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bovine spongiform encephalopathy: an overview.bovine spongiform encephalopathy (bse), a novel disease of cattle first described in 1986, has now reached epidemic proportion in great britain with about 500 cases a week. the clinical, epidemiological, and pathological characteristics of bse are described. moreover, the etiopathogenetic mechanisms of spongiform encephalopathies are reviewed. legislative measures to prevent the spread of bse in italy and in other eec countries and to minimize the theoretical risk to man are reported.19921303043
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 ...19921348501
bovine spongiform encephalopathy (bse): a stimulus to wider research.the severity of the epidemic of bovine spongiform encephalopathy which is currently afflicting cattle in the british isles has stimulated a considerable research effort, much of which is directed toward understanding the aetiology and pathogenesis of the bovine disease. however, a significant thrust has also been orchestrated to address more fundamental issues such as the nature of the uncharacterized causal agents of the wider range of unusual animal and human diseases which share similar chara ...19921364085
recently described scrapie-like encephalopathies of animals: case definitions.since 1986, naturally occurring scrapie-like encephalopathies have been described in the united kingdom in domestic cattle, in five species of captive exotic bovids and in domestic cats. the disease in domestic cattle, bovine spongiform encephalopathy, has been characterised by all currently available diagnostic criteria as a transmissible spongiform encephalopathy or 'prion' disease, and has been shown to have a dietary origin. the pathology in the other species is also entirely consistent with ...19921410804
idiopathic brainstem neuronal chromatolysis and hippocampal sclerosis: a novel encephalopathy in clinically suspect cases of bovine spongiform encephalopathy.some of the brains submitted for neurohistopathological examination under the bovine spongiform encephalopathy (bse) orders did not show lesions of bse. they showed neuronal chromatolysis and necrosis of the brainstem, perivascular cuffs and meningeal infiltrates of mononuclear cells and large irregularly shaped vacuoles in the neuropil. about half of them also showed loss of pyramidal neurons in the hippocampus, with astrocytic gliosis. the topography of the brainstem neuronal degeneration and ...19921441151
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 ...19921462768
[spongiform encephalopathies with special reference to bovine spongiform encephalopathy].in switzerland bovine spongiform encephalopathy (bse) was detected for the first time in november 1990. it is a transmissible disease of the central nervous system similar to creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker syndrome (gss) and kuru in man, and, in animals, scrapie in sheep and goats, chronic wasting disease (cwd) in captive mule deer and elk of north america and transmissible mink encephalopathy (tme) of farm reared mink. the infectious agent of the spongiform ence ...19921615298
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 ...19921644932
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 ...19921644933
bovine spongiform encephalopathy: a neuropathological perspective.the occurrence of bovine spongiform encephalopathy (bse), recognition that it is a new scrapie-like disease epidemic in domestic cattle in the united kingdom and concern of a remote zoonotic potential has, in four years, produced a plethora of documented information. while much of this information has been communicated outwith the scientific literature, this review attempts to summarise, from a neuropathological viewpoint, the main findings to emerge. the initial studies established the nosologi ...19911688299
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- ...19911761112
beef and bovine spongiform encephalopathy: the risk persists.bovine spongiform encephalopathy (bse) is one of the transmissible spongiform encephalopathies (tse) that are currently known to the authors to affect only mammals, including man. the diseases are progressive, fatal paralyses and dementias, for which there are no methods of certain diagnosis and no treatment. in this review the disease in cattle, the mode of transfer of these tses between animals by mouth, the possible presence of infective agents in the food that we eat, the resistance of bse t ...19911923069
is bse simply scrapie in cattle? 19902111595
[british cattle plague--also a danger for man? bovine spongiform encephalopathy (bse)--scrapie disease]. 19902210580
spongiform encephalopathy in an arabian oryx (oryx leucoryx) and a greater kudu (tragelaphus strepsiceros)clinical, pathological and epidemiological details of scrapie-like encephalopathies are described in an arabian oryx and a greater kudu. clinical signs included ataxia and loss of condition with a short, progressive clinical course (22 and three days, respectively). histopathological examination of the brains revealed spongiform encephalopathy characteristic of that observed in scrapie and bovine spongiform encephalopathy (bse). it seems probable that these cases have a common aetiology with bse ...19902264242
