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lack of germline mutation at codon 211 of the prion protein gene (prnp) in korean native cattle - short communication.bovine prion diseases are composed of two types of bovine spongiform encephalopathy (bse), classical bse and atypical bse. recent studies have identified one case of atypical bse with an e211k mutation. e211k is homologous to the human e200k mutation, which is related to familial creutzfeldt-jakob disease (cjd), one of the familial forms of human prion diseases. to date, familial forms of prion diseases have not been reported in non-human animals. because the familial forms of human prion diseas ...201728244340
diseases, dilemmas, decisions-converting epidemiological dilemmas into successful disease control decisions.this paper describes 50 years of personal experience in dealing with a range of animal and zoonotic diseases at national and global level, using a series of selected examples to illustrate both the nature of the various dilemmas and difficulties faced, and the way in which they were solved using the tools and techniques that were available at the particular time. a major theme throughout has been the dependence on advancing computer technology, which initially allowed only simple analyses and mo ...201526072199
genetic diversity in the prion protein gene (prnp) of domestic cattle and water buffaloes in vietnam, indonesia and thailand.there has been an accumulation of information on frequencies of insertion/deletion (indel) polymorphisms within the bovine prion protein gene (prnp) and on the number of octapeptide repeats and single nucleotide polymorphisms (snps) in the coding region of bovine prnp related to bovine spongiform encephalopathy (bse) susceptibility. we investigated the frequencies of 23-bp indel polymorphism in the promoter region (23indel) and 12-bp indel polymorphism in intron 1 region (12indel), octapeptide r ...201424705506
case-control study on the risks of bse infections in northern germany.this study was to identify risk factors for bovine spongiform encephalopathy (bse) by means of individual case-control data. 43 bse cases in a defined region in lower saxony and schleswig-holstein were compared with 84 control animals. purchase of new breeding stock and cross contamination between feed on the farm did not seem to have influence on the bse incidence in these regions. the results indicate independent risk patterns. pattern 1: cows with high milk yield seemed to be at risk on big f ...201323758037
prnp and sprn genes polymorphism in atypical bovine spongiform encephalopathy cases diagnosed in polish cattle.polymorphisms in the coding region of the prion protein gene (prnp) have been associated with the susceptibility and incubation period of prion diseases in humans and sheep. however, polymorphisms in this part of the bovine prnp gene do not affect the classical bovine spongiform encephalopathy (bse) susceptibility in cattle. studies carried out in germany have shown that insertion/deletion-type polymorphisms located in the promoter region of the bovine prion gene are possible genetic factors mod ...201222723200
geographical bse risk assessment and its impact on disease detection and dissemination.bovine spongiform encephalopathy (bse) rapidly evolved into an issue of major public concern particularly when, in 1996, evidence was provided that this disease had crossed the species barrier and infected humans in the uk with what has become known as "variant creutzfeldt jakob disease" (vcjd). the aim of this paper is to describe the european geographical bse risk assessment (gbr) that was successfully used for assessing the qualitative likelihood that bse could be present in a country where i ...201222305879
neuroanatomical distribution of disease-associated prion protein in experimental bovine spongiform encephalopathy in cattle after intracerebral inoculation.the pathologic disease-associated prion protein (prp(sc)) has been shown to be expressed in the central nervous system of holstein cattle inoculated intracerebrally with 3 sources of classical bovine spongiform encephalopathy (bse) isolates. several regions of the brain and spinal cord were analyzed for prp(sc) expression by immunohistochemical and western blotting analyses. animals euthanized at 10 months post-inoculation (mpi) showed prp(sc) deposits in the brainstem and thalamus, but no vacuo ...201222274156
risk of prion disease transmission through bovine-derived bone substitutes: a systematic review.background: despite the causal association between variant creutzfeldt - jakob disease and bovine spongiform encephalopathy (bse), bovine origin graft materials are widely used during dental surgical procedures. the aim of this study was to assess the risk of bse transmission through anorganic bovine bone substitutes. methods: electronic database of medline was searched to identify relevant studies regarding our focused questions, presence of bse prion infectivity in raw bovine bone, bse prion i ...201122171533
polymorphism of the prion protein gene (prnp) in polish cattle affected by classical bovine spongiform encephalopathy.recent attempts to discover genetic factors affecting cattle resistance/susceptibility to bovine spongiform encephalopathy (bse) have led to the identification of two insertion/deletion (indel) polymorphisms, located within the promoter and intron 1 of the prion protein gene prnp, showing a significant association with the occurrence of classical form of the disease. because the effect of the polymorphisms was studied only in few populations, in this study we investigated whether previously desc ...201122170597
heart rate variability analysis in sheep affected by transmissible spongiform encephalopathies.abstract:201122168827
recent concerns about stunning and slaughter.this review summarises information that is relevant to concerns that have recently been expressed about stunning and slaughter. it is known that captive bolt stunning can result in brain material passing to the lungs via the jugular veins. if future studies show that bse prions pass beyond the lungs to the edible carcass, there will be a move away from captive bolt stunning in large cattle towards electrical stunning. greater use of electrical stunning in large cattle will increase the importanc ...200522063747
sensitive and semi-quantitative taqman™ real-time polymerase chain reaction systems for the detection of beef (bos taurus) and the detection of the family mammalia in food and feed.consumers distrust beef or products that could contain beef because of the bse (bovine spongiform encephalopathy) and vcjd (variant creutzfeld jacob disease) cases during recent years. cows could be fed with meat and bone meal-containing food. to regain consumer confidence methods are needed that allow the detection of smallest amounts of beef in the most different kind of products. polymerase chain reaction (pcr) analysis can be used to detect the smallest amounts of even highly degraded dna. i ...200322063254
