| 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 ... | 2001 | 11816801 |
| 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 ... | 2002 | 11849686 |
| 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 ... | 2002 | 11862621 |
| [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 ... | 2001 | 11873615 |
| [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 ... | 2002 | 11938572 |
| 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. | 2002 | 11939480 |
| [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 ... | 2002 | 11998351 |
| epidemiology and control of bovine spongiform encephalopathy (bse). | bse is a new disease of cattle. the first clinical case occurred in april 1985 but the existence of a new disease was first confirmed microscopically in november 1986. epidemiological studies show that cattle suddenly became effectively exposed to a scrapie-like agent in ruminant-derived feed in the form of meat and bone meal in 1981/2. most cases have occurred in holstein friesian dairy cattle and have been exposed as calves. there is no evidence that cattle to cattle transmission sufficient to ... | 1993 | 8137136 |
| 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. | 2002 | 12027519 |
| 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 ... | 2002 | 12064251 |
| experimental infection of mink with bovine spongiform encephalopathy. | to determine whether the aetiological agent of bovine spongiform encephalopathy (bse) is pathogenic for mink, standard dark mink were inoculated with coded homogenates of bovine brain from the u.k. two homogenates were from cows affected with bse. the third was from a cow that came from a farm with no history of having had bse or having been fed ruminant-derived, rendered by-products, the proposed vehicle for introduction of the bse agent. each homogenate was inoculated intracerebrally into sepa ... | 1994 | 8077914 |
| 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 ... | 2002 | 12109876 |
| numbers of neurons in vacuolated and non-vacuolated neuroanatomical nuclei in bovine spongiform encephalopathy-affected brains. | morphometric studies of the brains of seven holstein-friesian cows affected with bovine spongiform encephalopathy (bse) and five control cows are reported. the numbers of neurons were counted in five selected neuroanatomical nuclei, namely the vestibular complex and red nucleus, both of which consistently show a high degree of neuronal perikaryonal vacuolation, the dorsal vagal nucleus, which shows inconsistent perikaryonal vacuolation, and the caudate and hypoglossal nuclei, which do not usuall ... | 1994 | 8040393 |
| bovine spongiform encephalopathy surveillance in argentina. | bovine spongiform encephalopathy (bse) is a new disease of cattle first described in the united kingdom in november 1986. bse belongs to the scrapie-related group of diseases. the epidemiological studies performed in the united kingdom demonstrate that the bse epidemic was caused by feeding cattle with ruminant-derived protein contaminated by a scrapie-like agent. until june 1994, the disease had been detected in indigenous cattle in ireland, switzerland and france. three cases reported in germa ... | 1994 | 7949355 |
| 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 ... | 2002 | 12204641 |
| 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, ... | 2002 | 12212894 |
| 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 ... | 2002 | 12222676 |
| [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 ... | 2002 | 12224461 |
| [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 ... | 2002 | 12224464 |
| 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 ... | 2002 | 12362140 |
| 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. | 2002 | 12376075 |
| 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 ... | 2002 | 12389035 |
| 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 ... | 2002 | 12410862 |
| 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 ... | 2002 | 12418529 |
| 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 ... | 2002 | 12424693 |
| 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 ... | 2002 | 12442578 |
| bse in cattle born after july 31, 1996, in great britain. | | 2002 | 12448497 |
| 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 ... | 2002 | 12449682 |
| a study of the inheritance of susceptibility to bovine spongiform encephalopathy. | a genetic study of 75 cases of bovine spongiform encephalopathy (bse) of which 51 were confirmed by histopathology in 29 pedigree and seven non-pedigree herds of holstein friesian cattle revealed that 73 per cent of 60 bse cases had first or second degree relatives also affected. all the 44 cases assigned to families could be traced back in the previous three generations to one cow and 11 bulls, which were of canadian holstein or dutch friesian heredity. no single common ancestor could be identi ... | 1990 | 2301106 |
| [british cattle plague--also a danger for man? bovine spongiform encephalopathy (bse)--scrapie disease]. | | 1990 | 2210580 |
| [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 ... | 2002 | 12585212 |
| 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 starte | 2000 | 12631966 |
| 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 ... | 2003 | 12747703 |
| 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 ... | 2003 | 12755818 |
| risk management of the transmissible spongiform encephalopathies in north america. | as 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 ... | 2003 | 12793780 |
