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transmission barriers for bovine, ovine, and human prions in transgenic mice.transgenic (tg) mice expressing full-length bovine prion protein (boprp) serially propagate bovine spongiform encephalopathy (bse) prions without posing a transmission barrier. these mice also posed no transmission barrier for suffolk sheep scrapie prions, suggesting that cattle may be highly susceptible to some sheep scrapie strains. tg(boprp) mice were also found to be susceptible to prions from humans with variant creutzfeldt-jakob disease (cjd); on second passage in tg(boprp) mice, the incub ...200515827140
[the requirements of society in terms of the safety of food of animal origin--a reflection on the case of france].are consumers obsessed by 'zero risk'? is their relationship to food of animal origin irrational? the comments and reactions during the bovine spongiform encephalopathy (bse) crisis that began in november 2000 certainly suggest so. however, sociological surveys conducted at the height of the crisis refute this impression: judging by individual perceptions, risk is not an obsession; it is only one of several aspects of people's relationship to animal products. furthermore, concern about healthy e ...200315884575
[variant creutzfeldt-jakob disease in france: estimating the number of cases related to travel to the united kingdom between 1980 and 1995].the outbreak of variant creutzfeldt-jakob disease (vcjd) cases rose serious concerns about secondary transmission of the disease, particularly through blood transfusion. protective measures leading to the exclusion of potentially infectious blood donors were settled: in france, donors who had stayed more than one year in the uk were excluded. in this work, which was part of a larger study aiming to estimate the french epidemic of vcjd, the number of vcjd cases who were infected during a trip to ...200515888987
expression of prion protein in the gut of mice infected orally with the 301v murine strain of the bovine spongiform encephalopathy agent.transmissible spongiform encephalopathies (tses) are characterized by the accumulation of an abnormal, disease-associated prion protein (prp(d)). expression of its normal cellular counterpart (prp(c)) by the host is a pre-requisite for the spread of infection to the central nervous system and the development of disease. moreover, cells expressing prp(c) at specific sites such as the gastrointestinal tract might be regarded as the initial point of prp(c)-prp(d) conversion after infection by the o ...200515893985
differentiation of bovine from porcine gelatines using polyclonal anti-peptide antibodies in indirect and competitive indirect elisa.gelatine is a collagen derivative obtained from bones and hides/skin mainly from bovine and pigs. as a consequence of the outbreak of bovine spongiform encephalopathy (bse), the use of bovine gelatine in feed, food and pharmaceutical products has been restricted by regulatory authorities. however, no method was presently available for its specific detection. the large similarity in amino-acid sequences of collagens from different species make their immunochemical differentiation difficult when u ...200515899572
fertility of yearling beef bulls during mating.crossbred (bos taurus) yearling beef bulls were assessed for breeding soundness and physical traits prior to multi-sire natural mating at pasture. bulls (n = 60) were assigned to six groups of nine or 10 bulls and two bull-groups were rotated on 14-day intervals during a 63-day mating season in each breeding herd (n = 3) of 191-196 cows. the remaining bulls (n = 14) were maintained under similar environmental conditions without mating exposure. bulls were observed during mating and assessed for ...200515961148
frequency of brain tissue embolism associated with captive bolt gun stunning of sheep.in accordance with controls instituted to protect the consumer from meat potentially infected with bovine spongiform encephalopathy (bse), brain tissue emboli caused by the use of captive bolt gun (cbg) stunning have been identified as a potential public health risk that requires further investigation. as the natural occurrence of bse in sheep remains uncertain we have investigated the frequency of brain tissue embolism associated with stunning by two types of cbg that are in commercial use in t ...200415992291
bov-ta short interspersed nucleotide element sequences in circulating nucleic acids from sera of cattle with bovine spongiform encephalopathy (bse) and sera of cattle exposed to bse.circulating nucleic acids (cna) are known to be enriched in repetitive dna sequences in humans. here, bovine sera cna were analyzed to determine if cell stress-related short interspersed nucleotide elements (sines) could be detected in sera from cattle associated with bovine spongiform encephalopathy (bse). nucleic acids were extracted, amplified, cloned, and sequenced from the sera of protease-resistant prion protein (prp(res))-positive cattle (n = 2) and sera from bse-cohort cows (n = 6); 150 ...200516002628
characterization and enzymatic degradation of sup35nm, a yeast prion-like protein.transmissible spongiform encephalopathies (tses) are believed to be caused by an unconventional infectious agent, the prion protein. the pathogenic and infectious form of prion protein, prpsc, is able to aggregate and form amyloid fibrils, very stable and resistant to most disinfecting processes and common proteases. under specific conditions, prpsc in bovine spongiform encephalopathy (bse) brain tissue was found degradable by a bacterial keratinase and some other proteases. since this disease-c ...200516081647