Communicating Food Risks in an Era of Growing Public Distrust: Three Case Studies.The communication and regulation of risk has changed significantly over the past 30 years in Europe and to a noticeable but lesser extent in the United States. In Europe, this is partly due to a series of regulatory mishaps, ranging from mad cow disease in the United Kingdom to contamination of the blood supply in France. In the United States, general public confidence in the American government has been gradually declining for more than three decades, driven by a mix of cultural and political c ...201122050421
The role of psychological determinants and demographic factors in consumer demand for farm-to-fork traceability systems.Traceability systems are an important tool (1) for tracking, monitoring, and managing product flows through the supply chain for better efficiency and profitability of suppliers, and (2) to improve consumer confidence in the face of serious food safety incidents. After the global bovine spongiform encephalopathy (BSE) crisis affected producers, consumers, trade, and the health status of animals and humans, new systems to help confirm the status of cattle products along the supply chain from farm ...201122043914
Construction of a Policy Arena: The Case of Public Health in France.In this article we examine the transformation over the past two decades of public health as a policy arena in France from a backwater of little interest to politicians, bureaucrats, the media, and the public into a central preoccupation of the state. Recent dramatic health crises (the scandal over HIV-contaminated blood, mad cow disease, etc.) have substantially raised the political profile of (and corresponding state investment in) public health in France, offering opportunities and incentives ...201122003098
effect of prp genotype and route of inoculation on the ability of discriminatory western blot to distinguish scrapie from sheep bovine spongiform encephalopathy.procedures for discriminating scrapie from bovine spongiform encephalopathy (bse) in sheep are relevant to ascertain whether bse has entered the sheep population. this study was aimed at investigating whether the suitability of an official eu discriminative method is affected by the sheep prp genotype and route of infection.201121994325
high-risk biodegradable waste processing by alkaline hydrolysis.biodegradable waste is by definition degraded by other living organisms. every day, meat industry produces large amounts of a specific type of biodegradable waste called slaughterhouse waste. traditionally in europe, this waste is recycled in rendering plants which produce meat and bone meal and fat. however, feeding animals with meat and bone meal has been banned since the outbreaks of bovine spongiform encephalopathy (bse). in consequence, new slaughterhouse waste processing technologies have ...201121971109
BSE: where are we now? 201121965237
Simulating the bovine spongiform encephalopathy situation in Japan.Despite various measures taken by the Japanese government to protect the cattle population from exposure to the bovine spongiform encephalopathy (BSE) agent, the first case of BSE was detected in September 2001. Subsequently, BSE surveillance was enhanced, involving mandatory reporting and investigation of all clinical BSE suspects, and testing of fallen stock and all cattle slaughtered for human consumption. Tests on over nine million cattle led to the detection of 35 additional cases by the en ...201121961232
Evaluation of two sets of immunohistochemical and Western blot confirmatory methods in the detection of typical and atypical BSE cases.ABSTRACT:201121958476
experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle.prion diseases or transmissible spongiform encephalopathies (tses) of animals include scrapie of sheep and goats; transmissible mink encephalopathy (tme); chronic wasting disease (cwd) of deer, elk and moose; and bovine spongiform encephalopathy (bse) of cattle. the emergence of bse and its spread to human beings in the form of variant creutzfeldt-jakob disease (vcjd) resulted in interest in susceptibility of cattle to cwd, tme and scrapie. experimental cross-species transmission of tse agents p ...201121908269
Impact of the national full herd depopulation policy on the recurrence of bovine tuberculosis in Irish herds, 2003 to 2005.This study evaluated the impact of the Irish herd bovine tuberculosis (bTB) depopulation policy (depopulation, disinfection, contiguous testing and local badger removal where implicated) on the recurrence of bTB infection, by comparing the future risk in restocked herds following depopulation for either bTB or bovine spongiform encephalopathy (BSE) during 2003 to 2005. Each herd was assigned a 'previous bTB risk', based on bTB history during the five years before depopulation. Future bTB risk wa ...201121868436
detection of mycobacterium avium ssp. paratuberculosis in ileocaecal lymph nodes collected from elderly slaughter cows using a semi-nested is900 polymerase chain reaction.the aim of this study was to investigate the occurrence of subclinical mycobacterium avium spp. paratuberculosis (map) infections at slaughter by testing ileocaecal lymph nodes with a semi-nested is900 pcr. tissue samples were available within the framework of a parallel study investigating bse-susceptibility factors in members of bse-cohorts in the german federal state of lower saxony. ileocaecal lymph nodes were collected over a 2-year sampling period from 99 slaughter cattle of a mean age of ...201121775077
survival of embryos and calves derived from somatic cell nuclear transfer in cattle: a nationwide survey in japan.to obtain data concerning the survival of embryos and calves derived from somatic cell nuclear transfer (scnt) in japan, a nationwide survey was carried out in april, 2009. as a result, data concerning 3264 embryo transfers (ets) with scnt embryos which produced 301 calves were accumulated and their survival was analyzed. the present survey revealed that survival rates of transferred bovine embryos and produced calves derived from scnt had not improved over a decade (1998-2007). a remarkable fea ...201121729218
relaxation of bse controls 'acceptable', says fsa. 201121705505
genetic variability of the coding region for the prion protein gene (prnp) in gayal (bos frontalis).the gayal (bos frontalis) is a rare semi-wild bovid species in which bovine spongiform encephalopathy (bse) has not been reported. polymorphisms of the prion protein gene (prnp) have been correlated significantly with resistance to bse. in this study, the coding region of prnp was cloned and characterized in samples from 125 gayal. a total of ten single nucleotide polymorphisms (snps), including six silent mutations (c60t, g75a, a108t, g126a, c357t and c678t) and four mis-sense mutations (c8a, g ...201121633886
molecular discrimination of sheep bovine spongiform encephalopathy from scrapie.sheep ch1641-like transmissible spongiform encephalopathy isolates have shown molecular similarities to bovine spongiform encephalopathy (bse) isolates. we report that the prion protein prpsc from sheep bse is extremely resistant to denaturation. this feature, combined with the n-terminal prpsc cleavage, allowed differentiation of classical scrapie, including ch1641-like, from natural goat bse and experimental sheep bse.201121470463