bovine spongiform encephalopathy: a scrapie-like disease of british cattle.scrapie is a cns degenerative infection of sheep and goats, which is invariably fatal after incubation periods of several months to years. related disorders are found naturally in man and other species. there is a impairment of protein catabolism in scrapie and related diseases which leads to the accumulation of sparingly-soluble protein deposits in brain. these protein aggregates may share with the amyloid of alzheimer's disease (ad) some common stage in the biochemical pathways of their format ...19892574875
occurrence of 14 cases of bovine spongiform encephalopathy in a closed dairy herd.fourteen cases of bovine spongiform encephalopathy (bse) were diagnosed on the basis of clinical examination in a closed herd of british friesian cows during a 9-month period from october 1987 until june 1988. the diagnosis was confirmed on histopathological examination of brain tissue from five of the six samples submitted. the main presenting clinical signs were of altered behaviour: apprehension, anxiety and hyperaesthesia. one cow was euthanized after a short period of recumbency; the remain ...19892713640
bovine spongiform encephalopathy in an adult british friesian cow. 19883195029
bovine spongiform encephalopathy: epidemiological studies.this study, initiated in june 1987, describes the epidemiology of bovine spongiform encephalopathy (bse), a recently described novel neurological disease of domestic cattle first identified in great britain in november 1986. records suggested that the earliest suspected cases occurred in april 1985. there was variability in the presenting signs and the disease course, but the majority of cases developed behavioural disorders, gait ataxia, paresis and loss of bodyweight; pruritus was not a predom ...19883218047
tubulovesicular structures (tvs): virus-like particles specific for all subacute spongiform virus encephalopathies--what are they really?tubulovesicular structures (tvs) are virus-like particles specific for all the subacute spongiform virus encephalopathies (ssve). i report here the presence of tvs in the highest range of naturally occurring and experimentally induced ssve studied so far: natural and experimental creutzfeld-jakob disease (cjd), gerstmann-sträussler-scheinker syndrome, natural bovine spongiform encephalopathy (bse) and bse transmitted to pigs and four models of experimental scrapie in hamsters. tvs are spherical ...19947503652
western blot mapping of disease-specific amyloid in various animal species and humans with transmissible spongiform encephalopathies using a high-yield purification method.saf-protein, an amyloid, is the main constituent of scrapie-associated fibrils (saf) and a specific marker for transmissible spongiform encephalopathies (tse). using an improved extraction method and western blot detection, the disease-specific amyloid was found in various parts of the central nervous system of hamsters orally infected with scrapie, of squirrel monkeys orally infected with kuru, sporadic creutzfeldt-jakob disease (cjd) and scrapie, of human patients with sporadic cjd, of a sheep ...19957595360
studies on a species-specific epitope in murine, ovine and bovine prion protein.transmissible spongiform encephalopathies are fatal neurodegenerative disorders which are linked to abnormal isoforms of the prion protein (prp), which is expressed in different cells of various mammalian species. susceptibility to disease and reduced transmission rates upon the first passage to another species are thought to be a result of functional and biochemical differences of the prp as a consequence of amino acid sequence among species. in 1985 an epidemic of bovine spongiform encephalopa ...19937687651
bovine spongiform encephalopathy in a holstein cow born in the united kingdom during september 1989. 19957773920
[scrapie in sheep and transmissible encephalopathy of the mink].scrapie in sheep and goat is the prototype of the group of the transmissible spongiform encephalopathies which affect man and some animal species, notably other ruminants with bovine spongiform encephalopathy (bse) and chronic wasting disease of wild ruminants. transmissible mink encephalopathy (tme) is a rare disease of ranch-raised mink caused by exposure to a contaminated food ingredient in the ration scrapie, unrecognised bse-like disease...). there is clinical and pathological similarities ...19957777384
epidemiological observations on spongiform encephalopathies in captive wild animals in the british isles.since 1986, scrapie-like spongiform encephalopathy has been diagnosed in 19 captive wild animals of eight species at or from eight zoological collections in the british isles. the affected animals have comprised members of the family bovidae: one nyala (tragelaphus angasi), four eland (taurotragus oryx), and six greater kudu (tragelaphus strepsiceros), one gemsbok (oryx gazella), one arabian oryx (oryx leucoryx), and one scimitar-horned oryx (oryx dammah), and members of the family felidae: four ...19947817514
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 ...19947949355