| bovine spongiform encephalopathy (bse): the current situation and research. | bovine spongiform encephalopathy (bse), discovered in great britain in 1986, was to pose one of the most serious threats to the well-being of the british cattle industry this century. the disease is now established as a member of the group of diseases known as the sub-acute spongiform encephalopathies caused by unconventional, transmissible agents and which includes scrapie of sheep. it is from scrapie of sheep that it appears bse has resulted though it is possible bse may have existed in a sub- ... | 1991 | 1761112 |
| 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 ... | 2003 | 12793786 |
| ultrastructural pathology of axons and myelin in experimental scrapie in hamsters and bovine spongiform encephalopathy in cattle and a comparison with the panencephalopathic type of creutzfeldt-jakob disease. | we report the ultrastructural pathology of axons and myelin sheaths in bovine spongiform encephalopathy (bse) and experimental scrapie in hamsters and compare it with that found in a panencephalopathic model of creutzfeldt-jakob disease (cjd). intramyelinic vacuoles (myelin ballooning), dystrophic axons, phagocytic astrocytes and macrophages were found in all three models but to different degrees, while axons containing numerous cellular processes and concentric cisterns were observed only in ex ... | 1992 | 1644933 |
| 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 ... | 2003 | 12873216 |
| comparative ultrastructural neuropathology of naturally occurring bovine spongiform encephalopathy and experimentally induced scrapie and creutzfeldt-jakob disease. | we report the ultrastructural neuropathology of bovine spongiform encephalopathy (bse), a recently described slow virus disease first recognized in friesian/holstein cattle, and compare it to that of experimental scrapie and creutzfeldt-jakob disease. the spongiform change, which was most pronounced in the central grey matter of the midbrain, consisted of membrane-bound vacuoles within neuronal processes, containing curled membrane fragments, secondary chambers and vesicles. axons and dendrites ... | 1992 | 1644932 |
| 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 ... | 2003 | 14522859 |
| ultrastructural features of spongiform encephalopathy transmitted to mice from three species of bovidae. | the ultrastructural neuropathology of mice experimentally inoculated with brain tissue of nyala (tragelaphus angasi; subfamily bovinae), or kudu (tragelaphus strepsiceros; subfamily bovinae) affected with spongiform encephalopathy was compared with that of mice inoculated with brain tissue from cows (bos taurus; subfamily bovinae) with bovine spongiform encephalopathy (bse). as fresh brain tissue was not available for nyala or kudu, formalin-fixed tissues were used for transmission from these sp ... | 1992 | 1462768 |
| 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 ... | 2003 | 14645932 |
| 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 ... | 2004 | 14685769 |
| recommendations of the international roundtable workshop on bovine spongiform encephalopathy. | recommendations of the working party were summarized as follows: determine the status in all countries of their national cattle herds with respect to bse. attempt to develop a test to recognize bse-infected animals before they become clinically ill. establish procedures to prevent spread of bse agent into the cattle populations, especially by eliminating feeds containing rendered ruminant proteins. review the rendering processes, identify the sources and destinations of rendered products, and su ... | 1992 | 1348501 |
| 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 ... | 2004 | 14712176 |
| [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, ... | 2003 | 14740398 |
| [lessons from prion disease, mad cow disease, and iatrogenic creutzfeldt-jakob disease]. | | 2003 | 14743768 |
| 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 ... | 2003 | 14746770 |
| [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 ... | 2004 | 14770333 |
| 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. | 2004 | 14962521 |
| 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( ... | 2004 | 14967054 |
| 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. ... | 2004 | 14970340 |
| prion protein and species barriers in the transmissible spongiform encephalopathies. | in the transmissible spongiform encephalopathies (tse), the conversion of the normal protease-sensitive host protein prp-sen to an abnormal protease-resistant form, prp-res, is a critical step in disease pathogenesis. amino acid mismatches between prp-sen and prp-res can dramatically affect the amount of prp-res made and modulate the resistance to cross-species transmission of tse infectivity. experiments in transgenic mice, tissue culture cells, and cell-free systems have been used to identify ... | 1999 | 10221165 |
| 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 ... | 2004 | 15016886 |
| maternal transmission of bse: interpretation of the data on the offspring of bse-affected pedigree suckler cows. | | 1998 | 9634708 |
| evaluation of rapid tests for the diagnosis of transmissible spongiform encephalopathies in sheep and goats. | in 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 ... | 2004 | 15067554 |
| [does mad cow disease cause the new variant of creutzfeldt-jakob disease?]. | | 1998 | 9528287 |
| identification of a new prion strain in cattle--is there more than one form of bse? | | 2004 | 15181930 |
| 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 ... | 2004 | 15300459 |