prions: a mystery unravelled?prions result in fatal degeneration of the central nervous system (cns) in the form of diseases known as transmissible spongiform encephalopathies (tses). the discovery in 1996 of a new variant of creutzfeldt-jakob disease (a human tse) and experimental confirmation that it is caused by the prion strain responsible for bovine spongiform encephalopathy (bse) has greatly spurred research in this field. the mechanism underlying prion propagation is now reasonably clear. prions multiply, in fact, by ...201021384329
neuropathological changes in auditory brainstem nuclei in cattle with experimentally induced bovine spongiform encephalopathy.bovine spongiform encephalopathy (bse) is characterized by the appearance of spongy lesions in the brain, particularly in the brainstem nuclei. this study evaluated the degenerative changes observed in the central auditory brainstem of bse-challenged cattle. the neuropathological changes in the auditory brainstem nuclei were assessed by determining the severity of vacuolation and the presence of disease-associated prion protein (prp(sc)). sixteen female holstein-friesian calves, 2-4 months of ag ...201121333306
prpsc spreading patterns in the brain of sheep linked to different prion types.abstract: scrapie in sheep and goats has been known for more than 250 years and belongs nowadays to the so-called prion diseases that also include e.g. bovine spongiform encephalopathy in cattle (bse) and creutzfeldt-jakob disease in humans. according to the prion hypothesis, the pathological isoform (prpsc) of the cellular prion protein (prpc) comprises the essential, if not exclusive, component of the transmissible agent. currently, two types of scrapie disease are known - classical and atypic ...201121324114
comparison of brain prpd distribution in ovine bse and scrapie.scrapie and bovine spongiform encephalopathy (bse) are both prion diseases affecting ruminants, and these diseases do not share the same public health concerns. surveillance of the bse agent in small ruminants has been a great challenge, and the recent identification of diverse prion diseases in ruminants has led to the development of new methods for strain typing. in our study, using immunohistochemistry (ihc), we assessed the distribution of prp(d) in the brains of 2 experimentally bse-infecte ...201121245284
the evolution of risk perceptions related to bovine spongiform encephalopathy--canadian consumer and producer behavior.in this study the dynamics of risk perceptions related to bovine spongiform encephalopathy (bse) held by canadian consumers and cow-calf producers were evaluated. since the first domestic case of bse in 2003, canadian consumers and cow-calf producers have needed to make decisions on whether or not their purchasing/production behavior should change. such changes in their behavior may relate to their levels of risk perceptions about bse, risk perceptions that may be evolving over time and be affec ...201121218347
analysis of gene expression in white blood cells of cattle orally challenged with bovine amyloidotic spongiform encephalopathy.bovine amyloidotic spongiform encephalopathy (base) is one of the recently discovered atypical forms of bse, which is transmissible to primates, and may be the bovine equivalent of sporadic creutzfeldt-jacob disease (cjd) in humans. although it is transmissible, it is unknown whether base is acquired through infection or arises spontaneously. in the present study, the gene expression of white blood cells (wbcs) from 5 cattle at 1 yr after oral base challenge was compared with negative controls u ...201121218338
transmissible encephalopathy agents: virulence, geography and clockwork.transmissible spongiform encephalopathies (tses) are caused by infectious agents with stable virulence characteristics that are not encoded by the host. agent-specific features of virulence include variable disease latency and tissue pathology in a given host, as well as the ability to spread to many species. such cross-species infections contradict predictions based on the prion hypothesis. recent transmissions of several human agents to normal mice and to monotypic neural cells in culture, und ...201021178425
bovine spongiform encephalopathy: investigation of phenotypic variation among passive surveillance cases.bovine spongiform encephalopathy (bse) is a prion disease of domesticated cattle, first identified in great britain (gb) in 1986. the disease has been characterized by histopathological, immunohistochemical, biochemical and biological properties, which have shown a consistent disease phenotype among cases obtained by passive surveillance. with the advent of active surveillance in 2001, immunological tests for detection of the prion protein revealed some cases with different biochemical character ...201021145564
atypical transmissible spongiform encephalopathies in ruminants: a challenge for disease surveillance and control.since 1987, when bovine spongiform encephalopathy (bse) emerged as a novel disease in cattle, enormous efforts were undertaken to monitor and control the disease in ruminants worldwide. the driving force was its high economic impact, which resulted from trade restrictions and the loss of consumer confidence in beef products, the latter because bse turned out to be a fatal zoonosis, causing variant creutzfeldt-jakob disease in human beings. the ban on meat and bone meal in livestock feed and the ...201021088166
risk ranking: investigating expert and public differences in evaluating food safety hazards.the allocation of resources with respect to food safety issues requires that decision makers prioritize these issues, which may conflict with the public's opinions on these matters. the purpose of this study was to compare how canadian expert and lay respondents rank different food hazards, with a view to better understanding their underlying rationales for making decisions on food safety. a carnegie mellon risk ranking model was adapted for use by individuals with different backgrounds to rank ...201021067676
enhanced enteric invasion of scrapie agents into the villous columnar epithelium via maternal immunoglobulin.transmissible spongiform encephalopathies (tse) are caused by dietary oral exposure to infectious prion proteins (prpsc); however, the mechanism behind the uptake of prpsc in the intestines is poorly understood. in addition, epidemiological studies of bse showed that most cattle are exposed to the agents in the first 6 months of life, during the suckling and weaning periods. in the present study, to elucidate the enteric invasion mechanism of prions and to investigate the age-dependent transmiss ...201021042778
an overview of the legislation and light microscopy for detection of processed animal proteins in feeds.from the first cases of bovine spongiform encephalopathy (bse) among cattle in the united kingdom in 1986, the route of infection of bse is generally believed by means of feeds containing low level of processed animal proteins (paps). therefore, many feed bans and alternative and complementary techniques were resulted for the bse safeguards in the world. now the feed bans are expected to develop into a "species to species" ban, which requires the corresponding species-specific identification met ...201020945460
