temporal aspects of the epidemic of bovine spongiform encephalopathy in great britain: individual animal-associated risk factors for the disease.the objectives of this study were first to determine the cumulative incidence of bovine spongiform encephalopathy (bse) in the british cattle population from july 1986 to june 1997, secondly, to identify individual animal-associated risk factors that influenced the age of onset of clinical signs in confirmed bse cases, and, thirdly, to assess the effectiveness of the measures introduced to control bse during the epidemic. the analyses were based on the population of british cattle at risk, deriv ...200011083045
human prion diseases.the term 'prion diseases' refers to a group of neurodegenerative disorders thought to be caused by prions, pathogenic agents with novel modes of replication and transmission. prion diseases are characterized by long incubation periods ranging from months to years and are invariably fatal once clinical symptoms have appeared. they are also called transmissible spongiform encephalopathies (tse), on account of the predominant neuropathological change observed in the central nervous system. the most ...200011087170
spain's bse cattle embargo causes tension at european commission. 200011095270
bovine spongiform encephalopathy: an overview.bovine spongiform encephalopathy (bse), widely known as "mad cow disease," is a chronic, degenerative disease affecting the central nervous system of cattle. worldwide, there have been more than 180,000 cases since the disease was first diagnosed in 1986 in great britain. bovine spongiform encephalopathy has had a substantial impact on the livestock industry in the united kingdom. the disease has also been confirmed in native-born cattle in belgium, denmark, france, ireland, luxembourg, liechten ...200011110298
heat stability of prion rods and recombinant prion protein in water, lipid and lipid-water mixtures.prion rods, i.e. insoluble infectious aggregates of the n-terminally truncated form of the prion protein, prp 27-30, and the corresponding recombinant protein, rprp(90-231), were autoclaved in water, bovine lipid or lipid-water mixtures for 20 min at temperatures from 100 to 170 degrees c. a protocol was developed for the quantitative precipitation of small amounts of protein from large excesses of lipid. prp remaining undegraded after autoclaving was quantified by western blot and degradation f ...200111161287
[how difficult it is to prevent mad cow disease]. 200011189472
public health service recommendations for the use of vaccines manufactured with bovine-derived materials.the center for biologics evaluation and research (cber), u.s. food and drug administration (fda) learned earlier this year that some vaccines were manufactured with bovine-derived materials obtained from countries in which bovine spongiform encephalopathy (bse) or a substantial risk for bse exists. a list of these countries is published by the u.s. department of agriculture (usda). this information was of concern because cases of variant creutzfeldt-jakob disease (vcjd) have been attributed to, ...200011190118
[still a small problem with the mad cow disease? creutzfeldt-jakob disease and other prion diseases: current status].this review is based on recent published research on the bse/cjd/vcjd problem mainly from uk, germany and france. the situation in sweden seems to be fortunate for several reasons. the use of meat and bonemeal as animal fodder was forbidden in this country 13 years ago. sweden has not had any sheep with scrapie for many years. no animals with bse have so far been found in our country. the incidence of sporadic cjd in this country followed retrospectively from 1985 to 1996 and prospectively from ...200111213704
surveillance of bse.the current method used to identify suspect bse cases is based on reporting cattle displaying clinical signs compatible with bse. the reporting of such cases is dependent on the ability of farmers and veterinarians to recognise the disease symptoms and on the willingness to report such cases. furthermore, it depends on the stage of the disease, because early clinical signs of bse are not always typical. histology and immunohistochemistry are established and reliable to confirm bse in cattle, but ...200011214914
application of prionics western blotting procedure to screen for bse in cattle regularly slaughtered at swiss abattoirs.disease-specific prp (prp(sc)) is at least part of the infectious particle (prion) causing bovine spongiform encephalopathy (bse) or scrapie in sheep. digestion with protease allows a distinction between normal prp (prp(c)) and prp(sc) i.e. prp(c) is completely digested while prp(sc) is cleaved at the n-terminus leading to a fragment of reduced molecular weight (prp 27-30). detection of this fragment by western blotting has been described more than a decade ago for rodent prp. we have now optimi ...200011214922
characterization of bse and scrapie strains/isolates.following the bse epidemic in cattle and the emergence of a variant form of creutzfeldt-jakob disease in humans, the question was raised whether bse has been transmitted to small ruminants by the inadvertent feeding of infectious meat and bone meal. such infections could easily be concealed in countries where scrapie is endemic. to address this issue by immuno-chemically analyzing the prp(sc) fragments, we have developed two lines of research. firstly we have focused on the development of criter ...200011214925
[bse-cattle in germany. must we soon also count on vcjk patients?]. 200111219274
inconsistent detection of prp in extraneural tissues of cats with feline spongiform encephalopathy.feline spongiform encephalopathy (fse), a transmissible spongiform encephalopathy or prion disease of cats, first reported in great britain in 1990, is believed to result from the consumption of food contaminated by the agent of bovine spongiform encephalopathy (bse). the accumulation of prp in non-neural tissues of cats diagnosed as suffering from fse was investigated by immunohistochemistry. in the majority of the cats no disease-specific prp was detected in lymphoid tissues. small amounts of ...200111338713
[psychiatric manifestations of a new variant of creutzfeldt-jakob disease. apropos of a case].the new variant of creutzfeldt-jakob disease (nvcjd) was first described in the uk in 1996 (16). the nvcjd differs from sporadic, genetic and iatrogenic cjd. creutzfeldt-jakob disease is closely associated with an abnormal isoform prpsc of a cell-surface glycoprotein, prion protein (14). molecular analysis suggests that nvcjd is caused by the same prion strain as bovine spongiform encephalopathy (bse) (4, 10). to the end of september 2000, there have been 82 cases of nvcjd in the uk. we report t ...200111407273