| the cellular prion protein binds copper in vivo. | the normal cellular form of prion protein (prpc) is a precursor to the pathogenic protease-resistant forms (prpsc) believed to cause scrapie, bovine spongiform encephalopathy (bse) and creutzfeldt-jakob disease. its amino terminus contains the octapeptide phgggwgq, which is repeated four times and is among the best-preserved regions of mammalian prpc. here we show that the amino-terminal domain of prpc exhibits five to six sites that bind copper (cu(ii)) presented as a glycine chelate. at neutra ... | 1997 | 9414160 |
| 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 ... | 2004 | 15325025 |
| 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 ... | 2004 | 15354865 |
| 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 ( ... | 2004 | 15354868 |
| 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 ... | 2004 | 15457319 |
| 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 ... | 2004 | 15464187 |
| 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 ... | 2004 | 15468795 |
| 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 ... | 2004 | 15477588 |
| 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 ... | 2004 | 15520885 |
| 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 ... | 2004 | 15532881 |
| [the creutzfeld-jakob disease. a sphinx of current neurobiology]. | prospective epidemiological studies are being employed to determine the incidence and possible risk factors of creutzfeldt-jakob disease (cjd) in five european countries in which bovine spongiform encephalopathy (bse) occurs at different rates of incidence. | 1997 | 9340249 |
| bse and british cattle exports. | | 1997 | 9316246 |
| estimation of the age-dependent risk of infection to bse of dairy cattle in great britain. | an 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 ... | 2004 | 15579333 |
| bovine spongiform encephalopathy (bse): causes and consequences of a common source epidemic. | bovine spongiform encephalopathy (bse) is a transmissible spongiform encephalopathy (tse) or prion disease of cattle first recognized in 1986 in the united kingdom, where it produced a common source epidemic that peaked in january 1993 and has subsided markedly since that time. the epidemic began simultaneously at many geographic locations and was traced to contamination of meat and bone meal (mbm), a dietary supplement prepared from rendering of slaughterhouse offal. it appears that the epidemi ... | 1997 | 9169904 |
| phenotype of disease-associated prp accumulation in the brain of bovine spongiform encephalopathy experimentally infected sheep. | in 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 ... | 2005 | 15722546 |
| detection of bovine spongiform encephalopathy-specific prp(sc) by treatment with heat and guanidine thiocyanate. | the conversion of a ubiquitous cellular protein (prp(c)), an isoform of the prion protein (prp), to the pathology-associated isoform prp(sc) is one of the hallmarks of transmissible spongiform encephalopathies such as bovine spongiform encephalopathy (bse). accumulation of prp(sc) has been used to diagnose bse. here we describe a quantitative enzyme-linked immunosorbent assay (elisa) that involves antibodies against epitopes within the protease-resistant core of the prp molecule to measure the a ... | 1999 | 10516047 |
| 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 ... | 2005 | 15827140 |
| [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 ... | 2003 | 15884575 |
| [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 ... | 2005 | 15888987 |
| characterization of the murine bse infectious agent. | bovine spongiform encephalopathy (bse) is a prion-associated disease where the infectious agent is thought to be a host-encoded protein with a protease-resistant conformation (prp(sc)). here, data are presented on the solubilization of purified murine bse material, using guanidine-hcl as a denaturing agent. this treatment led to loss of infectivity, which was partially recovered on renaturation after dialysis to remove the chaotropic agent. the renatured product was then fractionated on an isopy ... | 2000 | 10811946 |
| 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 ... | 2005 | 15899572 |
| 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 ... | 2005 | 15961148 |
| 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 ... | 2004 | 15992291 |
| 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 ... | 2005 | 16002628 |
| 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 ... | 2005 | 16081647 |
| 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 ... | 2005 | 16216782 |
| 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 ... | 2005 | 16227190 |
| 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 ... | 2005 | 16284424 |
| 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 ... | 2005 | 16284804 |
| 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 ... | 2005 | 16301587 |
| 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 ... | 2006 | 16324707 |
| 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 ... | 1998 | 16363943 |
| 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 ... | 2006 | 16371015 |
| 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 ... | 2006 | 16450206 |
| 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 ... | 2000 | 11083045 |
| 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 ... | 2006 | 16542885 |
| 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 ... | 2007 | 16580230 |
| 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 ... | 2001 | 11161287 |
| 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 ... | 2006 | 16621340 |
| 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 ... | 2006 | 16632909 |