detection of metazoan species as a public health issue: simple methods for the validation of food safety and quality.species identification represents a critical issue in food chain safety and quality control. several procedures are available to detect animal proteins in cattle feed or to trace transgenic foods. the most effective approach is based on the use of dna as a marker. amplification of dna provides rapid, sensitive and specific protocols. several target genes can be used, but new insights come from the mitochondrial genome, which is naturally amplified in each cell and shows a remarkable resistance t ...200516216782
risk perception and communication: lessons for the food and food packaging industry.health risks are front-page news. be it bovine spongiform encephalitis (bse), surface ozone, or radiation from transmitter stations or mobile phones, the popular press puts out a constant stream of risk warnings and sensational reports about potential health threats. this paper examines how the general public perceives and assesses such information when it comes to food and food packaging risks. in the first part, the basic components of food risks are discussed and then compared with the percep ...200516227190
polymorphisms of the prion protein gene (prnp) in hanwoo (bos taurus coreanae) and holstein cattle.polymorphisms in the prion protein gene (prnp) in humans and sheep correlate with susceptibility to transmissible spongiform encephalopathies (tses). bovine spongiform encephalopathy (bse) has been reported in british and japanese cattle; it has occurred thus far in holstein cattle. bse in hanwoo (bos taurus coreanae) cattle has not been diagnosed up to now. to characterize the bovine prnp polymorphisms in korean cattle, we analyzed the open reading frame (orf) of prnp in 120 hanwoo (beef) cattl ...200516284424
dna polymorphisms of the prion doppel gene region in four different german cattle breeds and cows tested positive for bovine spongiform encephalopathy.polymorphisms of the prion protein gene prnp have been shown to influence the susceptibility/resistance to prion infections in human and sheep. in addition, the t174m polymorphism within the flanking prion doppel gene (prnd) was thought to be involved in susceptibility to sporadic creutzfeldt-jacob disease. to study a possible influence of dna polymorphisms of the bovine prnd gene in bovine spongiform encephalopathy (bse), previously identified and newly isolated dna polymorphisms were genotyped ...200516284804
meningoencephalitis tuberculosa in a holstein cow.the gross and histopathologic lesions of meningoencephalitis tuberculosa in a 4-year-old holstein cow showing clinical signs compatible with bovine spongiform encephalopathy are described in this report. grossly, numerous gray to yellow, firm and caseous nodules were seen on the ventral surfaces of the brain and in the lateral and fourth ventricles. histopathologically, foci of caseation and dystrophic mineralization were surrounded by multinucleated giant cells, epitheloid macrophages, plasma c ...200516301587
ovine infection with the agents of scrapie (ch1641 isolate) and bovine spongiform encephalopathy: immunochemical similarities can be resolved by immunohistochemistry.immunochemical ("rapid") tests, which recognize a partly protease-resistant conformer of the prion protein (prp(res)) are now widely used in europe for the diagnosis of transmissible spongiform encephalopathies (tses). some of these tests can be used to distinguish natural scrapie from experimental bovine spongiform encephalopathy (bse) in sheep, on the basis of migration pattern differences of prp(res) in western immunoblots. however, prp(res) from sheep inoculated with ch1641 scrapie gives an ...200616324707
bovine spongiform encephalopathy statement of possible relation with new variant creutzfeldt-jakob disease: effects on the welfare of united kingdom cattle.although measures to control bovine spongiform encephalopathy (bse) had been in force in the united kingdom for many years and had resulted in a marked decline in clinical cases, the announcement by the secretary of state for health on march 20, 1996, that a new variant form of creutzfeldt-jakob disease may be linked with exposure to bse, resulted in the introduction of several new control measures. these measures included a scheme banning human consumption of meat from cattle who were more than ...199816363943
managing the risk of transmission of variant creutzfeldt jakob disease by blood products.whereas plasma-derived clotting factor concentrates now have a very good safety record for not being infectious for lipid enveloped viruses, concern has arisen about the possibility that prion diseases might be transmitted by blood products. there is epidemiological evidence that classical sporadic creutzfeld jakob disease (cjd) is not transmitted by blood transfusion. there is now good evidence that the abnormal prion associated with variant cjd can be transmitted by transfusion of fresh blood ...200616371015
evaluation of the cumulative evidence for freedom from bse in birth cohorts.substantial resources are used for surveillance of bovine spongiform encephalopathy (bse) despite an extremely low detection rate, especially in healthy slaughtered cattle. we have developed a method based on the geometric waiting time distribution to establish and update the statistical evidence for bse-freedom for defined birth cohorts using continued surveillance data. the results suggest that currently (data included till september 2004) a birth cohort of danish cattle born after march 1999 ...200616450206
sequence variation of bovine prion protein gene in japanese cattle (holstein and japanese black).to assess relationships between nucleotide polymorphisms of the prion protein (prnp) gene and susceptibility to bovine spongiform encephalopathy (bse), we investigated polymorphisms in the open reading frame (orf) and 2 upper regions of the prnp gene from 2 japanese cattle breeds: 863 healthy holstein cattle, 6 bse-affected holstein cattle, and 186 healthy japanese black (jb) cattle. in the orf, we found single-nucleotide polymorphisms (snps) at nucleotide positions 234 and 576 and found 5 or 6 ...200616462113