scrapie strains maintain biological phenotypes on propagation in a cell line in culture.bovine spongiform encephalopathy (bse) and its human equivalent, variant creutzfeldt-jakob disease (vcjd), are caused by the same strain of infectious agent, which is similar to, but distinct from, >20 strains of their sheep scrapie homologue. a better understanding of the molecular strain determinants could be obtained from cells in monoculture than from whole animal studies where different cell targeting is commonly a strain-related feature. although a few cell types can be infected with diffe ...200111432823
bse as an organizational construction: a case study on the globalization of risk.this article examines the bse problem as an example of the 'globalization of risk'. in order to determine whether the 'globalization of risk' is a social construction depending on the context, the paper emphasizes the particular role of organizations. it makes an empirical comparison of the bse-related risk-constructions of five business associations in the german meat industry sector. the results show that the associations construct the risk in close relation to their horizons of globalization, ...200111440058
brain in human nutrition and variant creutzfeldt-jakob disease risk (vcjd): detection of brain in retail liver sausages using cholesterol and neuron specific enolase (nse) as information is available about the consumption of brain via meat products. with respect to the new variant of creutzfeldt-jakob disease (vcjd) and the presumed food-borne transmission of bovine spongiform encephalopathy (bse) to humans, a preliminary survey for brain and/or spinal cord (tissues of the central nervous system, cns) was conducted. we applied a previously developed integrated procedure using cholesterol and neuron specific enolase (nse) as markers. quantification of cholesterol h ...200111520429
mad cow disease comes to japan. 200111564996
mri of creutzfeldt-jakob disease: imaging features and recommended mri protocol.creutzfeldt-jakob disease (cjd) is a rare, progressive and invariably fatal neurodegenerative disease characterized by specific histopathological features. of the four subtypes of cjd described, the commonest is sporadic cjd (scjd). more recently, a new clinically distinct form of the disease affecting younger patients, known as variant cjd (vcjd), has been identified, and this has been causally linked to the bovine spongiform encephalopathy (bse) agent in cattle. characteristic appearances on m ...200111585394
the role of mri in the diagnosis of sporadic and variant creutzfeldt-jakob disease.creutzfeldt-jakob disease (cjd) is a rare but important fatal, dementing illness. a number of types of cjd are identified, each with distinct clinical features. characteristic mri changes have been described recently. sporadic cjd, the commonest type, is found worldwide, and causes hyperintensity of the putamen and caudate nuclei. in the recently described variant cjd, which affects younger patients and has been linked to bovine spongiform encephalopathy (bse) in cattle, a highly characteristic ...200111688725
the shifting biology of prions.transmissible spongiform encephalopathies (tses), or prion diseases, are rare fatal neurodegenerative diseases of humans and animals. although some tses, like scrapie in sheep, have been known to exist for centuries, bovine spongiform encephalopathy (bse) was recognized only 15 years ago. new variant creutzfeldt-jakob disease (nvcjd) of humans is probably caused by consumption of bse-infected materials. the nature of the infectious agent is not fully elucidated, but substantial evidence suggests ...200111690621
[still no swedish cases of mad cow disease and vcjd. continuous watchfulness is justified, especially of diffuse psychiatric symptoms]. 200111715241
immunohistochemistry of prpsc within bovine spongiform encephalopathy brain samples with graded autolysis.bovine spongiform encephalopathy (bse) is a transmissible neurodegenerative disease of cattle. clinical diagnosis can be confirmed by investigation of both spongiform changes and abnormal prion protein (prpsc), a marker considered specific for the disease. tissue autolysis, often unavoidable in routine field cases, is not compatible with histological examination of the brain even though prpsc is still detectable by immunoblotting. to determine how autolysis might affect accurate diagnosis using ...200111724899
prion strain causing bovine spongiform encephalopathy (bse) in cattle. 200211807378
a sensitive elisa for the detection of bovine crude heparin in porcine heparin.heparin is an effective anticoagulant drug which has been purified for decades from bovine or porcine tissues. however, with the emergence of bse, heparin purification is today restricted to porcine intestinal mucosa. to control the origin of crude heparins, polyclonal antibodies were raised against bovine contaminants. these antibodies were used to develop one sandwich and two competitive indirect elisas. optimal results were obtained with competitive indirect elisa, using bovine crude heparin ...200111816801
validation of a luminescence immunoassay for the detection of prp(sc) in brain homogenate.a luminescence immunoassay (lia) was developed for the diagnosis of bovine spongiform encephalopathy (bse) in brain tissue using two different monoclonal antibodies for capture and detection of the protease-resistant fragment of the pathological prion protein (prp27-30). prp27-30 currently represents the most reliable marker for the infectious particle (denominated prion) causing transmissible spongiform encephalopathies (tses). internal and official validation studies of this assay are describe ...200211849686
the transmission dynamics of bse and vcjd.the bovine spongiform encephalopathy (bse) epidemic in cattle has had a huge economic impact on the agricultural industries across europe. furthermore, scientific evidence now strongly supporting a link between a new variant of creutzfeldt-jakob disease (vcjd) and consumption of bse-infected animals has further heightened the need both to understand the transmission of these new diseases and to improve control measures to protect public health. in this paper we review work undertaken by our grou ...200211862621
[prions, epidemic of creutzfeldt-jakob variant disease and global emergency].we present here the current understanding of "prion" theory and global risk for epidemics of variant creutzfeldt-jakob disease (vcjd). prion is the infectious agent of all transmissible spongiform encephalopaties (tses). it is regarded as an aggregate of a pathological conformer (prpsc) of a normal cellular glycoprotein (prpc) encoded by a gene, in humans on chromosome 20. the differences between prpsc and prpc are largely if not exclusively conformational; prpc is mostly alpha-helical while prp ...200111873615
