Publications

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 ...	2004	14975501
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
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
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
[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
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
[lessons from prion disease, mad cow disease, and iatrogenic creutzfeldt-jakob disease].		2003	14743768
[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
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
proteinase k enhanced immunoreactivity of the prion protein-specific monoclonal antibody 2a11.	here, 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 ...	2004	14687883
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
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
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 ...	2003	14535367
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
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 ...	2003	14509159
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
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 ...	2003	12828865
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
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. ...	2003	12793782
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
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
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
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
[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
[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 ...	2002	12585209
uk dietary exposure to bse in head meat: by birth cohort and gender.	uk 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.	2002	12501957
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
bse in cattle born after july 31, 1996, in great britain.		2002	12448497
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
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
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
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
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
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
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
[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
[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
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
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
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
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 ...	2002	12166421
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 ...	2002	12133662
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
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.	2002	12084158
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
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
[what does one really know about "mad cow" disease"?].		2001	12014331
[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
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
[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
[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 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
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
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
prion strain causing bovine spongiform encephalopathy (bse) in cattle.		2002	11807378
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 ...	2001	11724899
[still no swedish cases of mad cow disease and vcjd. continuous watchfulness is justified, especially of diffuse psychiatric symptoms].		2001	11715241
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 ...	2001	11690621
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 ...	2001	11688725
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 ...	2001	11585394
mad cow disease comes to japan.		2001	11564996
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 markers.	no 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 ...	2001	11520429
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, ...	2001	11440058
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 ...	2001	11432823
[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 ...	2001	11407273
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 ...	2001	11338713
[bse-cattle in germany. must we soon also count on vcjk patients?].		2001	11219274
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 ...	2000	11214925
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 ...	2000	11214922
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 ...	2000	11214914
[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 ...	2001	11213704
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, ...	2000	11190118
[how difficult it is to prevent mad cow disease].		2000	11189472
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
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 ...	2000	11110298
spain's bse cattle embargo causes tension at european commission.		2000	11095270
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 ...	2000	11087170
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
descriptive spatial analysis of the epidemic of bovine spongiform encephalopathy in great britain to june 1997.	this was a spatial analysis of the epidemic of bovine spongiform encephalopathy (bse) in great britain, based on agricultural census data collected between 1986 and 1996 and bse case data collected up to june 1997. kernel smoothing techniques were used to plot the distribution of bse-positive cattle holdings per 100 holdings per square kilometre and the distribution of confirmed bse cases per 100 head of cattle per square kilometre. in the early stages of the epidemic reported bse cases were sca ...	2000	11072999