inactivation of transmissible spongiform encephalopathy agents in food products by ultra high pressure-temperature treatment.bovine spongiform encephalopathy (bse) contamination of the human food chain most likely resulted from nervous system tissue in mechanically recovered meat used in the manufacture of processed meats. the availability of effective decontamination methods for products considered at risk for bse or other transmissible spongiform encephalopathies (tses) would be an attractive safeguard to human health, but neither of the two proven inactivating methods, autoclaving or exposure to strong alkali or bl ...200616542885
development of recombinant chicken igy from single chain fragment of variable region for diagnosis of bse.we generated two recombinant chicken igys, designated ab3-15 and ab4-19, against mammalian prion protein (prp) from the single chain fragment of variable region (scfv) antibodies. these two antibodies recognized prp(sc) from bovine spongiform encephalopathy (bse) in cattle and were more sensitive than the corresponding scfv antibodies. these antibodies also recognized prp(sc) from other scrapie-infected mammals. these results indicate that ab3-15 and ab4-19 are useful for diagnosis of bse as wel ...200716580230
safety evaluation for a biodiesel process using prion-contaminated animal fat as a source.due to the bovine spongiform encephalopathy (bse), specified risk material (srm) as well as animal meat and bone meal (mbm) are banned from the food and feed chain because of a possible infection with pathogenic prions (prp(sc)). nowadays, prions are widely accepted to be responsible for tse(transmissible spongiform encephalopathies)-caused illnesses like bse and scrapie, and especially for the occurrence of the new variant of cjd in humans. presently, srm and mbm are burnt under high temperatur ...200616612902
bse infection in bovine prp transgenic mice leads to hyperphosphorylation of tau-protein.we observed the changes in the central nervous system (cns) of transgenic mice expressing bovine prion protein (bo-prp) as a contribution to our knowledge of the pathogenesis of bovine spongiform encephalopathy (bse). the main result was the detection of hyperphosphorylated tau. this protein was detected for the first time, using immunohistochemical techniques, in the neurons and glial cells of mice experimentally infected with bse. the results highlighted the involvement of tau protein in the p ...200616621340
distribution of prp(sc) in cattle with bovine spongiform encephalopathy slaughtered at abattoirs in japan.three 80- to 95-month-old holstein dairy cattle infected naturally with the agent of bovine spongiform encephalopathy (bse) and slaughtered at abattoirs in japan were examined for the distribution of disease-specific and protease-resistant prion protein (prp(sc)) by immunohistochemistry (ihc) and western blot (wb) analyses. the cattle showed no clinical signs or symptoms relevant to bse but were screened as positive by enzyme-linked immunosorbent assay, a rapid test for bse. this positive result ...200616632909
an evaluation of united kingdom environmental bovine spongiform encephalopathy risk assessment.as a member of the group of diseases known as transmissible spongiform encephalopathies (tses), bovine spongiform encephalopathy (bse) has been causally associated with a new variant of creutzfeldt-jakob disease (vcjd) in humans. given the many uncertainties on the transmission and persistence of tse pathogens in the environment, quantitative assessment of risks to humans and animals continues to remain a public health issue. this paper reviews quantitative bse risk assessments undertaken in the ...200516639897
gene expression alterations in brains of mice infected with three strains of scrapie.transmissible spongiform encephalopathies (tses) or prion diseases are fatal neurodegenerative disorders which occur in humans and various animal species. examples include creutzfeldt-jakob disease (cjd) in humans, bovine spongiform encephalopathy (bse) in cattle, chronic wasting disease (cwd) in deer and elk, and scrapie in sheep, and experimental mice. to gain insights into tse pathogenesis, we made and used cdna microarrays to identify disease-associated alterations in gene expression. brain ...200616700923
a quantitative assessment of the risk of exposure to bovine spongiform encephalopathy via meat-and-bone meal in japan.the feeding of meat-and-bone meal (mbm) derived from cattle infected with bovine spongiform encephalopathy (bse) is a major source of bse infection. the risks of bse infection via mbm in japan were examined quantitatively to estimate infectivity to cattle via mbm derived from a single clinically infected animal being rendered. three routes of exposure were modeled: (i) feeding cattle concentrates containing mbm as an ingredient, (ii) feeding cattle concentrates contaminated with mbm from non-rum ...200616712985
brahman bull fertility in a north australian rangeland herd.low and variable bull fertility was identified as a constraint on reproductive rates in beef cattle grazed in an extensive, multiple sire mating regimen on mount bundey station in the darwin pastoral district of northwestern australia. erratic conception patterns were attributed to a high proportion of bulls with low breeding soundness evaluation scores (bse), a high proportion of aged bulls (40%>8 yr), and to running bulls of mixed age groups. liveweight, scrotal circumference (sc) and age were ...198916726675
breed predisposition for bse: epidemiological evidence in bavarian cattle.we present epidemiological data from bavaria that indicates that animals of the brown swiss (bs) cattle breed might be more susceptible to bse than animals from other breeds, both in terms of disease prevalence and length of the incubation period. bs animals were disproportionately represented among the bse cases (bs represented about 9% of the susceptible population but 27% of actual cases). bs were slaughtered at a higher age (5.8 years vs. 5.0 years for other breeds), and there is a higher pr ...200616739897