hungary remains free of scrapie and bovine spongiform encephalopathy (bse).brains from 44 sheep and 43 cattle with cns clinical signs not due to rabies virus infection were collected from diagnostic institutes throughout hungary. the brains were examined for histological lesions diagnostic of scrapie/bovine spongiform encephalopathy (bse) and all were found to be negative. these findings confirm that hungary remains free of scrapie and bse.19938017235
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 ...19948077914
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 ...19938137136
transmissible spongiform encephalopathies and the safety of naturally-derived biologicals.late in 1986, a new neurodegenerative disease, referred to as bovine spongiform encephalopathy (bse), was recognized in domestic cattle in southern england. since then, tens of thousands of cases have been confirmed throughout the u.k. and the disease has also appeared sporadically outside of the british isles. bse belongs to a group of rare, progressive and fatal disorders of the central nervous system of animals and man caused by anomalous infectious agents whose properties are not yet full un ...19938217119
bse--a risk for man through pharmaceutical products? position and politics of the german pharmaceutical industry.since bse is not a zoonosis and the occurrence is with some exceptions extremely low or absent, the risk to man through pharmaceuticals is remote. however, the agent of bse is very resistant and the disease in cattle is always lethal, as are analogous diseases of man. therefore, the german pharmaceutical industry, through a working group, actively contributes to reasonable measurements leading to a further reduction of any theoretical risk. this theoretical risk has to be evaluated by a balanced ...19938270110
pathology of nonhuman spongiform encephalopathies: variations and their implications for pathogenesis.microscopic cavitation of the central nervous system (cns) is a variable, non-specific feature of several different diseases of animals. in none, however, has it received more discussion than in scrapie, a naturally occurring disease of sheep, the clinical signs of which have been known for at least two centuries; yet consensus on the essential neurodegenerative pathology of scrapie emerged only three decades ago. the subsequent recognition of such changes in other species, including man, was a ...19938270117
world health organization consultation on public health issues related to bovine spongiform encephalopathy and the emergence of a new variant of creutzfeldt-jakob disease.at a world health organization (who) consultation organized in geneva on april 2-3, 1996, a group of international experts reviewed the public health issues related to bovine spongiform encephalopathy (bse) and the emergence of a new variant of creutzfeldt-jakob disease (v-cjd), as officially reported by the united kingdom on march 20, 1996. the consultation made recommendations, based on the latest scientific information, to minimize transmission of bse among animals and to reduce as completely ...19968598828
the association between prp and infectivity in scrapie and bse infected mouse brain.the structure of the scrapie agent remains unknown. however, scrapie infectivity tends to co-sediment with an infection specific fraction of the glycoprotein prp (prpsc) under conditions which solubilise the normal form of this protein (prpc); accordingly, prp has been proposed as a candidate component of the agent. to investigate this further we have been examining a new scrapie-related murine model in conjunction with established scrapie models. a bovine spongiform encephalopathy (bse) derived ...19968634020
[prion diseases. review of the literature on the light of two case reports of creutzfeldt-jakob disease].during the last ten years the diseases scrapie in sheep and bovine spongiform encephalopathy (or mad cow disease) in cattle have received increased attention. through the 1960s it became apparent that scrapie in sheep and kuru and later creutzfeldt-jakob disease in man were infectious diseases. during the last decade the appearance of mad cow disease in great britain has increased the fear that humans can develop creutzfeldt-jakob disease through their food. a special characteristic of the infec ...19968658476
[mad-cow disease; a strange cow disease]. 19968693479
nmr structure of the mouse prion protein domain prp(121-321).the 'protein only' hypothesis states that a modified form of normal prion protein triggers infectious neurodegenerative diseases, such as bovine spongiform encephalopathy (bse), or creutzfeldt-jakob disease (cjd) in humans. prion proteins are thought to exist in two different conformations: the 'benign' prpcform, and the infectious 'scrapie form', prpsc. knowledge of the three-dimensional structure of prpc is essential for understanding the transition to prpsc. the nuclear magnetic resonance (nm ...19968700211
maternal transmission of bse demonstrated in cattle. 19968709742
transmission dynamics and epidemiology of bse in british cattle.a comprehensive analysis of the bovine spongiform encephalopathy (bse) epidemic in cattle in great britain assesses past, present and future patterns in the incidence of infection and disease, and allows a critical appraisal of different culling policies for eradication of the disease.19968752271
