain as variant creutzfeldt-jakob disease (vcjd). unlike scrapie and chronic wasting disease there is no cell culture model allowing the replication of proteinase k resistant bse (prpbse) and the further in vitro study of this disease. we have generated a cell line based on the madin-darby bovine kidney (mdbk) cell line over-expressing the bovine prion prote ...	2015	25647616
recent us case of variant creutzfeldt-jakob disease-global implications.	variant creutzfeldt-jakob disease (vcjd) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. we describe the clinical presentation of a recent case of vcjd in the united states and provide an update on diagnostic testing. the location of this patient's exposure is less clear than those in the 3 previously reported us cases, but strong evidence indicates that exposure to contaminated beef occurred outside the united sta ...	2015	25897712
donor recruitment for fecal microbiota transplantation.	increasing demand for fecal microbiota transplantation (fmt) has created a need for stool banks sourced from long-term healthy donors. here, we describe our experience in recruiting and screening fecal donors.	2015	26070003
susceptibility of european red deer (cervus elaphus elaphus) to alimentary challenge with bovine spongiform encephalopathy.	european red deer (cervus elaphus elaphus) are susceptible to the agent of bovine spongiform encephalopathy, one of the transmissible spongiform encephalopathies, when challenged intracerebrally but their susceptibility to alimentary challenge, the presumed natural route of transmission, is unknown. to determine this, eighteen deer were challenged via stomach tube with a large dose of the bovine spongiform encephalopathy agent and clinical signs, gross and histological lesions, presence and dist ...	2015	25615837
an inventory of concerns behind blood safety policies in five western countries.	the availability of costly safety measures against transfusion-transmissible infections forces western countries to confront difficult ethical questions. how to decide about implementing such measures? when are such decisions justified? as a preliminary to addressing these questions, we assessed which concerns shape actual donor blood safety policymaking in five western countries.	2015	26331441
chronic wasting disease: fingerprinting the culprit in risk assessments.	transmissible spongiform encephalopathies (prion diseases) in animals may be associated with a zoonotic risk potential for humans as shown by the occurrence of variant creutzfeldt-jakob disease in the wake of the bovine spongiform encephalopathy epidemic. thus, the increasing exposure of humans in north america to cervid prions of chronic wasting disease (cwd) in elk and deer has prompted comprehensive risk assessments. the susceptibility of humans to cwd infections is currently under investigat ...	2015	22453172
exploring the zoonotic potential of animal prion diseases: in vivo and in vitro approaches.	following the discovery of a causal link between bovine spongiform encephalopathy (bse) in cattle and variant creutzfeldt-jakob disease (vcjd) in humans, several experimental approaches have been used to try to assess the potential risk of transmission of other animal transmissible spongiform encephalopathies (tses) to humans. experimental challenge of non-human primates, humanised transgenic mice and cell-free conversion systems have all been used as models to explore the susceptibility of huma ...	2016	24549113
detection of prions in blood from patients with variant creutzfeldt-jakob disease.	human prion diseases are infectious and invariably fatal neurodegenerative diseases. they include sporadic creutzfeldt-jakob disease (scjd), the most common form, and variant cjd (vcjd), which is caused by interspecies transmission of prions from cattle infected by bovine spongiform encephalopathy. development of a biochemical assay for the sensitive, specific, early, and noninvasive detection of prions (prp(sc)) in the blood of patients affected by prion disease is a top medical priority to inc ...	2016	28003548
detection of prions in the plasma of presymptomatic and symptomatic patients with variant creutzfeldt-jakob disease.	variant creutzfeldt-jakob disease (vcjd) is a human prion disease resulting from the consumption of meat products contaminated by the agent causing bovine spongiform encephalopathy. evidence supporting the presence of a population of silent carriers that can potentially transmit the disease through blood transfusion is increasing. the development of a blood-screening assay for both symptomatic vcjd patients and asymptomatic carriers is urgently required. we show that a diagnostic assay combining ...	2016	28003547