monitoring for bovine spongiform encephalopathy in sheep in great britain, 1998-2004.bovine spongiform encephalopathy (bse) may have been transmitted to british sheep via contaminated feed in the 1980s. strain-typing techniques based on immunohistochemical (ihc) detection of abnormal protein (prp(d)) and the molecular analysis of proteinase-resistant protein (prp(res)) by western blotting (wb) can discriminate between natural or experimental scrapie and experimental bse in sheep. between 1 january 1998 and 31 october 2001, 1247 sheep, clinically suspected of scrapie, were found ...200616760414
prp(d) accumulation in organs of arq/arq sheep experimentally infected with bse by peripheral routes.to study the pathogenesis of bovine spongiform encephalopathy infection in small ruminants, two lacaune sheep with the aa136rr154qq171 and one with the aa136rr154rr171 genotype for the prion protein, were inoculated with a brain homogenate from a french cattle bse case by peripheral routes. sheep with the arq/arq genotype are considered as susceptible to prion diseases contrary to those with the arr/arr genotype. the accumulation of disease-associated prion protein (prp(d)) was analysed by bioch ...200616770445
sequential pressurized liquid extraction to determine brain-originating fatty acids in meat products as markers in bovine spongiform encephalopathy risk assessment studies.a new approach using sequential pressurized liquid extraction described recently [j. poerschmann, r. carlson, j. chromatogr. a, 1127 (2006) 18-25] was applied to determine lipid markers originating from central nervous system (cns) tissue of cows in heat-processed sausages. these studies are very important in quality control as well as risk assessment studies in the face of the bovine spongiform encephalopathy (bse) crisis. diagnostic cns lipid markers, which should not be present in meat produc ...200616782118
variant cjd (vcjd) and bovine spongiform encephalopathy (bse): 10 and 20 years on: part 2.up until february 2006, variant cjd (vcjd), the human disease associated with transmission of bse from cattle, has been confirmed in 160 patients resident in the uk and 28 elsewhere, some of whom have never visited the uk. cases have been reported in france (16 cases), ireland (3), usa (2), canada, italy, japan, the netherlands, portugal, saudi arabia and spain (1 each). the presumed main period of hazard for ingestion of the bse agent in bovine products in the uk is 1984-89, or perhaps up to 19 ...200616823692
a country that never had a bse crisis: consensus and tensions in transforming the norwegian food system.norway is often described as a country where the safety of domestically produced food is not questioned and where there is a prevailing consensus about the division of responsibility for food safety. for this reason it was surprising to find that norwegian consumers trust the safety of their meat less than do their british counterparts. this result is particularly interesting, as norway is one of the few countries that has never experienced bse, while britain has been the country most affected b ...200616828930
increased expression of water channel aquaporin 1 and aquaporin 4 in creutzfeldt-jakob disease and in bovine spongiform encephalopathy-infected bovine-prp transgenic mice.spongiform change is a cardinal feature in transmissible spongiform encephalopathies, including creutzfeldt-jakob disease (cjd) and bovine spongiform encephalopathy (bse). it is characterized by swelling of the neuronal processes and vacuolization of the neuropil, leading to increased intraneuronal water content. the present study examines, by gel electrophoresis and western blotting, the expression levels of the water channels aquaporin 1 (aqp1) and aquaporin 4 (aqp4) in the frontal cortex (are ...200616871401
from rabies to transmissible spongiform encephalopathies: an immune-mediated microbial trigger involving molecular mimicry could be the answer.the concept of experimental allergic encephalomyelitis (eae) being linked to both rabies post-vaccination encephalomyelitis and multiple sclerosis (ms) has raised the intriguing question whether animal studies carried out for the induction and transmission of transmissible spongiform encephalopathies (tses) using brain antigens including prions do have a similar immunopathogenetic mechanism. although an essential link between autoimmunity and ms has been well established, its role in the pathoge ...200716920276
relative levels of semen platelet activating factor-receptor (pafr) and ubiquitin in yearling bulls with high content of semen white blood cells: implications for breeding soundness evaluation.high content of the platelet activating factor (paf) and its plasma membrane receptor (pafr) in semen is thought to benefit fertility in farm animals and humans. we used flow cytometric, biochemical, and immunocytochemical analysis to examine pafr levels alone (trial 1, n = 156 bulls) or in a dual assay with sperm defect marker ubiquitin (ubi; trial 2, n = 88 bulls), in semen samples from 160 yearling bulls undergoing breeding soundness evaluations (bse). in both trials, we observed increased pa ...200716928895
comparison of dna variants in the prnp and nf1 regions between bovine spongiform encephalopathy and control cattle.dna from 252 bovine spongiform encephalopathy (bse) cattle and 376 non-diseased control cattle were genotyped for nine loci in the prion protein (prnp) gene region, three loci in the neurofibromin 1 (nf1) region and four control loci on different chromosomes. the allele and genotype frequencies of the control loci were similar in bse and control cattle. in the analysed 7.4 mb prnp region, the largest differences between bse and control cattle were found for the loci reg2, r16 and r18, which are ...200616978176