strain specific and common pathogenic events in murine models of scrapie and bovine spongiform encephalopathy.the development of transmissible spongiform encephalopathies in experimental models depends on two major factors: the intracerebral accumulation of an abnormal, protease-resistant isoform of prp (prpres), which is a host protein mainly expressed in neurons; and the existence of different strains of agent. in order to make a distinction between pathogenic mechanisms depending upon the accumulation of host-derived prpres and the strain-specific effects, we quantified and compared the sequence of m ...19968758005
protease-resistant prp deposition in brain and non-central nervous system tissues of a murine model of bovine spongiform encephalopathy.infectivity within the central nervous system has been demonstrated by the transmission of bovine spongiform encephalopathy (bse) from affected cattle to inbred laboratory mice. sedimentable, protease-resistant prp (prpsc) has also been extracted from bse-affected cattle brain. both infectivity and prpsc have been reported in the lymphoreticular tissues of sheep and mice clinically and preclinically affected with scrapie. neither infectivity nor prpsc has yet been detected in non-neural tissues ...19968760446
differential effects of a new amphotericin b derivative, ms-8209, on mouse bse and scrapie: implications for the mechanism of action of polyene antibiotics.mice were infected intracerebrally with the bovine spongiform encephalopathy (bse) or the scrapie agent and treated during 8 weeks postinfection to test the protective effect of a new amphotericin b (amb) derivative, ms-8209, in experimental transmissible spongiform encephalopathies. the results show that (i) the treatment prolonged the incubation period of both bse-infected and scrapie-infected mice, (ii) ms-8209 and amb were much more efficient in delaying the onset of scrapie than that of bse ...19968837228
separating the environmental and genetic factors that may be causes of bovine spongiform encephalopathy.the initial cause of the bovine spongiform encephalopathy (bse) epidemic is generally accepted to have been the feeding of infected animal protein to cattle. the proportion of animals affected in any year in a particular herd has generally been low. this suggests either considerable variation in the extent of challenge of the individual animals or variation in their susceptibility to challenge or both. there is known to be genetic variation in susceptibility in other spongiform encephalopathies, ...19968856808
reduction of the infectivity of scrapie agent as a model for bse in the manufacturing process of trasylol.the trasylol manufacturing process was investigated with respect to its capacity for the inactivation/removal of infectivity causing bovine spongiform encephalopathy (bse). four process steps were selected for this investigation and scaled down to laboratory scale. authentic samples of bovine lungs used in the trasylol manufacturing plant were taken and spiked in laboratory scale experiments with high infectious titres of the rodent adapted scrapie strain me 7 which served as model for bse. afte ...19968889056
idiopathic brainstem neuronal chromatolysis of cattle: a disorder with clinical similarity to bse. 19968913015
transmissible spongiform encephalopathies (tse): minimizing the risk of transmission by biological/biopharmaceutical products: an industry perspective.several guidelines and recommendations have been published on assessing the potential risk of a biological product being contaminated with an agent causing a transmissible spongiform encephalopathy (tse). basic principles which can be used during the manufacturing of biological products to minimize the risk of transmission of tse agents include the following: (i) obtaining animals, tissues or animal-derived raw materials from countries in which the relevant tse agent is reported to be absent; (i ...19969119148
[prion diseases and a new variant of creutzfeldt-jakob disease].the causal link of a new variant of cjd (v-cjd) with bovine spongiform encephalopathy (bse) has led to world-wide panic. bse emerged in 1986 through dietary products contaminated with scrapie pathogen, bse case reports increased in number up to 37,000/year in 1993, then declined in 1994 when the first case of v-cjd emerged. there is a 3-year gap between the emergence of bse and the introduction of a ban on the use of specified bovine offal in human food. people might have consumed dietary produc ...19969128415
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 ...19979169904
evolution of a strain of cjd that induces bse-like plaques.bovine spongiform encephalopathy (bse) has become a public health issue because a recently evolved bse agent has infected people, yielding an unusual form of creutzfeld-jakob disease (cjd). a new cjd agent that provokes similar amyloid plaques and cerebellar pathology was serially propagated. first-passage rats showed obvious clinical signs and activated microglia but had negligible prp-res (the more protease-resistant form of host prp) or cerebellar lesions. microglia and astrocytes may partici ...19979204907
prion protein nmr structure and species barrier for prion diseases.the structural basis of species specificity of transmissible spongiform encephalopathies, such as bovine spongiform encephalopathy or "mad cow disease" and creutzfeldt-jakob disease in humans, has been investigated using the refined nmr structure of the c-terminal domain of the mouse prion protein with residues 121-231. a database search for mammalian prion proteins yielded 23 different sequences for the fragment 124-226, which display a high degree of sequence identity and show relevant amino a ...19979207082