[the prion hypothesis and the human prion diseases].our understanding of the pathogenesis of the transmissible spongiform encephalopathies (tse) has made terrific headway over the past 40 years and some scientists are even of the opinion that this group of diseases belongs to the neurodegenerative syndromes best understood. on the other hand, the investigation of tse has led to a multitude of unexpected and surprising results and consequently has initiated impassioned discussions among scientists. although the human forms of tse are very rare, th ...200211938572
bovine spongiform encephalopathy: are the cows mad or full of carbohydrates?the non-forage feeding of dairy cows rich in fast absorption carbohydrates, the low value of their euglycemic-hyperinsulinemic clamp suggest a dysregulation of carbohydrate metabolism able to produce neurodegenerative disorders. comparisons between alzheimer's disease developed in diabetes mellitus and bovine spongiform encephalopathy (bse) direct the discussion of the origin of bse not only towards a contamination by prion proteins.200211939480
[creutzfeldt-jakob disease: diagnosis, incidence, prevention and treatment].creutzfeldt-jakob disease (cjd) is a rare, neurodegenerative disorder belonging to the spongiform encephalopathies. a variant form (vcjd) is most likely the result of infection with the agent that causes bovine spongiform encephalopathy (bse). diagnostic information can be obtained by eeg, testing cerebrospinal fluid for the presence of the 14-3-3 protein, mri, brain biopsy, tonsil biopsy, and postmortem brain examination. some tests, such as mri and postmortem brain examination, can be used to ...200211998351
[what does one really know about "mad cow" disease"?]. 200112014331
bse: a consequence of cattle feeding with glycated molecules host-unknown?although there is much evidence supporting a prion contribution in the pathogenesis of transmissible spongiform encephalopathies, a novel point of view as to the induction of the diseases can be proposed. it is hypothesized that neurodegenerative diseases, such as scrapie in sheep and goats and bovine spongiform encephalopathy in cattle (bse), originate from the consumption of glycated proteins contained in their feed. these components are obtained during a high-temperature glycation process.200212027519
prion diseases: epidemiology in man.prion disease in man was first described as creutzfeldt-jacob disease (cjd) in the 1920s. cjd may have three different origins: sporadic, familial, due to mutations in the prion gene, or infectious, due to iatrogenic exposure to infectious brain material. as an example of the latter, kuru, in papua new guinea, was a variant of cjd transmitted by cannibalism. between 1957 and 1982 more than 2500 died of kuru. sporadic cjd is the most common form of cjd and occurs with an incidence of around one p ...200212064251
brain and buffy coat transmission of bovine spongiform encephalopathy to the primate microcebus murinus.more than 100 cases of variant cjd resulting from infections with bovine spongiform encephalopathy (bse) have accumulated in the united kingdom since 1995. concern about the possibility of secondary transmissions via blood and blood components donated by infected individuals has prompted a variety of international donor deferral policies that will continue until laboratory and epidemiologic evidence provides a consensus about potential risk.200212084158
molecular advances in understanding inherited prion diseases.the prion diseases are neurodegenerative disorders that have attracted great interest because of the possible link between bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease (ctd) in humans. possible transmission of these diseases has been linked to a single protein termed the prion protein. this protein is an abnormal isoform of a normal synaptic glycoprotein. the majority of prion diseases does not appear to be caused by transmission of an infectious agent but occur s ...200212109876
postexposure prophylaxis against prion disease with a stimulator of innate immunity.the absence of an immune response to prions--the infectious agents of scrapie, bovine spongiform encephalopathy (bse), and creutzfeldt-jakob disease--might be related to the fact that these agents do not contain nucleic acids. we aimed to use cpg oligodeoxynucleotides, which have been shown to stimulate innate immunity, as a form of postexposure prophylaxis in mice. we inoculated healthy mice with brain homogenates from mice infected with the rml scrapie prion, and then injected cpg oligodeoxynu ...200212133662
targeted surveillance to assess the prevalence of bse in high-risk populations in western france and the associated risk factors.a pilot study was set up for the first time in france in august 2000, to obtain more precise estimates on the bse epidemic in france. three categories of cattle at risk of bse (found dead on-farm, euthanased and emergency slaughtered) were sampled exhaustively from august 7 to december 22, 2000, in the three regions assumed to be the most affected with bse in france (basse-normandie, bretagne and pays de la loire). the samples were checked by using prionics tests, and positive samples were confi ...200212166421
plasma metabolites indicate energy metabolism disruption during the preclinical phase of bovine spongiform encephalopathy infection.during the preclinical phase of bovine spongiform encephalopathy (bse), significantly increased concentrations of lactic acid were measured in the blood of infected dairy cows. other plasma metabolites, including alanine, leucine, serine, and glutamic acid, also showed significantly altered concentrations in the preclinical bse animals compared to a control group. this appears consistent with the exaggerated stress response observed in clinical bse, and precedes the development of clinical signs ...200212204641
breeding soundness evaluations of 3,648 yearling beef bulls using the 1993 society for theriogenology guidelines.our objective was to perform a retrospective analysis of breeding soundness evaluations (bses) as classified by the 1993 society for theriogenology (sft) guidelines [chenoweth et al., guidelines for using the bull breeding soundness evaluation form, in: theriogenology handbook, 1993, pp. b-10]. data included bse information obtained from five performance-testing stations in south carolina (sc1, sc2, sc3) and tennessee (tn1, tn2) from 1986 through 1999 on 3648 angus, brangus, charolais, chianina, ...200212212894