experimental transmission of h-type bovine spongiform encephalopathy to bovinized transgenic mice.to characterize the biological and biochemical properties of h-type bovine spongiform encephalopathy (bse), a transmission study with a canadian h-type isolate was performed with bovinized transgenic mice (tgboprp), which were inoculated intracerebrally with brain homogenate from cattle with h-type bse. all mice exhibited characteristic neurologic signs, and the subsequent passage showed a shortened incubation period. the distribution of disease-associated prion protein (prp(sc)) was determined ...201020921323
prnp haplotype associated with classical bse incidence in european holstein cattle.classical bovine spongiform encephalopathy (bse) is an acquired prion disease of cattle. the bovine prion gene (prnp) contains regions of both high and low linkage disequilibrium (ld) that appear to be conserved across bos taurus populations. the region of high ld, which spans the promoter and part of intron 2, contains polymorphic loci that have been associated with classical bse status. however, the complex genetic architecture of prnp has not been systematically tested for an association with ...201020862290
transcytosis of murine-adapted bovine spongiform encephalopathy agents in an in vitro bovine m cell model.transmissible spongiform encephalopathies (tse), including bovine spongiform encephalopathy (bse), are fatal neurodegenerative disorders in humans and animals. bse appears to have spread to cattle through the consumption of feed contaminated with bse/scrapie agents. in the case of an oral infection, the agents have to cross the gut-epithelial barrier. we recently established a bovine intestinal epithelial cell line (bie cells) that can differentiate into the m cell type in vitro after lymphocyti ...201020861256
digestion and transportation of bovine spongiform encephalopathy-derived prion protein in the sheep intestine.bovine spongiform encephalopathy (bse) is acquired orally and the mechanisms involved in the absorption and transportation of infectivity across the gut wall are therefore critical. isolated gut loops were created in lambs, massaged to remove intestinal contents (flushed) or left non-flushed, inoculated with cattle bse homogenate and excised at different time-points. gut loops were examined by immunohistochemistry (ihc) for disease-associated prion protein (prp(d)), and the contents were analyse ...201020826616
examination of the offspring of a japanese cow affected with l-type bovine spongiform encephalopathy.the offspring of a beef cow affected with l-type bovine spongiform encephalopathy (l-bse) was kept in a pen at a bse-dedicated animal facility till the offspring was 48 months of age. the steer was then euthanized and subjected to a test for bse. the abnormal isoform of the prion protein was not detected in the brain and spinal cord of the steer. transmission of l-bse was not observed during 4 years of observation, though the steer was born when the dam was in the terminal stages of the disease.201120805640
increasing hybridoma viability and antibody repertoire after the cell fusion by the use of human plasma as an alternative supplement.prion diseases such as bovine spongiform encephalopathy (bse) and new variant creutzfeldt-jakob disease (nvcjd) have caused a major safety concern in cell cultures using fetal calf serum (fcs). in this study, we found that screened and tested human plasma (hp) obtained from blood centers may be an ideal alternate nutrient substitute to fcs for culturing hybridoma. in addition to the inherent safety, a ten-fold increase in the fusion efficiency has been observed if the hp was used as the nutrient ...201020723546
differential protein expression profiling in bse disease.bovine spongiform encephalopathy (bse) is a fatal neurodegenerative disease affecting cattle. current tests for the detection of bse are based solely on the only definitive marker of the disease, an abnormal conformer (prp(d)), of the host encoded prion protein (prp(c)). recent evidence that other transmissible spongiform encephalopathy diseases can be present in the absence of prp(d), coupled with the need to establish pre-mortem diagnostic assays have led to a search for alternative diagnostic ...201020698762
risk reduction strategies for variant creutzfeldt-jakob disease transmission by uk plasma products and their impact on patients with inherited bleeding disorders.summary: the appearance and rapid evolution of bse in uk cattle in the mid 1980s, with compelling data supporting variant creutzfeldt-jakob disease (vcjd) as its human manifestation, pose a potentially severe threat to public health. three clinical cases and one asymptomatic case of vcjd infection have been reported in uk recipients of non-leucodepleted red cell transfusions from donors subsequently diagnosed with vcjd. plasma from both these and other donors who later developed vcjd has contrib ...201020487442
epidemiological features of the bovine spongiform encephalopathy epidemic in japan.on 10 september 2001, the first case of bovine spongiform encephalopathy (bse) in japan was confirmed in a five-year-old dairy cow born in hokkaido and raised in chiba prefecture. subsequently, bse surveillance was enhanced. as a result, 35 additional cases were detected by the end of march 2009, with two to ten cases being detected each year up to the end of 2007. the epidemic appeared to peak in 2006. cases are detected mostly in dairy cattle; in cattle born in 1996 and 2000; and in cattle bor ...200920462152
[protection from bse : efforts, measures, success, and costs].by the mid 1980s, bovine spongiform encephalopathy (bse) emerged in the united kingdom (uk) and reached its peak in the early 1990s with up to 37,000 cases. in the year 2000, bse was diagnosed for the first time for a cow born in germany. since then, 413 cases of bse have been detected. about 10 years after the first bse cases were detected, variant creutzfeldt-jakob disease (vcjd), a new variant of creutzfeldt-jakob disease (cjd), was described in the uk. legal measures for protection from bse ...201020449555
bovine spongiform encephalopathy in japan and options for control.since september 2001, japan has reported to the world organisation for animal health (oie: office international des epizooties) 31 cases of typical bovine spongiform encephalopathy (bse) and two atypical cases. there were at least two major exposure episodes in japan. the first, attributed mainly to imported contaminated feed ingredients, occurred between 1995 and 1996 and 13 affected holstein cows (group a) were detected in different parts of japan. the second exposure occurred in hokkaido, the ...200720411498
a 2cm genome-wide scan of european holstein cattle affected by classical bse.classical bovine spongiform encephalopathy (bse) is an acquired prion disease that is invariably fatal in cattle and has been implicated as a significant human health risk. polymorphisms that alter the prion protein of sheep or humans have been associated with variations in transmissible spongiform encephalopathy susceptibility or resistance. in contrast, there is no strong evidence that non-synonymous mutations in the bovine prion gene (prnp) are associated with classical bse disease susceptibi ...201020350325