[epidemiological studies on the transmission of bovine spongiform encephalopathy (bse)--remarks from a biometric viewpoint].this contribution discusses biometrical aspects of the design and analysis of epidemiological case-control studies. the study chosen as an example was undertaken to investigate the transmission of bovine spongiform encephalopathy (bse) to calves and the role of the inclusion of meat and bonemeal in proprietary feedstuffs. the hypothesis was examined by means of a case-control study which is summarized with emphasis on its study design and the collection of data. the analysis included the estimat ...19979290036
bse and british cattle exports. 19979316246
bovine spongiform encephalopathy: is it an autoimmune disease due to bacteria showing molecular mimicry with brain antigens?bovine spongiform encephalopathy (bse) could be an autoimmune disease produced following exposure of cattle to feedstuffs containing bacteria showing molecular mimicry between bacterial components and bovine tissue. analysis of molecular sequence databases (genbank and swissprot) shows that three bacteria (acinetobacter calcoaceticus,ruminococcus albus, and agrobacter tumefaciens) share sequences with the encephalitogenic peptide of bovine myelin, while three molecules in escherichia coli show m ...19979370514
[slow virus infections: mad cow disease and the debate about a disease not transmitted by a virus]. 19979376398
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 ...19979414160
[does mad cow disease cause the new variant of creutzfeldt-jakob disease?]. 19989528287
emerging viruses.an emerging virus is a term applied to a newly discovered virus, one that is increasing in incidence or with the potential to increase in incidence. many viruses fit into this definition. hiv is the clearest example of a previously unknown virus that has now produced one of the largest pandemics in history. recent advances have occurred in the identification and understanding of new hantaviruses in the americas, causing an acute respiratory disease. the possible causal role of human herpesvirus ...19989529635
kuru and "new variant" cjd.acquired transmissible spongiform encephalopathies in humans include kuru (a disease which was associated with ritualistic cannibalism in papua new guinea), iatrogenic creutzfeldt-jakob disease and a newly recognized variant form of creutzfeldt-jakob disease (nvcjd). clinical and neuropathological features of nvcjd are reminiscent of kuru: early and progressive cerebellar ataxia and numerous characteristic kuru-type amyloid plaques surrounded by spongiform change. in contrast to typical cases of ...19979561604
new variant creutzfeldt-jakob disease.since the report of new variant creutzfeldt-jakob disease (nvcjd) in humans last year, the search was on for direct evidence to link the condition to bovine spongiform encephalopathy (bse). the first case nvcjd was noted 10 years after the recognition of bse in uk cattle. a direct link is now established. there are, however, some 'protective' mechanisms, the most important of which are the inefficiency of the gastric route of introducing the infected material, the species barrier and genetic 'pr ...19989562257
synthetic peptide vaccines yield monoclonal antibodies to cellular and pathological prion proteins of ruminants.transmissible spongiform encephalopathies are closely linked to the accumulation of a pathological isoform of a host-encoded prion protein (prp(c)), designated prp(sc). in an attempt to generate mono- and polyclonal antibodies to ruminant prp, 32 mice were vaccinated with peptide vaccines which were synthesized according to the amino acid sequence of ovine prp. by this approach five prp-reactive polyclonal antisera directed against four different domains of the protein were stimulated. splenocyt ...19989568991
bse: can we predict the future?prion diseases are transmissible neurodegenerative disorders of humans and animals. the prion protein (prpc) gene is expressed to some extent in many cell types but principally in neurons. normal prpc may contribute in the protection of neurons and are protease sensitive. abnormal prions consist of a post-translationally modified form of prp, prpsc, which is partly protease resistant. prpsc is a protein with high resistance to inactivation by irradiation, heat and harsh chemical treatments. it i ...19979581370
maternal transmission of bse: interpretation of the data on the offspring of bse-affected pedigree suckler cows. 19989634708
new variant creutzfeldt-jakob disease.new variant creutzfeldt-jakob disease is a novel human prion disorder with characteristic clinical and neuropathological features, which results from exposure to the bovine spongiform encephalopathy agent. the probably lengthy incubation period makes it difficult to predict future new variant creutzfeldt-jakob disease case numbers; further studies are required to clarify risk factors and the potential for human spread.19989642546