analysis of the prion protein in primates reveals a new polymorphism in codon 226 (y226f).bovine spongiform encephalopathy has been epizootic in cows for the last two decades, and most probably causes variant creutzfeldt-jakob disease in humans. a thorough understanding of prion pathogenesis relies on suitable animal models. modeling the transmission of bse to primates is a crucial public health priority, necessary for determining the tissue distribution of the agent and for devising therapies. susceptibility of humans to bse is partly determined by polymorphism within the gene encod ...200212222676
[consequences of bse/tse for the clinical diagnostic in cattle and sheep].the first case of bovine spongiform encephalopathy (bse) in germany induced a profound irritation not only of the consumers but also of the farmers and the veterinarians in germany. the following bse-crisis accelerated the structural changes in beef and dairy industries. the analysis of the detected bse-cases of the last years in germany and switzerland shows that the sensitivity of bse-tests is much higher in clinically preselected bse-suspected cases compared to bse-tests in normal slaughter c ...200212224461
[disposal of animal byproducts, dead and slaughtered animals].the current state of epidemiological knowledge about bse clearly indicates that certain practices in carcass rendering had a significant impact on maintenance and spreading of bse in the cattle population. this awareness did not come up spontaneously. as a reflection community legislation continuously developed and still does. the decisive move was done in the year 2000 by eliminating ruminant tissues with a high infectious potential with regard to bse (specified risk material--srm) from the hum ...200212224464
nursing patients with variant creutzfeldt-jakob disease at home.variant creutzfeldt-jakob disease (vcjd) is a rare variant of a rare neurodegenerative disease, with a rapid and fatal course. the emergence of vcjd in humans in 1996 is believed to have resulted from the consumption of bovine spongiform encephalopathy (bse)-infected meat. by july 2002, the number of vcjd cases in the uk had increased to over 120 and it is not yet known how many more people will be affected. the majority of affected individuals are cared for within their own homes with the suppo ...200212362140
factors governing speed of societal responses to threats: precision of definition of the threat may be more important than its anticipated likely severity.responses to two recent international public health threats, aids and bse, both exemplify missed opportunities for early intervention. they are in contrast to the response to the y2k computer issue, a threat of lesser-anticipated consequence but one that was more precisely defined. lessons from these experiences should inform the public health response to new threats, such as the emergence of multiple drug resistance in human pathogens.200212376075
measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice.there is increasing concern over the extent to which bovine spongiform encephalopathy (bse) prions have been transmitted to humans, as a result of the rising number of variant creutzfeldt-jakob disease (vcjd) cases. toward preventing new transmissions, diagnostic tests for prions in livestock have been developed using the conformation-dependent immunoassay (cdi), which simultaneously measures specific antibody binding to denatured and native forms of the prion protein (prp). we employed high-aff ...200212389035
variant creutzfeldt-jakob disease: an unfolding epidemic of misfolded proteins.variant creutzfeldt-jakob disease (vcjd) is an emerging infectious disease believed to be the human manifestation of bovine spongiform encephalopathy (bse). variant cjd belongs to a family of human and animal diseases called transmissible spongiform encephalopathies (tse). the pathogenesis of tse is not fully understood, but a modified form of a normal cellular protein plays a central role. current measures to control vcjd aim to prevent transmission of the infectious agent from animals to human ...200212410862
geographical clustering of cases of bovine spongiform encephalopathy (bse) born in switzerland after the feed ban.over one-third of the cases of bse in switzerland have been born after the feed ban of december 1, 1990. evidence for the geographical clustering of these cases emerged in two distinct regions. all the 354 bse cases recorded until june 30, 2000 (set a), and the 376 cases recorded up to may 14, 2001 (set b), were georeferenced to the centres of the communities in which the herds of origin were located, and control populations were georeferenced to the centres of the communities in which these her ...200212418529
subclinical scrapie infection in a resistant species: persistence, replication, and adaptation of infectivity during four passages.cross-species infection with transmissible spongiform encephalopathy agents may lead to subclinical infection and to adaptation of the infection to new species. this is of particular concern for the millions of people possibly exposed to bovine spongiform encephalopathy (bse) by consumption of bse-infected beef. subclinical infection was studied by making 4 serial passages of hamster scrapie agent (263k) in mice. at each step, infectivity was followed by inoculation of hamsters and mice. subclin ...200212424693
the transmissible spongiform encephalopathies: disease risks for north america.transmissible spongiform encephalopathies exotic to north america (bse and associated diseases) are unlikely to be introduced or to persist should they be introduced into the united states [2]. domestic tses (scrapie, cwd, and tme) seem to be relatively restricted in their host range, and none of these diseases is known to naturally cause disease in cattle. it is important that surveillance for tses continues, however, particularly in cattle because of the extreme consequences to the livestock i ...200212442578
bse in cattle born after july 31, 1996, in great britain. 200212448497
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 ...200212449682
uk dietary exposure to bse in head meat: by birth cohort and dietary exposure in 1980-1996 to the bovine spongiform encephalopathy (bse) infectious agent through the consumption of beef mechanically recovered meat (mrm) contained in burgers, sausages and other meat products has already been quantified by birth cohort (born pre-1940, 1940-1969 or post-1969) and gender. in this paper, similar quantification is undertaken for the consumption of bovine head meat.200212501957