mouse neuronal cells expressing exogenous bovine prnp and simultaneous downregulation of endogenous mouse prnp using sirnas.prion diseases, which are called transmissible spongiform encephalopathies (tses), comprise a group of fatal infectious neurodegenerative disorders. investigation of prion strains and generation of species dependent tse model are necessary to understand pathogenesis of the disease. to establish a bse-specific in vitro cell culture model, n2a and gt1 mouse neuronal cell lines were generated to express the bovine prion protein by transfection of the bovine prion gene (prnp). in addition, the endog ...201020215868
gene and haplotype polymorphisms of the prion gene (prnp) in japanese brown, japanese native and holstein cattle.polymorphisms in the prion protein gene (prnp) are known to be associated with transmissible spongiform encephalopathies in human, sheep and goats. there is tentative association between prnp promoter polymorphism and bovine spongiform encephalopathy (bse) susceptibility in cattle. in this study, we genotyped for six bovine prnp polymorphic sites including a 23-bp indel in the promoter, a 12-bp indel in the intron 1, two nonsynonymous single nucleotide polymorphisms (snps), octapeptide repeats i ...200920163615
prion protein gene (prnp) polymorphisms in native chinese cattle.polymorphisms in four regions of the bovine prion protein gene (prnp) confer susceptibility to bovine spongiform encephalopathy (bse). these polymorphisms include a 23-bp insertion/deletion (indel) in the promoter region, a 12-bp indel in intron 1, an octapeptide repeat or 24-bp indel in the open reading frame, and a single nucleotide polymorphism (snp) in the coding region. in this study, we investigated the frequency distributions of genotypes, alleles, and haplotypes at these indel sites in 3 ...201020140032
in this issue.the proteome of an innate small molecule drug: pp. 23-37isatin is an interesting molecule in its own right - endogenous, it is delivered in a discrete pattern to specific organs, including the brain. it is also the starting material for synthesis of a diverse set of pharmaceuticals and pesticides. what are the targets of this multi-talented molecule? buneeva et al. tackled the question with proteomic tools, including affinity chromatography and seldi, and demonstrated the utility of a validated ...200920043301
[m129v prnp gene polymorphism in castilla y león shows a similar distribution to other spanish regions and other european countries].it has been reported that the polymorphism m129v in the prnp gene modifies the phenotype in all the subtypes of prion diseases in humans. all cases diagnosed to date as suffering the human variant of mad cow disease (vcjd) carry the allele 129m. in the last five years five cases of vcjd have been reported in spain, three of them in castilla y león (in western spain). observation of differences in the genotypic frequency of this polymorphism in different populations prompted us to analyze the dis ...201020004419
food safety and food labeling from the viewpoint of the consumers.distrust of food safety has grown among the japanese people after the occurrence of bovine spongiform encephalitis (bse) in 2001. the food safety commission was formed under the cabinet office and made a network among the ministries. the newly-established consumer agency may strengthen the quick response to emergencies. shoku-iku (food and dietary education) law is being implemented by the cabinet office with cooperation from relevant ministries and ngos. food sanitation law and health promotion ...200919965344
passive surveillance for bse in great britain, 2005 to 2008. 200919933544
detection of prion gene promoter and intron1 indel polymorphisms in anatolian water buffalo (bubalus bubalis).bovine spongiform encephalopathy (bse) is a fatal disease caused by miss folded prion protein. studies in the cattle, comparing genetic data from bse diseased and healthy animals have shown that indel polymorphisms in the promoter and intron 1 of prnp gene were associated with disease susceptibility. several studies were conducted to find out allele and genotypic frequencies of indel polymorphisms in promoter and intron 1 of the cattle prnp gene. unlike domestic cattle and bison, no indel polymo ...200919912420
biocrude oils from the fast pyrolysis of poultry litter and hardwood.the safe and economical disposal of poultry litter is becoming a major problem for the usa poultry industry. current disposal methods such as land application and feeding to cattle are now under pressure because of pollution of water resources due to leaching, runoffs and concern for mad cow disease contamination of the food chain. incineration or combustion is potentially applicable to large scale operations, but for small scale growers and epa non-attainment areas, this is not a suitable optio ...201019880302
increase in cd230 (cellular prion protein) fluorescence on blood lymphocytes in bovine spongiform encephalopathy-infected nonhuman primates.the cellular prion protein (prp(c)) plays a central role in prion diseases such as variant creutzfeldt-jakob disease. this disease can be transmitted by blood transfusion. however, the exact kinetics of blood infectivity and the blood fraction carrying infectivity have not yet been identified.201019843289
knockdown of the prion gene expression by rna interference in bovine fibroblast cells.prnp is the gene encoding prion protein whose misfolded and β-sheet-rich isoform is the infectious agent of transmissible spongiform encephalopathy (tse). tse, also called prion diseases, cause fatal neurodegenerative and transmissible disorders in human and animals. among these diseases, bovine spongiform encephalopathy (bse) has tremendous impact on economy and human health in the world. in the present study, we hypothesize suppression of the prnp gene expression could raise resistance to bse ...201019821149
molecular analysis of carbohydrate n-acetylgalactosamine 4-o sulfotransferase 8 (chst8) as a candidate gene for bovine spongiform encephalopathy susceptibility.endogenous prion proteins (prp) play the central role in the pathogenesis of transmissible spongiform encephalopathies. the carbohydrate n-acetylgalactosamine 4-o sulfotransferase 8 (chst8) promotes the conversion of the cellular prp(c) into the pathogenic prp(d). six sequence variants within the chst8 gene were identified by comparative sequencing and genotyped for a sample of 623 animals comprising bovine spongiform encephalopathy (bse)-affected and healthy control cows representing german fle ...201019744145
establishment of bovine prion peptide-based monoclonal antibodies for identifying bovine prion.to obtain high titer monoclonal antibodies (mcabs) which can react with mammalian prion protein (prp), balb/c mice were immunized with bovine (bo) prp peptide (boprp 209-228 aa) coupled to keyhole limpet hemocyanin (klh). the hybridoma cell lines secreting monoclonal antibodies against the peptide were established by cell fusion and cloning. the obtained mcabs were applied to detect recombinant human, bovine and hamster prp, cellular prion protein (prp(c)) in normal bovine brain and pathogenic s ...200919727594