[transmissible spongiform encephalopathy].transmissible spongiform encephalopaties (tse) are a group of rare fatal diseases of humans and animals. prions, small infectious proteinaceous particles, are their supposed cause. prusiner's theory (nobel price 1997) proves that pathogenetically active prions are conformated physiological prions but problems of pathogenesis of tse are still open. most important representative of human tse is creutzfeldt-jakob's disease (cjd), that of animal tes is bovine spongiform encephalopathy (bse). it seem ...19989650345
the mad cow problem in the uk: risk perceptions, risk management, and health policy development.mad cow disease or bovine spongiform encephalopathy (bse) is a fatal neurological disease of cattle first recognized in the united kingdom (uk) in 1986. until recently, the uk government considered the chance of a human becoming infected with the bse agent to be extremely remote. as a result of new developments, alarmist media attention, bureaucratic mishandling of the issues, scientific uncertainty, bickering among technical experts, and a dearth of easily assimilated and balanced information o ...19989670700
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 ...19989695172
[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 ...19989854377
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 ...19999934675
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 ...199910073715
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 ...199910221165
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.199910386559
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 ...199910415165
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 ...199910516047
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 ...199910569779
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 ...199910626542
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 ...200010714874
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 ...200010790765
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 ...200010839972
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 ...200011072985
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 ...200011072999
temporal aspects of the epidemic of bovine spongiform encephalopathy in great britain: individual animal-associated risk factors for the disease.the objectives of this study were first to determine the cumulative incidence of bovine spongiform encephalopathy (bse) in the british cattle population from july 1986 to june 1997, secondly, to identify individual animal-associated risk factors that influenced the age of onset of clinical signs in confirmed bse cases, and, thirdly, to assess the effectiveness of the measures introduced to control bse during the epidemic. the analyses were based on the population of british cattle at risk, deriv ...200011083045
human prion diseases.the term 'prion diseases' refers to a group of neurodegenerative disorders thought to be caused by prions, pathogenic agents with novel modes of replication and transmission. prion diseases are characterized by long incubation periods ranging from months to years and are invariably fatal once clinical symptoms have appeared. they are also called transmissible spongiform encephalopathies (tse), on account of the predominant neuropathological change observed in the central nervous system. the most ...200011087170
bovine spongiform encephalopathy: an overview.bovine spongiform encephalopathy (bse), widely known as "mad cow disease," is a chronic, degenerative disease affecting the central nervous system of cattle. worldwide, there have been more than 180,000 cases since the disease was first diagnosed in 1986 in great britain. bovine spongiform encephalopathy has had a substantial impact on the livestock industry in the united kingdom. the disease has also been confirmed in native-born cattle in belgium, denmark, france, ireland, luxembourg, liechten ...200011110298
heat stability of prion rods and recombinant prion protein in water, lipid and lipid-water mixtures.prion rods, i.e. insoluble infectious aggregates of the n-terminally truncated form of the prion protein, prp 27-30, and the corresponding recombinant protein, rprp(90-231), were autoclaved in water, bovine lipid or lipid-water mixtures for 20 min at temperatures from 100 to 170 degrees c. a protocol was developed for the quantitative precipitation of small amounts of protein from large excesses of lipid. prp remaining undegraded after autoclaving was quantified by western blot and degradation f ...200111161287
public health service recommendations for the use of vaccines manufactured with bovine-derived materials.the center for biologics evaluation and research (cber), u.s. food and drug administration (fda) learned earlier this year that some vaccines were manufactured with bovine-derived materials obtained from countries in which bovine spongiform encephalopathy (bse) or a substantial risk for bse exists. a list of these countries is published by the u.s. department of agriculture (usda). this information was of concern because cases of variant creutzfeldt-jakob disease (vcjd) have been attributed to, ...200011190118