absence of evidence for a causal link between bovine spongiform encephalopathy strain variant l-bse and known forms of sporadic creutzfeldt-jakob disease in human prp transgenic mice.	prions are proteinaceous pathogens responsible for subacute spongiform encephalopathies in animals and humans. the prions responsible for bovine spongiform encephalopathy (bse) are zoonotic agents, causing variant creutzfeldt-jakob disease (cjd) in humans. the transfer of prions between species is limited by a species barrier, which is thought to reflect structural incompatibilities between the host cellular prion protein (prp(c)) and the infecting pathological prp assemblies (prp(sc)) constitut ...	2016	27681129
a case cluster of variant creutzfeldt-jakob disease linked to the kingdom of saudi arabia.	as of mid-2016, 231 cases of variant creutzfeldt-jakob disease-the human form of a prion disease of cattle, bovine spongiform encephalopathy-have been reported from 12 countries. with few exceptions, the affected individuals had histories of extended residence in the uk or other western european countries during the period (1980-96) of maximum global risk for human exposure to bovine spongiform encephalopathy. however, the possibility remains that other geographic foci of human infection exist, ...	2016	27671029
prion diseases: immunotargets and therapy.	transmissible spongiform encephathalopathies or prion diseases are a group of neurological disorders characterized by neuronal loss, spongiform degeneration, and activation of astrocytes or microglia. these diseases affect humans and animals with an extremely high prevalence in some species such as deer and elk in north america. although rare in humans, they result in a devastatingly swift neurological progression with dementia and ataxia. patients usually die within a year of diagnosis. prion d ...	2016	27529062
are prions transported by plasma exosomes?	blood has been shown to contain disease-associated misfolded prion protein (prp(tse)) in animals naturally and experimentally infected with various transmissible spongiform encephalopathy (tse) agents, and in humans infected with variant creutzfeldt-jakob disease (vcjd). recently, we have demonstrated prp(tse) in extracellular vesicle preparations (evs) containing exosomes from plasma of mice infected with mouse-adapted vcjd by protein misfolding cyclic amplification (pmca). here we report the d ...	2016	27499183
guinea pig prion protein supports rapid propagation of bovine spongiform encephalopathy and variant creutzfeldt-jakob disease prions.	the biochemical and neuropathological properties of bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (vcjd) prions are faithfully maintained upon transmission to guinea pigs. however, primary and secondary transmissions of bse and vcjd in guinea pigs result in long incubation periods of ∼450 and ∼350 days, respectively. to determine if the incubation periods of bse and vcjd prions could be shortened, we generated transgenic (tg) mice expressing guinea pig prion protei ...	2016	27440899
ten-year follow-up of two cohorts with an increased risk of variant cjd: donors to individuals who later developed variant cjd and other recipients of these at-risk donors.	transmission of variant creutzfeldt-jakob disease (vcjd) through blood transfusion is implicated in three deaths and one asymptomatic infection. based on this evidence, individuals assessed to be at increased risk of vcjd through donating blood transfused to individuals who later developed vcjd, or through being other recipients of such donors, are followed up to further understand the risks of vcjd transmission through blood.	2016	27432362
clinical issues-may 2016.	variations in documenting surgical wound classification key words: surgical wound classification, clean, clean-contaminated, contaminated, dirty. wearing long-sleeved jackets while preparing and packaging items for sterilization key words: long-sleeved jackets, organic material, sterile processing. endoscopic transmission of prions key words: prions, high-risk tissue, low-risk tissue, creutzfeldt-jakob disease (cjd), variant creutzfeldt-jakob disease (vcjd). wearing gloves when handling flexible ...	2016	27129755
persistent residual contamination in endoscope channels; a fluorescence epimicroscopy study.	the increasing demand for endoscopic procedures poses new contamination challenges, given developing antimicrobial resistance worldwide and potential viral or prion diseases in populations at risk. we examined working channels from reusable luminal endoscopes used in recent years.	2016	27110695
meeting report: 2015 pda virus & tse safety forum.	the report provides a summary of the presentations at the virus & tse safety forum 2015 organized by the parenteral drug association (pda) and held in cascais, portugal, from 9 to 11 june, 2015. as with previous conferences of this series, the pda virus & tse safety forum 2015 provided an excellent forum for the exchange of information and opinions between the industry, research organizations, and regulatory bodies. regulatory updates on virus and tse safety aspects illustrating current topics o ...	2016	27020643