aspects of the metabolism of dairy cows during the incubation of bovine spongiform encephalopathy.	as part of a nutritional study lasting from six weeks before calving to 22 weeks of lactation, blood samples collected from 47 dairy cows maintained under well-defined conditions were analysed for a variety of metabolites and hormones. at various times after the completion of the study, six of the animals developed clinical signs of bovine spongiform encephalopathy (bse), although when they were sampled it was not known that they were incubating the disease. the data were used to make comparison ...	2000	11072985
protease-resistant prion protein in brain and lymphoid organs of sheep within a naturally scrapie-infected flock.	the hallmark of transmissible spongiform encephalopathies (tse), such as scrapie in sheep, is the accumulation in tissues of an insoluble and protease resistant form (prpres) of the cellular prion protein. in this study, we evaluated whether the diversity in both the clinical pattern and the prp genotypes of scrapied sheep from the same flock was connected with different levels and/or glycoform patterns of the prpres in the brain and lymphoid organs of the animals. whereas the prpres levels in s ...	2000	10839972
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
ecosystems supporting clusters of sporadic tses demonstrate excesses of the radical-generating divalent cation manganese and deficiencies of antioxidant co factors cu, se, fe, zn. does a foreign cation substitution at prion protein's cu domain initiate tse?	analyses of food chains supporting isolated clusters of sporadic tses (cwd in n colorado, scrapie in iceland, cjd in slovakia) demonstrate a consistent 2 1/2+ fold greater concentration of the pro-oxidant divalent cation, manganese (mn), in relation to normal levels recorded in adjoining tse-free localities. deficiencies of the antioxidant co factors cu/se/zn/fe and mg, p and na were also consistently recorded in tse foodchains. similarities between the clinical/pathological profile of tses and ...	2000	10790765
feed-borne transmission and case clustering of bse.	an unresolved issue in the epidemiology of bovine spongiform encephalopathy (bse) in the uk is what precisely determines the degree to which cases of disease in cattle are clustered within herds throughout the course of the epidemic. this paper presents an analysis of feed-borne transmission at the herd level and tests various models of case-clustering mechanisms, associated with heterogeneity in exposure to infectious feed, against observed epidemic pattern. we use an age-structured metapopulat ...	2000	10714874
plus ça change ... from rinderpest to bovine spongiform encephalopathy.	cattle plague (rinderpest) caused serious loss of cattle in europe up to the beginning of the 20th century. effective control measures were developed in the 18th century by lancisi in italy and vicq d'azyr in france long before the viral nature of the disease was understood. similar measures are used to control bse, which unlike rinderpest, also infects man. much can be learned from earlier work on such problems as failure to notify outbreaks, inadequate application or deliberate evasion of cont ...	1999	10633340
the transmission of prions to humans.	the identification of new-variant creutzfeldt-jakob disease (nvcjd) in 1996 led to the proposal that this new disease was caused by the transmission of bovine spongiform encephalopathy (bse) to the human population. the ramifications of such a proposal have been extensive and profound, both politically and on the general public in the uk and other countries. patients with nvcjd exhibit a consistent set of clinicopathological features, and cases of nvcjd continue to be reported almost exclusively ...	1999	10626542
autoantibodies to brain components and antibodies to acinetobacter calcoaceticus are present in bovine spongiform encephalopathy.	bovine spongiform encephalopathy (bse) is a neurological disorder, predominantly of british cattle, which belongs to the group of transmissible spongiform encephalopathies together with creutzfeldt-jakob disease (cjd), kuru, and scrapie. autoantibodies to brain neurofilaments have been previously described in patients with cjd and kuru and in sheep affected by scrapie. spongiform-like changes have also been observed in chronic experimental allergic encephalomyelitis, at least in rabbits and guin ...	1999	10569779
nuclear dna fragmentation and immune reactivity in bovine spongiform encephalopathy.	to investigate whether apoptosis contributes to neuronal degeneration in bovine spongiform encephalopathy (bse), morphological changes consistent with apoptosis were sought and in-situ end labelling (isel) was applied, in a series of 20 bse cases and 10 age-matched normal control cattle. apoptotic changes were not found in neurons but were occasionally seen in glial cells. relatively few isel-positive neurons were found, but many labelled nuclei were seen in glial cells in certain areas. none of ...	1999	10542125
creutzfeldt-jakob disease, new variant creutzfeldt-jakob disease, and bovine spongiform encephalopathy.	creutzfeldt-jakob disease (cjd) is a subacute spongiform encephalopathy (sse) that is manifested by a variety of neurologic signs that usually include dementia, myoclonus, and an abnormal electroencephalogram (eeg). in 1996, a new variant of cjd (nvcjd) with a somewhat distinctive clinical presentation and neuropathology was reported in adolescents and young adults, a cohort of patients not normally affected with cjd. the appearance of nvcjd coincided temporally and geographically with the emerg ...	1999	10517931