bse inoculation to prion diseases-resistant sheep reveals tricky silent carriers.the possible transmission of bovine spongiform encephalopathy (bse) agent to sheep contributed to select genetically sheep considered as resistant to prion diseases i.e., with prp arr/arr genotype. here, we report the infection of two prp arr/arr genotype sheep using the cattle bse agent inoculated by peripheral routes. disease-associated prion protein (prp(d)) was detected in the brain for one case (at 2191 days post-infection (dpi)) and only in the nervous enteric system for the other one (at ...200617049491
isolation from cattle of a prion strain distinct from that causing bovine spongiform encephalopathy.to date, bovine spongiform encephalopathy (bse) and its human counterpart, variant creutzfeldt-jakob disease, have been associated with a single prion strain. this strain is characterised by a unique and remarkably stable biochemical profile of abnormal protease-resistant prion protein (prp(res)) isolated from brains of affected animals or humans. however, alternate prp(res) signatures in cattle have recently been discovered through large-scale screening. to test whether these also represent sep ...200617054396
prion gene haplotypes of u.s. cattle.bovine spongiform encephalopathy (bse) is a fatal neurological disorder characterized by abnormal deposits of a protease-resistant isoform of the prion protein. characterizing linkage disequilibrium (ld) and haplotype networks within the bovine prion gene (prnp) is important for 1) testing rare or common prnp variation for an association with bse and 2) interpreting any association of prnp alleles with bse susceptibility. the objective of this study was to identify polymorphisms and haplotypes w ...200617092337
radiological assessment of creutzfeldt-jakob disease.creutzfeldt-jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. there is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. additionally, it is of public interest to differentiate the sporadic form of the disease from the variant cjd type (vcjd), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (bse). high signal in the striatum on t2-weigh ...200717093966
a review of genetic resistance to disease in bos taurus cattle.cattle show considerable variability in their responses to a wide range of disease challenges, and much of the variability is genetic. this review highlights genetic variation in disease susceptibility in bos taurus cattle, with variation found at the breed level and also within breeds. disease challenges come from bacteria and viruses, parasites and feed-borne toxins. for an animal to survive, it needs its own mechanisms for resisting these challenges, or for being resilient to them, or it must ...200717095270
milk yield, age at first calving, and the risk of bse: an analysis at the farm level in france.the epidemic of bovine spongiform encephalopathy (bse) in france, as in the uk, has affected dairy cattle much more than beef cattle. however, the intensification of dairy herd management as a risk factor for bse has not to date been analyzed. for this purpose, two databases were merged: the french milk records database, and the french bse database, which can be considered as being devoid of notification bias since july 2001, when systematic tests were implemented. only pure holstein herds were ...200717097171
brief review on the epidemiology of transmissible spongiform encephalopathies (tse).transmissible spongiform encephalopathies (tse) form a group of human and animal diseases that share common features such as (a) distinct pathological lesions in the central nervous system, (b) transmissibility at least in experimental settings, and (c) a long incubation period. considerable differences exist in the host range of individual tses, their routes of transmission, and factors influencing the host susceptibility (such as genotype). the objective of this review was to briefly describe ...200717126962
fucosyl-gm1a, an endoglycoceramidase-resistant ganglioside of porcine brain.the use of bovine brain has been prohibited in many countries because of the world-wide prevalence of mad cow disease, and thus porcine brain is expected to be a new source for the preparation of gangliosides. here, we report the presence of a ganglioside in porcine brain which is strongly resistant to hydrolysis by endoglycoceramidase, an enzyme capable of cleaving the glycosidic linkage between oligosaccharides and ceramides of various glycosphingolipids. five major gangliosides (designated pb ...200717167042
[study on bovine spongiform encephacitis and current status of safety control of medical devices deriving from animals].to introduce the basic information about mad cow disease and the current status of safety control of medical devices derived from mammalian animal tissues.200617191585
changes in plasma metabolites and muscle glycogen are correlated to bovine spongiform encephalopathy in infected dairy cattle.during the clinical phase of bovine spongiform encephalopathy (bse), a significant decrease was observed in the ratio of muscle glycogen to plasma l-lactic acid concentrations in bse infected field case and experimentally infected dairy cattle compared with healthy control cattle (p<0.001), this being due to changes in the concentration of both metabolites in the bse infected cattle compared with the control group. furthermore, the concentration of plasma alanine was significantly increased (p<0 ...200717197001
multivariate feature selection and hierarchical classification for infrared spectroscopy: serum-based detection of bovine spongiform encephalopathy.a hierarchical scheme has been developed for detection of bovine spongiform encephalopathy (bse) in serum on the basis of its infrared spectral signature. in the first stage, binary subsets between samples originating from diseased and non-diseased cattle are defined along known covariates within the data set. random forests are then used to select spectral channels on each subset, on the basis of a multivariate measure of variable importance, the gini importance. the selected features are then ...200717237926