[transmissible spongiform encephalopathies in humans].transmissible spongiform encephalopathies (tse) are dementing diseases and have been known to affect humans for over 90 years. the most common of these is the sporadic form of creutzfeldt-jakob disease (scjd), followed by its familial (fcjd) and an iatrogenic (icjd) form. 1996 a variant of cjd (vcjd) has been described in the uk, of which so far 131 cases have been observed worldwide. specific biochemical and neuropathological signatures allow to distinguish between vcjd and scjd and lead to the ...200212585209
[risk assessment as an indicator for the distribution of bse in the world].for a long time, bse was considered a problem of the uk exclusively. even after the detection of bse cases in countries outside the uk, the risk of having bse was categorically denied by many other countries. only after the introduction of active surveillance did several "bse free" countries detect bse in their country. however, before the detection of the first cases in several of these countries, a risk assessment (gbr) conducted by the scientific steering committee of the eu showed that a ris ...200212585212
bse : the european regulatory context.the bovine spongiform encephalopathy crisis provoked a fundamental re-appraisal of the way in which the european community approaches matters of food safety. between 28 july 1989, when restrictions on the dispatch of certain live cattle from the uk starte200012631966
autonomous nervous system with respect to dressing of cattle carcasses and its probable role in transfer of prp(res) molecules.pathogen prions are widely recognized as the causative agent in bovine spongiform encephalopathy (bse) and other transmissible spongiform encephalopathies. however, more research on the possible transmission mutes of this agent once it has reached the host is needed. there is evidence based on the anatomy and physiology of the autonomous nervous system (ans), as well as observations for different animal species, that the ans might be involved in the axonal drainage of pathogen prions toward the ...200312747703
sequence variation in the bovine and ovine prnp genes.a resequencing approach was adopted to identify sequence variants in the prnp gene that may affect susceptibility or resistance to bovine spongiform encephalopathy. the entire prnp gene (>21 kb) was sequenced from 26 chromosomes from a group of holstein-friesian cows, as well as exon 3 of prnp (>4 kb) from a further 24 chromosomes from six diverse breeds. we identified 51 variant sequences of which 42 were single nucleotide polymorphisms and nine were insertion/deletion (indel) events. the study ...200312755818
risk management of the transmissible spongiform encephalopathies in north north american free trade agreement partners, canada, the united states of america (usa) and mexico apply independent but harmonised transmissible spongiform encephalopathy (tse) risk management strategies in observance of office international des epizooties guidelines. the divergence between bovine spongiform encephalopathy (bse) risk management approaches in north american and europe reflects comparatively reduced external and internal bse risks in north america. the external quarantine and ...200312793780
risk management of transmissible spongiform encephalopathies in asia.a questionnaire-based survey was distributed to the office international des epizooties (oie: world organisation for animal health) member countries in asia to assess the use of risk management for transmissible spongiform encephalopathies. the author presents a summary of 16 responses received in july 2002. the survey revealed that import risk analysis on bovine spongiform encephalopathy (bse) is not routinely carried out in ten countries, indicating an urgent need for further training courses. ...200312793782
the potential for transmissible spongiform encephalopathies in non-ruminant livestock and fish.pigs and poultry in the united kingdom have undeniably been exposed to the bovine spongiform encephalopathy (bse) agent. they consumed the same ruminant protein that gave rise to the bse epidemic in cattle, but there has been no evidence of an epidemic in these species. experimental investigations have shown pigs to be susceptible to infection by multiple parenteral challenge, but resistant to oral exposure with bse-infected cattle brain. current but incomplete evidence suggests that they are al ...200312793786
transmissible encephalopathies: speculations and realities.virtually all transmissible encephalopathies (tses), such as scrapie, cjd, and bse, are caused by a type of infectious particle that remains enigmatic. the language of prion theory supersedes the reality of what is, and what is not known. this review questions the predictive value, consistency and accuracy of this now dominant assumption. many people believe the normal cellular prion protein (prp) self-converts into an infectious amyloid protein or prion. although the amyloidogenic capacity of p ...200312828865
analysis of polymorphic microsatellites within the bovine and ovine prion protein (prnp) genes.twenty-four microsatellite sites with at least three repeats were found in the bovine prion protein gene (prnp) and 23 in the ovine prnp gene. eight microsatellite sites were polymorphic in cattle and six in sheep with up to 10 alleles per site. in many cases allelic dna fragments had variants in microsatellite sites and in flanking regions. distances between microsatellite sites in eight genes from cattle and sheep occurred on average every 0.9 kb. the numerous polymorphic microsatellite sites ...200312873216
bovine spongiform encephalopathy in israel: implications for human health.only one case of a cow infected with bovine spongiform encephalopathy has been reported in israel. its publication, in 2002, caused both public and professional concern. the inevitable health policy question raised was whether or not to recommend against consuming beef and what public health measures should be taken. in this article we describe the prion diseases among animals and humans, their interaction and the precautionary procedures that were carried out by the state veterinary services an ...200314509159
bovine spongiform encephalopathy (bse) and its epidemiology.since the recognition of bse in 1986, over 180,000 cattle in the uk have developed the disease and 1-3 million are likely to have been infected with the bse agent, most of which were slaughtered for human consumption before developing signs of the disease. the origin of the first case of bse is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the bse agent, to other cattle in feed. control measures have resulted in the con ...200314522859