rapid and highly sensitive detection of variant creutzfeldt-jakob disease abnormal prion protein on steel surfaces by protein misfolding cyclic amplification: application to prion decontamination studies.	the prevalence of variant creutzfeldt-jakob disease (vcjd) in the population remains uncertain, although it has been estimated that 1 in 2000 people in the united kingdom are positive for abnormal prion protein (prptse) by a recent survey of archived appendix tissues. the prominent lymphotropism of vcjd prions raises the possibility that some surgical procedures may be at risk of iatrogenic vcjd transmission in healthcare facilities. it is therefore vital that decontamination procedures applied ...	2016	26800081
mononucleated blood cell populations display different abilities to transmit prion disease by the transfusion route.	previous experiments carried out in a sheep scrapie model demonstrated that the transfusion of 200 μl of prion-infected whole blood has an apparent 100% efficacy for disease transmission. these experiments also indicated that, despite the apparent low infectious titer, the intravenous administration of white blood cells (wbc) resulted in efficient disease transmission. in the study presented here, using the same transmissible spongiform encephalopathy (tse) animal model, our aim was to determine ...	2016	26764000
whole blood gene expression profiling in preclinical and clinical cattle infected with atypical bovine spongiform encephalopathy.	prion diseases, such as bovine spongiform encephalopathies (bse), are transmissible neurodegenerative disorders affecting humans and a wide variety of mammals. variant creutzfeldt-jakob disease (vcjd), a prion disease in humans, has been linked to exposure to bse prions. this classical bse (cbse) is now rapidly disappearing as a result of appropriate measures to control animal feeding. besides cbse, two atypical forms (named h- and l-type bse) have recently been described in europe, japan, and n ...	2016	27073865
disease burden of 32 infectious diseases in the netherlands, 2007-2011.	infectious disease burden estimates provided by a composite health measure give a balanced view of the true impact of a disease on a population, allowing the relative impact of diseases that differ in severity and mortality to be monitored over time. this article presents the first national disease burden estimates for a comprehensive set of 32 infectious diseases in the netherlands.	2016	27097024
clinical features of variant creutzfeldt-jakob disease.	since 1996, over one hundred cases of variant creutzfeldt-jakob disease have appeared, mostly in the united kingdom. in this review, we summarise the major clinical features of this progressive neurodegenerative condition and compare them with those of sporadic creutzfeldt-jakob disease. we emphasise the young age (median 26 years) at presentation and the dominant psychiatric/behavioural features, particularly depression. sensory symptoms are present initially in half the cases and florid psychi ...	2016	11987139
meeting report: pda virus and tse safety forum.	the report provides a summary of the presentations and discussions of the virus & tse (transmissible spongiform encephalopathy) safety forum 2011 that was organized by the parenteral drug association and held in barcelona, spain, on 28-30 june, 2011. the conference was accompanied by a workshop named "virus removal by filtration: trends and new developments." a summary of the workshop is provided as a separate report and will be published in this journal as well. the risk of virus contamination ...	2016	23569070
creutzfeldt-jakob disease and blood transfusion: updated results of the uk transfusion medicine epidemiology review study.	this paper reports the results to 31 may 2015 of an ongoing uk study to look for additional cases of variant creutzfeldt-jakob disease (vcjd) transmission by blood transfusion, and to seek evidence whether other subtypes of creutzfeldt-jakob disease (cjd) may be transmissible via blood components.	2016	26709606
variant creutzfeldt-jakob disease deferral in canada: impact of stop dates.	to reduce the risk of variant creutzfeldt-jakob disease (vcjd) transmission via blood transfusion in canada, potential donors who spent a cumulative time in the united kingdom, western europe or saudi arabia are deferred. "stop dates" for accumulated time were later implemented for 3 months spent in the united kingdom or france (1980-1996) and for 5 years elsewhere in western europe (1980-2007); saudi arabia deferral was implemented with the "stop date" (1980-1996). we evaluated the long-term im ...	2016	28151391
blood transmission studies of prion infectivity in the squirrel monkey (saimiri sciureus): the baxter study.	four secondary transmissions of variant creutzfeldt-jakob disease (vcjd) infectivity have been associated with the transfusion of nonleukoreduced red blood cells collected from vcjd patients during the asymptomatic phase of the disease. establishing efficient experimental models for assessing the risk of future transmissions of vcjd infectivity via blood transfusion is of paramount importance in view of a study of archived appendix samples in which the prevalence of asymptomatic vcjd infection i ...	2016	26594017