functional relevance of dna polymorphisms within the promoter region of the prion protein gene and their association to bse infection.transmissible spongiform encephalopathies (tses) are a group of neurodegenerative diseases that can occur spontaneously or can be caused by infection or mutations within the prion protein gene prnp. nonsynonymous dna polymorphisms within the prnp gene have been shown to influence susceptibility/resistance to infection in sheep and humans. analysis of dna polymorphisms within the core promoter region of the prnp gene in four major german bovine breeds resulted in the identification of both snps a ...200717255470
bovine spongiform encephalopathy and the safety of milk from canadian dairy cattle.the detection of bovine spongiform encephalopathy (bse) in beef cattle closed canadian beef export markets to 30 countries, including the usa, with devastating financial losses. the detection and confirmation of the fifth and seventh bse-infected animals but first infected dairy cows extended the problem of risk management to canadian dairy farmers. as the public are concerned about the safety not only of beef but also of milk and milk products that may contain disease-causing prions, this revie ...200717308017
on the question of sporadic or atypical bovine spongiform encephalopathy and creutzfeldt-jakob disease.strategies to investigate the possible existence of sporadic bovine spongiform encephalopathy (bse) require systematic testing programs to identify cases in countries considered to have little or no risk of orally acquired disease or to detect a stable occurrence of atypical cases in countries in which orally acquired disease is disappearing. to achieve 95% statistical confidence that the prevalence for sporadic bse is no greater than 1 per million (i.e., the annual incidence of sporadic creutzf ...200617326930
spongiform encephalopathy in a miniature zebu.the first case of spongiform encephalopathy in a zebu (bos indicus) was identified in a zoo in switzerland. although histopathologic and immunohistochemical analyses of the central nervous system indicated a diagnosis of bovine spongiform encephalopathy (bse), molecular typing showed some features different from those of bse in cattle (b. taurus).200617326950
identification of a proteinase k resistant protein for use as an internal positive control marker in prp western blotting.the routine use of an internal positive control (ipc) marker could prove useful in the diagnosis of transmissible spongiform encephalopathy (tse) diseases, particularly in surveillance programmes where large numbers of negative results are reported. detection of an endogenous ipc protein in a negative sample adds confidence to the correct sample processing throughout the analytical procedure and could avoid the reporting of false negative diagnoses. proteinase k (pk) resistance is one of the key ...200717336356
conversion of the base prion strain into the bse strain: the origin of bse?atypical neuropathological and molecular phenotypes of bovine spongiform encephalopathy (bse) have recently been identified in different countries. one of these phenotypes, named bovine "amyloidotic" spongiform encephalopathy (base), differs from classical bse for the occurrence of a distinct type of the disease-associated prion protein (prp), termed prp(sc), and the presence of prp amyloid plaques. here, we show that the agents responsible for bse and base possess different biological propertie ...200717352534
risk assessment and surveillance for bovine spongiform encephalopathy.the national risk of bovine spongiform encephalopathy (bse) has not been assessed by many countries, and many countries are conducting little or no bse surveillance. national measures implemented, including import restrictions, surveillance systems, and sanitary controls, should be based on actual bse risk. thus, as a first step, the national bse status must be determined, particularly through assessment of the national risk. the world organisation for animal health (oie) provides recommendation ...200617361761
bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle.the dose-response of cattle exposed to the bovine spongiform encephalopathy (bse) agent is an important component of modelling exposure risks for animals and humans and thereby, the modulation of surveillance and control strategies for bse. in two experiments calves were dosed orally with a range of amounts of a pool of brainstems from bse-affected cattle. infectivity in the pool was determined by end-point titration in mice. recipient cattle were monitored for clinical disease and, from the inc ...200717374783
lesion profiles and gliosis in the brainstem of 135 swiss cows with bovine spongiform encephalopathy (bse).lesion profiles are considered to be an important tool for the comparison of the various animal and human spongiform encephalopathies and to obtain information upon prion strain variations. histological and immunohistochemical reactions (prpsc, gfap) in 13 brain areas at 4 levels in the brainstem from 135 bse-positive and 45 bse-negative cases were retrospectively evaluated. in this retrospective study a lesion profile based on histological features was worked out on the basis of bse cases origi ...200717410969
genotype distribution of the prion protein gene (prnp) promoter polymorphisms in korean cattle.recently, an association between bovine spongiform encephalopathy (bse) and insertion/deletion (indel) polymorphisms in the bovine prion protein gene (prnp) promoter region has been reported in german cattle. these prnp polymorphisms cause changes in prnp expression and are thought to play an important role in bse susceptibility. bse has been reported in british and japanese holstein cattle but has not been diagnosed in hanwoo cattle (bos taurus coreanae) up to now. these results prompted us to ...200617426768