impact of bse on livestock production system.the small number of bse cases diagnosed in italy from january 2001 to 12 september 2001 (a total of 28, one every 9000 head) does not allow for a statistical analysis of the relationship between this disease and the livestock systems. however, some indications can be noted: (a) only dairy cattle, which represent three-quarters of the cattle raised in italy, are involved; (b) 58% of the cases belong to medium-large farms that breed 27% of all head; (c) 13 out of 28 cases are 5-year-old animals an ...200314535367
epidemiological implications of the susceptibility to bse of putatively resistant sheep.the experimental infection of sheep with bovine spongiform encephalopathy (bse) by the oral route and the likelihood that sheep were fed bse-infected meat and bone meal has led to extensive speculation as to whether or not sheep are naturally infected with bse. in response, the uk government has initiated the national scrapie plan (nsp), an ambitious pound 120 million per year project to create a bse- and scrapie-resistant national sheep flock, by selectively breeding for a genotype of sheep bel ...200314645932
quantifying bse control by calculating the basic reproduction ratio r0 for the infection among cattle.the safety of using meat and bone meal (mbm) in mammal feed was studied in view of bse, by quantifying the risk of bse transmission through different infection routes. this risk is embodied in the basic reproduction ratio r(0) of the infection, i.e. the average number of new infections induced by one initial infection. only when r(0) is below 1, will the disease die out with certainty and the population will become free from bse. unfortunately this is a slow process due to the slow progression o ...200414685769
proteinase k enhanced immunoreactivity of the prion protein-specific monoclonal antibody, we report the development and further characterisation of a novel prp-specific monoclonal antibody: 2a11. by western blot analysis, 2a11 reacts with prpc from a variety of species including cow, sheep, pig, hamster, rabbit, cat, dog, deer and mouse but fails to react with human, chicken and turtle prp. reactivity to prpc in western blot was found to be dependent on the redox state of the protein since binding of mab 2a11 to its epitope was more effective in reducing conditions. 2a11 bindin ...200414687883
bovine spongiform encephalopathy in a dairy cow--washington state, 2003.on december 23, 2003, the u.s. department of agriculture (usda) made a preliminary diagnosis of bovine spongiform encephalopathy (bse) in a single "downer" (i.e., nonambulatory disabled) dairy cow in washington state. on december 25, this diagnosis was confirmed by the bse international reference laboratory in weybridge, england. this report summarizes the findings of the initial investigation of this case and describes the public health prevention measures adopted by usda to protect the human f ...200414712176
[safety information project on drug, food and chemicals. division of safety information on drug, food and chemicals. national institute of health sciences].recent issues on bse(bovine spongiform encephalopathy) and health hazards caused by adverse reactions of medical drugs, have strongly emphasized the necessity for safety measures to secure public health. these issues have been attributed to the delay to obtain overseas information on safety and regulation, and the lack of an adequate system for acquirement and assessment of such information. in order to develop a system where domestic and international safety information is collected, analyzed, ...200314740398
[lessons from prion disease, mad cow disease, and iatrogenic creutzfeldt-jakob disease]. 200314743768
descriptive spatial analysis of bse in western france.the spatial heterogeneity of bovine spongiform encephalopathy (bse) was analysed on the 84 cases confirmed in western france (wf) between august and december 2000, when both the mandatory reporting system and an active surveillance on cattle at risk were running. ninety-four percent of these cases were born between june 1993 and june 1996, and we analysed the location at birth. one disease mapping and two clustering methods (scan of kulldorff and the method of besag and newell) were used. in ord ...200314746770
[comments on present-day spread and epidemiology of bse and prion diseases].prion diseases of animals and man are neurological diseases with amyloidal deposition of the respective proteins. as to prion disease, the cellular prion protein is in its abnormal isoform(s) an essential component of prion protein aggregates found in affected tissue. in contrast to all neurodegenerative diseases like morbus alzheimer or huntington's disease, prion diseases are transmissible. therefore, prion diseases were designated transmissible spongiform encephalopathies (tse). the diseases ...200414770333
tissue distribution of bovine spongiform encephalopathy agent in primates after intravenous or oral infection.the disease-associated form of prion protein (prp(res)) has been noted in lymphoreticular tissues in patients with variant creutzfeldt-jakob disease (vcjd). thus, the disease could be transmitted iatrogenically by surgery or use of blood products. we aimed to assess transmissibility of the bovine spongiform encephalopathy (bse) agent to primates by the intravenous route and study its tissue distribution compared with infection by the oral route.200414962521
copper binding in the prion protein.a conformational change of the prion protein is responsible for a class of neurodegenerative diseases called the transmissible spongiform encephalopathies that include mad cow disease and the human afflictions kuru and creutzfeldt-jakob disease. despite the attention given to these diseases, the normal function of the prion protein in healthy tissue is unknown. research over the past few years, however, demonstrates that the prion protein is a copper binding protein with high selectivity for cu( ...200414967054
identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic creutzfeldt-jakob disease.transmissible spongiform encephalopathies (tses), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (prp(sc)) of the host-encoded cellular prion protein (prp(c)). human and animal tse agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant prp(sc) fragments and the degree of glycosylation. ...200414970340