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
risk assessment of bovine spongiform encephalopathy transmission through bone graft material derived from bovine bone used for dental applications.	several commercial products are currently available for clinical application as bone graft substitutes. these products can be broadly classified into two categories: synthetic and natural. bovine bone is a popular source for several of the natural bone substitutes. the availability of bovine derived xenogenic bone substitutes has made it possible to avoid traumatic and expensive secondary surgery to obtain autogenous bone once thought essential for effective bone replacement. while autogenous bo ...	1999	10505809
differences in proteinase k resistance and neuronal deposition of abnormal prion proteins characterize bovine spongiform encephalopathy (bse) and scrapie strains.	prion diseases are associated with the accumulation of an abnormal isoform of host-encoded prion protein (prp(sc)). a number of prion strains can be distinguished by "glycotyping" analysis of the respective deposited prp(sc) compound. in this study, the long-term proteinase k resistance, the molecular mass, and the localization of prp(sc) deposits derived from conventional and transgenic mice inoculated with 11 different bse and scrapie strains or isolates were examined. differences were found i ...	1999	10415165
risk of transmission of bovine spongiform encephalopathy to humans in the united states: report of the council on scientific affairs. american medical association.	the risk of possible transmission of bovine spongiform encephalopathy (bse) in the united states is a substantial public health concern.	1999	10386559
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
experimentally induced bovine spongiform encephalopathy did not transmit via goat embryos.	goats are susceptible to experimental challenge with bovine spongiform encephalopathy (bse). this study set out to investigate whether the transmission of bse could occur in goats following the transfer of embryos from experimentally infected donor females into uninfected recipient females. the results showed no evidence of transmissible spongiform encephalopathy disease in any of the offspring which developed from embryos from infected donors, nor indeed in any of the recipient females used as ...	1999	10073715
molecular analysis of ovine prion protein identifies similarities between bse and an experimental isolate of natural scrapie, ch1641.	new variant creutzfeldt-jakob disease (vcjd) and bovine spongiform encephalopathy (bse) are caused by the same strain of pathogen and, as sheep can develop experimental bse, this has raised concern that humans may be at risk from eating mutton if bse has naturally transmitted to sheep. biochemical typing of abnormal prion proteins (prpsc) has been suggested to detect bse in sheep. although this approach is ingenuous, we can now report biochemical evidence of strain variation in contemporary and ...	1999	9934675
[a review of the current research on prions. the evidence suggests the possibility of transmission of the mad cow disease to humans].	further evidence of the transmissibility of bovine spongiform encephalopathy (bse) across the species barrier from cow to man has been derived from epidemiological analysis and the characterisation of prion strains. recent research has shown the persistence of prions after experimental transmission to resistant murine species, and subclinical persistence in cows. the accumulation of pathological prion proteins in tonsils and appendix has been demonstrated prior to clinical confirmation of the pr ...	1998	9854377
an overview of the bse epidemic in the uk.	the bse epidemic in the uk is declining rapidly in response to control measures and is en route for eradication by the year 2001. bse infectivity is restricted in clinically affected natural cases of bse to central nervous tissues. bse agent has been isolated from the intestine of experimentally orally-infected cattle from six to 18 months post-challenge. a small maternal risk factor for bse has been identified but this will not stop the eradication of bse. to protect the integrity of biological ...	1998	9737379
why fibrous proteins are romantic.	here i give a personal account of the great history of fibrous protein structure. i describe how astbury first recognized the essential simplicity of fibrous proteins and their paradigmatic role in protein structure. the poor diffraction patterns yielded by these proteins were then deciphered by pauling, crick, ramachandran and others (in part by model building) to reveal alpha-helical coiled coils, beta-sheets, and the collagen triple helical coiled coil-all characterized by different local seq ...	1998	9724602
transmissible spongiform encephalopathies, hypotheses and food safety: an overview.	the transmissible spongiform encephalopathies (tses) (in both humans and animals) have been reviewed with the principal focus on bovine spongiform encephalopathy (bse), its recent outbreak amongst cattle in the uk ('mad cow disease') and its sequelae. the possible reasons for this outbreak are discussed as well as a number of hypotheses reviewed for tses (e.g. prions, organophosphates, etc.) and current measures attendant with food safety and surveillance taken in the european union. it is gener ...	1998	9695172