surviving bovine spongiform encephalopathy (bse): farm women's discussion of the effects of bse on food provisioning practices.the economic vulnerability of beef and other farm families following the bovine spongiform encephalopathy (bse) crisis in canada poses a risk to their household food provisioning practices. the purpose of this study was to examine the effects of bse since 2003 on the food provisioning practices of beef and other farm families in three canadian provinces. semi-structured, face-to-face, in-depth interviews were conducted with 22 farm women (6 in alberta, 6 in ontario, and 10 in nova scotia) that f ...200919697243
prionet canada: a network of centres of excellence for research into prions and prion diseases.prionet canada's strength in basic, applied, and social research is helping to solve the food, health safety, and socioeconomic problems associated with prion diseases. prion diseases are transmissible, fatal neurodegenerative diseases of humans and animals. examples of prion diseases include bovine spongiform encephalopathy (bse, commonly known as "mad cow" disease), creutzfeldt-jakob disease in humans, and chronic wasting disease (cwd) in deer and elk. as of march 31, 2008, prionet's interdisc ...200919697232
impact of potential changes to the current bovine spongiform encephalopathy surveillance programs for slaughter cattle and fallen stock in japan.cattle slaughtered in japan for human consumption, regardless of their age, have been tested for bovine spongiform encephalopathy (bse) since october 2001. beginning in april 2004, all fallen stock from 24 months of age also have been tested. we evaluated the impact of potential changes to the current bse surveillance programs for both slaughter cattle and fallen stock using a simple stochastic model. we calculated the probability that a bse-infected dairy cow, wagyu beef animal, wagyu-holstein ...200919681270
a traceback phenomenon can reveal the origin of prion infection.the transmission of prions to animals with incongruent prion protein (prp) gene (referred to as cross-sequence transmission) results in a relatively long incubation period and can generate a new prion strain with unique transmissibility designated as a traceback phenomenon. for example, cross-sequence transmission of bovine spongiform encephalopathy (bse) prions to human generated variant creutzfeldt-jakob disease (vcjd) prions which retained the transmissibility to mice expressing bovine prp. t ...200919659941
evaluation of the possible transmission of bse and scrapie to gilthead sea bream (sparus aurata).in transmissible spongiform encephalopathies (tses), a group of fatal neurodegenerative disorders affecting many species, the key event in disease pathogenesis is the accumulation of an abnormal conformational isoform (prp(sc)) of the host-encoded cellular prion protein (prp(c)). while the precise mechanism of the prp(c) to prp(sc) conversion is not understood, it is clear that host prp(c) expression is a prerequisite for effective infectious prion propagation. although there have been many stud ...200919636413
seasonal pattern of mortality and relationships between mortality and temperature-humidity index in dairy cows.the 2 studies described investigated seasonal variations of mortality and temperature-humidity index (thi)-mortality relationships in dairy cows. mortality data were extracted from the italian bovine spongiform encephalopathy databases, which contain records on cows older than 24 mo that died on a farm from all causes (98% of total records), were slaughtered in an emergency state, or were sent for normal slaughter but were sick in the preslaughter inspection (2% of total records). both studies e ...200919620660
protective effect of the t112 prp variant in sheep challenged with bovine spongiform encephalopathy.sheep with an arq/arq prnp genotype at codon positions 136/154/171 are highly susceptible to experimental infection with bovine spongiform encephalopathy (bse). however, a number of sheep challenged orally or intracerebrally with bse were clinically asymptomatic and found to survive or were diagnosed as bse-negative when culled. sequencing of the full prnp gene open reading frame of bse-susceptible and -resistant sheep indicated that, in the majority of suffolk sheep, resistance was associated w ...200919587133
immunohistochemical characterisation of classical scrapie neuropathology in sheep.neuroinflammation elicited by prp(res) (resistant prion protein [prp]) deposits in the central nervous system (cns) has been shown to involve cellular and oxidative stress responses in bovine spongiform encephalopathy (bse) as well as in several murine models of transmissible spongiform encephalopathy (tse). additionally, deregulation of water homeostasis has been suggested to be a further component of the spongiform changes observed in tses. the aim of the present study was to characterize the ...200919515381
shadoo (sprn) and prion disease incubation time in mice.prion diseases are transmissible neurodegenerative disorders of mammalian species and include scrapie, bovine spongiform encephalopathy (bse), and variant creutzfeldt-jakob disease (vcjd). the prion protein (prp) plays a key role in the disease, with coding polymorphism in both human and mouse influencing disease susceptibility and incubation time, respectively. other genes are also thought to be important and a plausible candidate is sprn, which encodes the prp-like protein shadoo (sho). sho is ...200919513788
retinal function and morphology are altered in cattle infected with the prion disease transmissible mink encephalopathy.transmissible spongiform encephalopathies (tses) are a group of diseases that result in progressive and invariably fatal neurologic disease in both animals and humans. tses are characterized by the accumulation of an abnormal protease-resistant form of the prion protein in the central nervous system. transmission of infectious tses is believed to occur via ingestion of prion protein-contaminated material. this material is also involved in the transmission of bovine spongiform encephalopathy ("ma ...200919429980
comparison of strategies for substantiating freedom from scrapie in a sheep flock.the public health threat represented by a potential circulation of bovine spongiform encephalopathy agent in sheep population has led european animal health authorities to launch large screening and genetic selection programmes. if demonstrated, such a circulation would have dramatic economic consequences for sheep breeding sector. in this context, it is important to evaluate the feasibility of qualification procedures that would allow sheep breeders demonstrating their flock is free from scrapi ...200919405956
updated prediction for the bse epidemic in dairy cattle in japan.following the detection of the first case of bovine spongiform encephalopathy (bse) in japan in september 2001, nine million cattle were tested for bse up to the end of 2008. as a result, a further 28 cases were detected in dairy cattle. using the mathematical model previously developed and surveillance data up to the end of 2008, we estimated the prevalence of bse-infected animals within each birth cohort for the years 1995-2001. we predicted historic and future trends in the number of bse-infe ...200919356815