hypothesis of interference to superinfection between bovine spastic paresis and bovine spongiform encephalopathy; suggestions for experimentation, theoretical and practical interest.sub-acute transmissible spongiform encephalopathies (tses) or prion diseases are diseases of little known etiology. the origin of these diseases would appear to be an abnormal protease-resistant prion protein (prp(res)) which would be infectious by directly inducing its defective conformation to the normal native protein (prp(c)). this hypothesis does not account for certain aspects of tses, such as interference to superinfection: in laboratory animals, inoculation by means of an attenuated stra ...200414975501
comparative molecular analysis of the abnormal prion protein in field scrapie cases and experimental bovine spongiform encephalopathy in sheep by use of western blotting and immunohistochemical methods.since the appearance of bovine spongiform encephalopathy (bse) in cattle and its linkage with the human variant of creutzfeldt-jakob disease, the possible spread of this agent to sheep flocks has been of concern as a potential new source of contamination. molecular analysis of the protease cleavage of the abnormal prion protein (prp), by western blotting (prp(res)) or by immunohistochemical methods (prp(d)), has shown some potential to distinguish bse and scrapie in sheep. using a newly develope ...200415016886
trust in food in the age of mad cow disease: a comparative study of consumers' evaluation of food safety in belgium, britain and do food scandals like dioxines in food in belgium (summer 1999) and the detection of mad cow disease (bse) in britain affect consumers' confidence in food safety? in this paper, based on three thousand telephone interviews during the last quarter of 1999, consumers in belgium and britain are compared with consumers in norway, where there has been no such serious food scandal in recent years. 'trust' is a diffuse and complex concept to measure. in this article a consumer trust typology is dev ...200415036780
evaluation of rapid tests for the diagnosis of transmissible spongiform encephalopathies in sheep and accordance with eu regulation 999/2001, rapid tests already adopted for bovine spongiform encephalopathy (bse; prionics check western, platelia-bse and enfer tse) are to be applied in all european countries to a sub-population of over 18-month-old slaughtered or dead sheep and goats to improve scrapie surveillance and to determine the possible presence of bse in sheep; however, the three tests have thus far been evaluated only for bse and no official data are available about their performance ...200415067554
do bovine lymphocytes express a peculiar prion protein?the cellular prion protein (prpc) is a glycolipid-anchored cell surface protein that usually exhibits three glycosylation states. its post-translationally modified isoform, prpsc, is involved in the pathogenesis of various transmissible spongiform encephalopathies (tses). in bovine species, bse infectivity appears to be restricted to the central nervous system; few or no detectable infectivity is found in lymphoid tissues in contrast to scrapie or variant cjd. since expression of prpc is a prere ...200215144021
identification of a new prion strain in cattle--is there more than one form of bse? 200415181930
bovine spongiform encephalopathy and creutzfeldt-jakob disease: facts and uncertainties underlying the causal link between animal and human diseases.following an outbreak of bovine spongiform encephalopathy (bse) in dairy cows in the united kingdom (uk), 153 definite and probable human cases of new variant creutzfeldt-jakob disease (nvcjd) have been reported, almost exclusively in the uk. although exposure to the bse agent is the most plausible interpretation for the occurrence of nvcjd, the causal link between the bse prion and nvcjd is still debated. this review discusses the pros and cons of nvcjd as a separate nosographic entity, the sci ...200415300459
impaired motor coordination on static rods in bse-infected mice.scrapie and bovine spongiform encephalopathy (bse) are both progressive neurodegenerative diseases that are transmissible to mice. the onset of clinical symptoms is more subtle and variable in murine bse than in murine scrapie. assessment of behavioural changes that occur throughout disease would aid early diagnosis of disease so that more consistent end points could be made and potential therapies could be investigated. c57bl/6j mice inoculated via the intraperitoneal route with 301c bse or con ...200415302135
is mad cow disease caused by a bacteria?transmissible spongioform enchephalopathies (tse's), include bovine spongiform encephalopathy (also called bse or "mad cow disease"), creutzfeldt-jakob disease (cjd) in humans, and scrapie in sheep. they remain a mystery, their cause hotly debated. but between 1994 and 1996, 12 people in england came down with cjd, the human form of mad cow, and all had eaten beef from suspect cows. current mad cow diagnosis lies solely in the detection of late appearing "prions", an acronym for hypothesized, ge ...200415325025
the role of prp in health and disease.transmissible spongiform encephalopathies (tses) such as scrapie in sheep, bovine spongiform encephalopathy (bse) in cattle or creutzfeldt-jacob disease (cjd) and gerstmann-sträussler-scheinker syndrome (gss) in humans, are caused by an infectious agent designated prion. the "protein only" hypothesis states that the prion consists partly or entirely of a conformational isoform of the normal host protein prpc and that the abnormal conformer, when introduced into the organism, causes the conversio ...200415354865
genetic studies in relation to kuru: an overview.kuru is a subacute neurodegenerative disease presenting with limb ataxia, dysarthria, and a shivering tremor. the disease progress to complete motor and mental incapacity and death within 6 to 24 months. neuropathologically, a typical pattern of neuronal loss, astrocytic and microglial proliferation, characteristic "kuru-type" amyloid plaques, and prp deposits in the cerebral cortex and cerebellum are observed. kuru is the prototype of a group of human transmissible spongiform encephalopathies ( ...200415354868
classification of signatures of bovine spongiform encephalopathy in serum using infrared spectroscopy.signatures of bovine spongiform encephalopathy (bse) have been identified in serum by means of "diagnostic pattern recognition (dpr)". for dpr-analysis, mid-infrared spectroscopy of dried films of 641 serum samples was performed using disposable silicon sample carriers and a semi-automated dpr research system operating at room temperature. the combination of four mathematical classification approaches (principal component analysis plus linear discriminant analysis, robust linear discriminant ana ...200415457319