efficient propagation of variant creutzfeldt-jakob disease prion protein using the cell-protein misfolding cyclic amplification technique with samples containing plasma and heparin.	to prevent the iatrogenic spread of variant creutzfeldt-jakob disease (vcjd) between humans via blood products or transfusion, highly sensitive in vitro screening tests are necessary. protein misfolding cyclic amplification (pmca) is one such candidate test. however, plasma has been reported to inhibit the pmca reaction. therefore, we investigated the cell-pmca conditions that permit vcjd prion amplification in the presence of plasma.	2016	26347231
shadoo/prp (sprn(0/0) /prnp(0/0) ) double knockout mice: more than zeroes.	shadoo (sho) is a brain glycoprotein with similarities to the unstructured region of prp (c) . frameshift alleles of the sho gene, sprn, are reported in variant creutzfeldt-jakob disease (vcjd) patients while sprn mrna knockdown in prp-null (prnp(0/0) ) embryos produces lethality, advancing sho as the hypothetical prp-like "pi" protein. also, sho levels are reduced as misfolded prp accumulates during prion infections. to penetrate these issues we created sprn null alleles (daude et al., proc. na ...	2016	22929230
a closer look at prion strains: characterization and important implications.	prions are infectious proteins that are responsible for transmissible spongiform encephalopathies (tses) and consist primarily of scrapie prion protein (prp (sc) ), a pathogenic isoform of the host-encoded cellular prion protein (prp (c) ). the absence of nucleic acids as essential components of the infectious prions is the most striking feature associated to these diseases. additionally, different prion strains have been isolated from animal diseases despite the lack of dna or rna molecules. mo ...	2017	23357828
variant creutzfeldt-jakob disease in a patient with heterozygosity at prnp codon 129.		2017	28099827
a heparin purification process removes spiked transmissible spongiform encephalopathy agent.	in 2000, bovine heparin was withdrawn from the us market for fear of contamination with bovine spongiform encephalopathy (bse) agent, the cause of variant creutzfeldt-jakob disease in humans. thus, us heparin is currently sourced only from pig intestines. availability of alternative sources of crude heparin, a life-saving drug, would benefit public health. bovine heparin is an obvious option, but bse clearance by the bovine heparin manufacturing process should be evaluated. to this end, using ha ...	2017	28116677
evaluation of rapid post-mortem test kits for bovine spongiform encephalopathy (bse) screening in japan: their analytical sensitivity to atypical bse prions.	a classical type of bovine spongiform encephalopathy (c-bse), recognized in 1987, had a large impact on public health due to its zoonotic link to variant creutzfeldt-jakob disease by the human consumption of dietary products contaminated with the c-bse prion. thus, a number of countries implemented bse surveillance using rapid post-mortem test kits that were approved for detection of the c-bse prion in the cattle brain. however, as atypical bse (l- and h-bse) cases emerged in subsequent years, t ...	2017	28358272
geographic exposure risk of variant creutzfeldt-jakob disease in us blood donors: a risk-ranking model to evaluate alternative donor-deferral policies.	variant creutzfeldt-jakob disease (vcjd) has been transmitted by blood transfusion (ttvcjd). the us food and drug administration (fda) recommends deferring blood donors who resided in or traveled to 30 european countries where they may have been exposed to bovine spongiform encephalopathy (bse) through beef consumption. those recommendations warrant re-evaluation, because new cases of bse and vcjd have markedly abated.	2017	28261810
detection and partial discrimination of atypical and classical bovine spongiform encephalopathies in cattle and primates using real-time quaking-induced conversion assay.	the transmission of classical bovine spongiform encephalopathy (c-bse) through contaminated meat product consumption is responsible for variant creutzfeldt-jakob disease (vcjd) in humans. more recent and atypical forms of bse (l-bse and h-bse) have been identified in cattle since the c-bse epidemic. their low incidence and advanced age of onset are compatible with a sporadic origin, as are most cases of creutzfeldt-jakob disease (cjd) in humans. transmissions studies in primates and transgenic m ...	2017	28231300
molecular mechanisms of chronic wasting disease prion propagation.	prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. examples include kuru, once the leading cause of death among the fore people in papua new guinea and caused by mortuary feasting; bovine spongiform encephalopathy (bse) and its subsequent transmission to humans in the form of variant creutzfeldt-jakob disease (vcjd), and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. the ...	2017	28193766