early dysfunction of central 5-ht system in a murine model of bovine spongiform encephalopathy.the hypothesis of an early vulnerability of the serotonergic system to prion infection was investigated in a murine model of bovine spongiform encephalopathy (bse). behavioral tests targeted to 5-ht functions were performed in the course of infection to evaluate circadian activity, anxiety-like behavior, pain sensitivity and the 5-ht syndrome. the first behavioral change was a decrease in nocturnal activity detected at 30% of incubation time. further behavioral alterations including nocturnal hy ...200919285121
risk of introduction of bse into japan by the historical importation of live cattle from the united kingdom.all cattle imported from the united kingdom to japan since 1980 and slaughtered before 2002 were traced (n=33), and the number of cattle that were possibly infected with bse and entered the animal feed chain was calculated. because there was no effective system to avoid recycling of the bse agent via animal feed until the early 1990s, of the 33 cattle imported from the uk into japan, most probably 7 or 8 were infected and entered the animal feed chain, 2 of which entered the animal feed chain in ...200919262022
investigation of the effect of glycosylation on human prion protein by molecular dynamics.prion protein conformational isomerization, prp(c)-->prp(sc), has been attributed as the cause of tse diseases such as mad-cow disease. the mechanism of such isomerization, however, is little known due the experimental difficulties in studying the scrapie form. among factors that affect prp isomerization, the role which glycosylation plays remains vague. the number of innumerous glycan species, together with their high flexibility, leads to ineffective structural characterization. in this resear ...200919236103
detection of pathologic prion protein in the olfactory bulb of natural and experimental bovine spongiform encephalopathy affected cattle in great britain.to investigate the relative involvement of the olfactory region in classical bovine spongiform encephalopathy (bse), immunohistochemical labeling of prion protein scrapie (prp(sc)) was scored in the brainstem, frontal cerebral cortex, and olfactory bulb of cattle with natural and experimental clinical cases of bse in great britain. the intensity of immunolabeling was greatest in the brainstem, but prp(sc) was also detected in the olfactory bulb and the cerebral cortex. a diffuse, nonparticulate ...200919112116
disease-specific motifs can be identified in circulating nucleic acids from live elk and cattle infected with transmissible spongiform encephalopathies.to gain insight into the disease progression of transmissible spongiform encephalopathies (tse), we searched for disease-specific patterns in circulating nucleic acids (cna) in elk and cattle. in a 25-month time-course experiment, cnas were isolated from blood samples of 24 elk (cervus elaphus) orally challenged with chronic wasting disease (cwd) infectious material. in a separate experiment, blood-sample cnas from 29 experimental cattle (bos taurus) 40 months post-inoculation with clinical bovi ...200919059996
evaluation of methods for removing central nervous system tissue contamination from the surface of beef carcasses after splitting.since high levels of prions, the causative agent of bovine spongiform encephalopathy (bse), accumulate in the brain and spinal cord, contamination of beef carcasses with central nervous system tissue (cnst) may occur at post-slaughter process. in this study, we investigated cnst contamination on the surface of beef carcasses using glial fibrillary acidic protein as a marker after splitting and evaluated the effects of washing procedures on contamination removal. high levels of cnst contamination ...200819057142
pathogenesis of experimental bovine spongiform encephalopathy (bse): estimation of tissue infectivity according to incubation period.this paper reports the results of tissue infectivity assays of bovine spongiform encephalopathy (bse) agent in orally exposed cattle at stages during the incubation period. estimations of the titre of infectivity in central nervous system (cns), certain peripheral nerve ganglia and distal ileum tissue were made according to time post exposure from the relationship between incubation period and dose for riii mice and c57bl mice using data from titrations of brain material from cases of bse. the r ...200918950589
frequencies of polymorphisms associated with bse resistance differ significantly between bos taurus, bos indicus, and composite cattle.transmissible spongiform encephalopathies (tses) are neurodegenerative diseases that affect several mammalian species. at least three factors related to the host prion protein are known to modulate susceptibility or resistance to a tse: amino acid sequence, atypical number of octapeptide repeats, and expression level. these factors have been extensively studied in breeds of bos taurus cattle in relation to classical bovine spongiform encephalopathy (bse). however, little is currently known about ...200818808703
detection of bovine spongiform encephalopathy-related prion protein gene promoter polymorphisms in local turkish cattle.polymorphisms in open reading frames of the prion protein gene (prnp) have been shown to be associated with prion disease susceptibility in humans, sheep, and mice. studies in recent years have demonstrated a similar effect of prnp promoter and intron-1 polymorphisms on bovine spongiform encephalopathy (bse) susceptibility in cattle. in this study, the deletion/insertion (indel) polymorphisms of the bovine prnp gene within the promoter sequence (23 bp) and intron 1 (12 bp) were analyzed in local ...200818800245
a sequencing strategy for identifying variation throughout the prion gene of bse-affected cattle.classical and atypical bovine spongiform encephalopathies (bses) are cattle prion diseases. distinct bovine prion gene (prnp) alleles have been associated with classical and atypical bse susceptibility. however, the full extent of prnp allele association with bse susceptibility is not known. a systematic sequence-based genotyping method that detects variation throughout prnp would be useful for: 1) detecting rare prnp alleles that may be present in bse-affected animals and 2) testing prnp allele ...200818710485
prion infected meat-and-bone meal is still infectious after biodiesel production.the epidemic of bovine spongiform encephalopathy (bse) has led to a world-wide drop in the market for beef by-products, such as meat-and-bone meal (mbm), a fat-containing but mainly proteinaceaous product traditionally used as an animal feed supplement. while normal rendering is insufficient, the production of biodiesel from mbm has been suggested to destroy infectivity from transmissible spongiform encephalopathies (tses). in addition to producing fuel, this method simultaneously generates a nu ...200818698417