molecular discrimination of atypical bovine spongiform encephalopathy strains from a geographical region spanning a wide area in europe.transmissible spongiform encephalopathy strains can be differentiated by their behavior in bioassays and by molecular analyses of the disease-associated prion protein (prp) in a posttranslationally transformed conformation (prpsc). until recently, isolates from cases of bovine spongiform encephalopathy (bse) appeared to be very homogeneous. however, a limited number of atypical bse isolates have recently been identified upon analyses of the disease-associated proteinase k (pk) resistance-associa ...200717442800
atypical scrapie in a swiss goat and implications for transmissible spongiform encephalopathy surveillance.different types of transmissible spongiform encephalopathies (tses) affect sheep and goats. in addition to the classical form of scrapie, both species are susceptible to experimental infections with the bovine spongiform encephalopathy (bse) agent, and in recent years atypical scrapie cases have been reported in sheep from different european countries. atypical scrapie in sheep is characterized by distinct histopathologic lesions and molecular characteristics of the abnormal scrapie prion protei ...200717459826
reduced susceptibility to bovine spongiform encephalopathy prions in transgenic mice expressing a bovine prp with five octapeptide repeats.in this work, transgenic (tg) mice were generated expressing a bovine prion protein containing five octarepeats (boprp(5or)-tg). after intracerebral inoculation of bovine spongiform encephalopathy (bse) inoculum, these mice suffered a bse-like neuropathology but survived longer compared with homologous tg mice expressing similar levels of a six octarepeat boprp protein (boprp(6or)-tg). de novo-generated five octarepeat (5or) prp(sc) showed no biochemical differences from 6or-prp(sc), and the pro ...200717485546
imported and indigenous bse cases in germany.bovine spongiform encephalopathy (bse) is a transmissible fatal neurodegenerative disease in cattle with an average incubation time of five years. the first bse case in an indigenous cow was detected in germany in november 2000. this was almost eight years after the huge bse epidemic in the united kingdom had peaked, and several years after many eu member states had seen their first bse case. in the 1990s, bse had been diagnosed in six imported animals in germany. however, after the implementati ...200717493772
a model (bsurve) for estimating the prevalence of bovine spongiform encephalopathy in a national herd.we developed the bsurve spreadsheet model to estimate the true prevalence of bovine spongiform encephalopathy (bse) in a national cattle population, and evaluate national bse surveillance programs. bsurve uses bse surveillance data and demographic information about the national cattle population. the proportion of each cohort infected with bse is found by equating the observed number of infected animals with the number expected, following a series of probability calculations and assuming a binom ...200717507106
detection of central nervous system tissues in meat products: validation and standardization of a real-time pcr-based detection system.several phenotypic as well as genotypic methods have been published describing the detection of central nervous system (cns) tissues that are part of the bovine spongiform encephalopathy (bse) risk material in food products. however, none of these methods is able to differentiate between cns tissue of the banned ruminant species and tissues of other animal species. a quantitative and species-specific real-time rt-pcr method has been developed that enables the reliable identification of cns tissu ...200717507185
targeted surveillance to assess the presence of bse in the age risk population of cattle slaughtered in bursa, turkey: preliminary results of an immunohistochemical detection study for the 2004-2005 period.bovine spongiform encephalopathy (bse), a member of the transmissible spongiform encepahlopathies, has been a notifiable disease in turkey since 1997. in 2002, the bse status of turkey was assessed by the eu scientific steering committee as "it is likely but not confirmed". this study presents the results of a targeted surveillance study to assess the presence of bse in the age risk population of bursa, turkey. in the assessment procedure, the immunohistochemical detection of protease-resistant ...200717519575
estimating the prevalence of bse in dairy birth cohorts and predicting the incidence of bse cases in japan.following the detection of the first case of bse in japan in september 2001, four million cattle were subjected to a rapid test for bse up to the end of 2004. a further 10 cases were detected in the dairy cattle population and two cases in holstein steers. we focused on the dairy population and estimated the prevalence of bse infected animals within each birth cohort for the years 1992-2001 using bayesian inference. from this we were able to predict historic and future trends in the number of in ...200717617479
prion protein and the transmissible spongiform encephalopathies.transmissible spongiform encephalopathies (tses) are fatal neurodegenerative diseases that occur in a wide variety of mammals. in humans, tse diseases include kuru, sporadic and iatrogenic creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker syndrome (gss), and fatal familial insomnia (ffi). so far, tse diseases occur only rarely in humans; however, scrapie is a widespread problem in sheep, and the recent epidemic of bovine spongiform encephalopathy (bse or mad cow disease) has seriou ...199717708907