analysis of 2000 consecutive uk tonsillectomy specimens for disease-related prion protein.variant creutzfeldt-jakob disease (cjd) is thought to be caused by dietary or other exposure to bovine spongiform encephalopathy (bse) prions. the prevalence of preclinical or subclinical prion infection in the uk is currently unknown. since clinical variant cjd is uniformly associated with tonsillar prion infection, we screened 2000 anonymous surgical tonsillectomy specimens for disease-associated prion protein. analysis by both high sensitivity immunoblotting and immunohistochemistry detected ...200415464187
the significance of using pooled human serum in human articular cartilage tissue engineering.animal serum is commonly used in chondrocytes culture expansion to promote cell proliferation and shorten the time lag before new tissue reconstruction is possible. however, animal serum is not suitable for regeneration of clinical tissue because it has potential risk of viral and prion related disease transmission particularly mad cow disease and foreign protein contamination that can stimulate immune reaction leading to graft rejection. in this context, human serum as homologous supplement has ...200415468795
prion protein gene (prnp) variants and evidence for strong purifying selection in functionally important regions of bovine exon 3.amino acid replacements encoded by the prion protein gene (prnp) have been associated with transmissible and hereditary spongiform encephalopathies in mammalian species. however, an association between bovine spongiform encephalopathy (bse) and bovine prnp exon 3 has not been detected. moreover, little is currently known regarding the mechanisms of evolution influencing the bovine prnp gene. therefore, in this study we evaluated the patterns of nucleotide variation associated with prnp exon 3 fo ...200415477588
comparative prnp genotyping of u.s. cattle sires for potential association with bse.the recent discovery of significant associations between bovine spongiform encephalopathy (bse) susceptibility in german cattle and the frequency distributions of insertion/deletion (indel) polymorphisms within the bovine prnp gene prompted an evaluation of 132 commercial u.s. artificial insemination (ai) sires from 39 breeds. forward primer sequences from published primer sets targeting indels within the putative bovine prnp promoter, intron 1, and the 3' utr (untranslated region) were synthesi ...200415520885
the first canadian indigenous case of bovine spongiform encephalopathy (bse) has molecular characteristics for prion protein that are similar to those of bse in the united kingdom but differ from those of chronic wasting disease in captive elk and deer.brain tissue from a case of bovine spongiform encephalopathy (bse) from alberta was subjected to a western immunoblotting technique to ascertain the molecular profile of any disease-specific, abnormal prion protein, that is, prion protein that is protease-resistant (prp(res)). this technique can discriminate between isolates from bse, ovine scrapie, and sheep experimentally infected with bse. isolates of brain tissue from the bse case in alberta, 3 farmed elk with chronic wasting disease (cwd) f ...200415532881
prevalence of bse in cattle found dead, euthanased or emergency slaughtered on farms in western france in 2000, 2001 and 2002.the overall trend and the trend within birth cohorts of the prevalence of bovine spongiform encephalopathy (bse) in cattle found dead, euthanased or emergency slaughtered on farms in the bretagne, basse normandie and pays de la loire regions of france, during the periods from august 7 to december 22 in 2000, 2001 and 2002, were analysed by non-conditional logistic regression, adjusted for the region and for the type of animals. the overall prevalence of bse during these three periods decreased f ...200415537142
distribution of prp(res) in the brains of bse-affected cows detected by active surveillance in catalonia, spain. 200415551927
estimation of the age-dependent risk of infection to bse of dairy cattle in great important aspect of the bovine spongiform encephalopathy (bse) epidemic has been an apparent age-dependent risk of infection, with younger cattle being more likely to become infected than older cattle. our objective was to determine the age-dependent risk of infection of dairy cattle. we first reviewed unpublished data on the feeding patterns of proprietary concentrates for dairy-replacement cattle. these data showed that autumn- and spring-born cattle would receive different feeding patterns ...200415579333
thinking the unthinkable: alzheimer's, creutzfeldt-jakob and mad cow disease: the age-related reemergence of virulent, foodborne, bovine tuberculosis or losing your mind for the sake of a shake or burger.the possibility of the age-related reemergence of foodborne mycobacterium bovis (bovine tuberculosis) as a vector for creutzfeldt-jakob disease (cjd or human mad cow disease) and mad cow disease itself is real. the cdc reported last may of an outbreak of cjd linked to the consumption of meat contaminated "with the agent causing" bovine spongiform encephalopathy (bse) in a new jersey racetrack between the time frame 1995-2004. in the opinion of experts, ample justification exists for considering ...200515694685
phenotype of disease-associated prp accumulation in the brain of bovine spongiform encephalopathy experimentally infected view of the established link between bovine spongiform encephalopathy (bse) and variant creutzfeldt-jakob disease and of the susceptibility of sheep to experimental bse, the detection of potential cases of naturally occurring bse in sheep has become of great importance. in this study, the immunohistochemical (ihc) phenotype of disease-associated prion protein (prp(d)) accumulation has been determined in the brain of 64 sheep, of various breeds and prp genotypes, that had developed neurologica ...200515722546
testing the possibility to protect bovine prpc transgenic swiss mice against bovine prpsc infection by dna vaccination using recombinant plasmid vectors harboring and expressing the complete or partial cdna sequences of bovine prpc.the objective of this study was to investigate the molecular mechanisms of neurobiological processes involved in the degeneration of the central nervous system. the bovine spongiform encephalopathy (bse) was used as experimental model system for investigation of transmissible spongiform encephalopathy (tse). the experimental strategy was to evaluate the possibility for protection of bovine prp(c) transgenic mice against a bovine prp(sc) infection by dna vaccination using the complete or partial ...200515744583
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