control of scrapie in the uk sheep population.scrapie is a fatal transmissible spongiform encephalopathy (tse) of sheep, endemic in the uk for centuries. interest in the disease has been heightened over the last decade by the possibility of the related bse being transmissible to and between sheep and a range of control interventions has been proposed and implemented. in this paper, we examined the effect of these policies and their components on observed case rate, susceptible allele frequency and r0 within the framework of a large simulati ...200918687157
detection and characterization of proteinase k-sensitive disease-related prion protein with thermolysin.disease-related prp(sc) [pathogenic prp (prion protein)] is classically distinguished from its normal cellular precursor, prp(c)(cellular prp) by its detergent insolubility and partial resistance to proteolysis. although molecular diagnosis of prion disease has historically relied upon detection of protease-resistant fragments of prp(sc) using pk (proteinase k), it is now apparent that a substantial fraction of disease-related prp is destroyed by this protease. recently, thermolysin has been ide ...200818684106
spatial analysis of bse cases in the netherlands.in many of the european countries affected by bovine spongiform encephalopathy (bse), case clustering patterns have been observed. most of these patterns have been interpreted in terms of heterogeneities in exposure of cattle to the bse agent. here we investigate whether spatial clustering is present in the dutch bse case data.200818559085
characterization of a 320-kb region containing the hexa gene on bovine chromosome 10 and analysis of its association with bse susceptibility.bovine spongiform encephalopathy (bse) belongs to a group of neurodegenerative diseases known as transmissible prion diseases. recently, variants in the promoter region of the prion protein (prnp) gene have been shown to have a considerable effect on the susceptibility to bse. however, a previous genome scan revealed other putative bse-susceptibility loci. here, we analysed such a region on bta10, which contains the functional candidate gene hexa. three hundred and twenty kilobases that, besides ...200818513278
alpha-hemoglobin stabilizing protein is not a suitable marker for a screening test for variant creutzfeldt-jakob disease.a test is needed to identify blood donors who are in the preclinical phase of variant creutzfeldt-jakob disease (cjd). alpha-hemoglobin stabilizing protein (ahsp; syn. eraf, edrf) transcript levels are reduced in the blood of mice incubating transmissible spongiform encephalopathy.200818503615
transmissible spongiform encephalopathy strain-associated diversity of n-terminal proteinase k cleavage sites of prp(sc) from scrapie-infected and bovine spongiform encephalopathy-infected mice.assessment of the different conformational states of the abnormal prion protein (prp(sc)) in the cns provides an established basis for distinguishing transmissible spongiform encephalopathy (tse) strains. prp(sc) conformers are variably resistant to n-terminal proteinase k (pk) digestion, and analysis of the consensus products (prp(res)) by immunoassay enables effective, but relatively low-resolution differentiation. determination of the precise n-terminal amino acid profile (n-taap) of prp(res) ...200818484354
epidemiological study on bse outbreak in japan.the aim of this study is to identify, as hypotheses, all feasible sources and routes of infection for the bse cases in japan, and to study the probability of each hypothesis. the strategy of this epidemiological study is as follows. 1) bse risk status in japan is tentatively divided into 3 stages, i.e., before 1996 april when administrative guidance for feed ban of cattle mbm introduced. after that to 2001 september, the first case of bse in japan, then, after 2001 october with real feed ban in ...200818460825
frequencies of prnp gene polymorphisms in vietnamese dairy cattle for potential association with bse.summary since 2004, significant associations between bovine spongiform encephalopathy (bse) susceptibility in cattle and frequencies of insertion/deletion (ins/del; indel) polymorphisms within the bovine prion protein gene (prnp) have been reported. in this study, we investigated the frequencies of indel polymorphisms within two variable sites, a 23-bp indel polymorphism in the promoter region (23indel) and a 12-bp indel polymorphism in intron 1 region (12indel), in the prnp in 206 vietnamese da ...200818399944
rapid typing of transmissible spongiform encephalopathy strains with differential elisa.the bovine spongiform encephalopathy (bse) agent has been transmitted to humans, leading to variant creutzfeldt-jakob disease. sheep and goats can be experimentally infected by bse and have been potentially exposed to natural bse; however, whether bse can be transmitted to small ruminants is not known. based on the particular biochemical properties of the abnormal prion protein (prpsc) associated with bse, and particularly the increased degradation induced by proteinase k in the n terminal part ...200818394279
could pets be of help in achieving health literacy? a media analysis demonstration study.this paper asks whether, when seeking to reach the public, interest in the health of pets merits consideration. our data set consisted of 128 items from canadian media coverage, 1996-2006, that dealt with bovine spongiform encephalopathy (bse) as well as with cats, dogs or both. three main messages regarding pet health and human health were identified: 'do not worry', 'do worry' and 'be cautious'. a minority of articles did not convey a pet health message or a human health message (6%), and cont ...200918359950
association of a bovine prion gene haplotype with atypical bse.atypical bovine spongiform encephalopathies (bses) are recently recognized prion diseases of cattle. atypical bses are rare; approximately 30 cases have been identified worldwide. we tested prion gene (prnp) haplotypes for an association with atypical bse.200818350166
tse pathogenesis in cattle and sheep.many studies have been undertaken in rodents to study the pathogenesis of transmissible spongiform encephalopathies (tse). only a few studies have focused on the pathogenesis of bovine spongiform encephalopathy (bse) and scrapie in their natural hosts. in this review, we summarize the most recent insights into the pathogenesis of bse and scrapie starting from the initial uptake of tse agents and crossing of the gut epithelium. following replication in the gut-associated lymphoid tissues (galt), ...200818258167
phenotypic similarity of transmissible mink encephalopathy in cattle and l-type bovine spongiform encephalopathy in a mouse model.transmissible mink encepholapathy (tme) is a foodborne transmissible spongiform encephalopathy (tse) of ranch-raised mink; infection with a ruminant tse has been proposed as the cause, but the precise origin of tme is unknown. to compare the phenotypes of each tse, bovine-passaged tme isolate and 3 distinct natural bovine spongiform encephalopathy (bse) agents (typical bse, h-type bse, and l-type bse) were inoculated into an ovine transgenic mouse line (tgovprp4). transgenic mice were susceptibl ...200718258040
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