central nervous system tissue in meat products: an evaluation of risk, prevention strategies, and testing procedures.since the outbreak of bovine spongiform encephalopathy (bse) in the united kingdom in 1986 and its subsequent link to the human neurological disorder variant creutzfeldt-jakob disease (vcjd), presence of tissues from the central nervous system (cns) in meat products has been considered a public health concern and, thus, has been banned from entering the human food chain in many countries. despite this, potential can exist during harvesting to contaminate or cross-contaminate edible meat products ...200717900496
mechanical stress and formation of protein aggregates in neurodegenerative disorders.misfolded protein aggregates and inclusion bodies have been associated with various protein conformation disorders such as alzheimer's disease (ad), parkinson's disease (pd), amyotrophic lateral sclerosis (als), and prion diseases including bovine spongiform encephalopathy (bse). models have been proposed as plausible explanations for the extension and progression of protein aggregates; however, little is known about the initiation process of protein aggregation, particularly in sporadic neurode ...200817910993
epidemiological study of the decline of bse in italy.in this paper, data derived from the national database of the italian surveillance system for bovine spongiform encephalopathy (bse) are used to describe the italian epidemic of bse. two data flows were established to collect the results of active and passive surveillance, and 25 regional laboratories were involved. the national reference centre (cea) was in charge of the data analysis. crude and age-standardised estimates of the prevalence and incidence of bse were obtained to describe the dist ...200717938408
analyzing bse surveillance in low prevalence countries.if the prevalence of bovine spongiform encephalopathy (bse) varies among cohorts within a population, stratified analysis of bse surveillance data may allow identification of differences in bse exposure that are important with respect to the design and evaluation of disease prevention and control measures. in low bse prevalence populations, however, surveillance at levels that meet or exceed international guidelines may provide insufficient statistical power to distinguish prevalence levels amon ...200817980447
comparison of the historic recycling risk for bse in three european countries by calculating the basic reproduction ratio r0.a deterministic model of bse transmission is used to calculate the r(0) values for specific years of the bse epidemics in the united kingdom (uk), the netherlands (nl), and switzerland (ch). in all three countries, the r(0) values decreased below 1 after the introduction of a ban on feeding meat and bone meal (mbm) to ruminants around the 1990s. a variety of additional measures against bse led to further decrease of r(0) to about 0.06 in the years around 1998. the calculated r(0) values were con ...200718076489
bone regenerated via rhpdgf-bb and a deproteinized bovine bone matrix: backscattered electron microscopic element analysis.this study used backscattered electron microscopy (bse-sem) to analyze specimens in which bone was augmented both horizontally and vertically with a xenograft scaffold and recombinant human platelet-derived growth factor (rhpdgf-bb), with or without a resorbable collagen membrane. the study objective was to compare percentage weight and volume calcium-phosphorus ratios of regenerated bone and native bone and the nature of the bony contact with two different implant surfaces. examination of the n ...200718092448
short communication: allele, genotype, and haplotype data for bovine spongiform encephalopathy-resistance polymorphisms from healthy us holstein cattle.bovine spongiform encephalopathy (bse) is a neurodegenerative disease of cattle caused by abnormally folded prion proteins. two regulatory region polymorphisms in the bovine prion gene are associated with resistance to classical bse disease: a 23-bp region in the promoter that contains a binding site for the repressor protein rp58, and a 12-bp region in intron 1 that has a binding site for the transcription factor sp1. the presence of these binding sites enhances bse resistance in cattle, wherea ...200818096956
review on the epidemiology and dynamics of bse epidemics.the paper describes how the comprehensive surveillance of bovine spongiform encephalopathy (bse) and studies carried out on these data has enhanced our knowledge of the epidemiology of bse. around 7, 000 bse cases were detected through the screening of about 50 million cattle with rapid tests in europe. it confirmed that the clinical surveillance had a poor capacity to detect cases, and also showed the discrepancy of this passive surveillance efficiency between regions and production types (dair ...200818187031
simulation-based estimation of bse infection in japan.the first case of bovine spongiform encephalopathy (bse) in japan was found in september 2001. as a result, national bse surveillance systems in slaughterhouses and farms were introduced between october 2001 and april 2004. all cattle, with the exception of those under 24 months of age that die at farms, now undergo compulsory testing when they die or are slaughtered. the removal of specified risk material (srm) from all slaughtered cattle and a ban on the feeding of meat-and-bone meal to all fa ...200818192042
infectious diseases and governance of global risks through public communication and participation.in recent years a succession of health emergencies connected with the threat of new, possibly global, infectious diseases has stimulated the attention of the mass media, the scientific community, and international public opinion, setting a tough test for the institutions whose job is to manage the risks. on the basis of experience in the fields of aids, bse, sars and bird flu, this study discusses the strong and weak points of governance procedures for health risks. in particular, the paper illu ...200718209267
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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