[still a small problem with the mad cow disease? creutzfeldt-jakob disease and other prion diseases: current status].this review is based on recent published research on the bse/cjd/vcjd problem mainly from uk, germany and france. the situation in sweden seems to be fortunate for several reasons. the use of meat and bonemeal as animal fodder was forbidden in this country 13 years ago. sweden has not had any sheep with scrapie for many years. no animals with bse have so far been found in our country. the incidence of sporadic cjd in this country followed retrospectively from 1985 to 1996 and prospectively from ...200111213704
surveillance of bse.the current method used to identify suspect bse cases is based on reporting cattle displaying clinical signs compatible with bse. the reporting of such cases is dependent on the ability of farmers and veterinarians to recognise the disease symptoms and on the willingness to report such cases. furthermore, it depends on the stage of the disease, because early clinical signs of bse are not always typical. histology and immunohistochemistry are established and reliable to confirm bse in cattle, but ...200011214914
application of prionics western blotting procedure to screen for bse in cattle regularly slaughtered at swiss abattoirs.disease-specific prp (prp(sc)) is at least part of the infectious particle (prion) causing bovine spongiform encephalopathy (bse) or scrapie in sheep. digestion with protease allows a distinction between normal prp (prp(c)) and prp(sc) i.e. prp(c) is completely digested while prp(sc) is cleaved at the n-terminus leading to a fragment of reduced molecular weight (prp 27-30). detection of this fragment by western blotting has been described more than a decade ago for rodent prp. we have now optimi ...200011214922
characterization of bse and scrapie strains/isolates.following the bse epidemic in cattle and the emergence of a variant form of creutzfeldt-jakob disease in humans, the question was raised whether bse has been transmitted to small ruminants by the inadvertent feeding of infectious meat and bone meal. such infections could easily be concealed in countries where scrapie is endemic. to address this issue by immuno-chemically analyzing the prp(sc) fragments, we have developed two lines of research. firstly we have focused on the development of criter ...200011214925
[psychiatric manifestations of a new variant of creutzfeldt-jakob disease. apropos of a case].the new variant of creutzfeldt-jakob disease (nvcjd) was first described in the uk in 1996 (16). the nvcjd differs from sporadic, genetic and iatrogenic cjd. creutzfeldt-jakob disease is closely associated with an abnormal isoform prpsc of a cell-surface glycoprotein, prion protein (14). molecular analysis suggests that nvcjd is caused by the same prion strain as bovine spongiform encephalopathy (bse) (4, 10). to the end of september 2000, there have been 82 cases of nvcjd in the uk. we report t ...200111407273
scrapie strains maintain biological phenotypes on propagation in a cell line in culture.bovine spongiform encephalopathy (bse) and its human equivalent, variant creutzfeldt-jakob disease (vcjd), are caused by the same strain of infectious agent, which is similar to, but distinct from, >20 strains of their sheep scrapie homologue. a better understanding of the molecular strain determinants could be obtained from cells in monoculture than from whole animal studies where different cell targeting is commonly a strain-related feature. although a few cell types can be infected with diffe ...200111432823
bse as an organizational construction: a case study on the globalization of risk.this article examines the bse problem as an example of the 'globalization of risk'. in order to determine whether the 'globalization of risk' is a social construction depending on the context, the paper emphasizes the particular role of organizations. it makes an empirical comparison of the bse-related risk-constructions of five business associations in the german meat industry sector. the results show that the associations construct the risk in close relation to their horizons of globalization, ...200111440058
high-dose exposure to systemic phosmet insecticide modifies the phosphatidylinositol anchor on the prion protein: the origins of new variant transmissible spongiform encephalopathies?compulsory exposure of the uk bovine to exclusively high biannual doses of a 'systemic' pour-on formulation of an organo-phthalimido-phosphorus warblecide, phosmet, during the 1980s (combined with exposure to the lipid-bound residues of 'bioconcentrated' phosmet recycled back via the intensive feeding of meat and bone meal), initiated the 'new strain' modification of the cns prion protein (prp) causing the uk's bovine spongiform encephalopathy (bse) epidemic. a lipophilic solution of phosmet was ...19989572563
mad cow disease comes to japan. 200111564996
mri of creutzfeldt-jakob disease: imaging features and recommended mri protocol.creutzfeldt-jakob disease (cjd) is a rare, progressive and invariably fatal neurodegenerative disease characterized by specific histopathological features. of the four subtypes of cjd described, the commonest is sporadic cjd (scjd). more recently, a new clinically distinct form of the disease affecting younger patients, known as variant cjd (vcjd), has been identified, and this has been causally linked to the bovine spongiform encephalopathy (bse) agent in cattle. characteristic appearances on m ...200111585394
the role of mri in the diagnosis of sporadic and variant creutzfeldt-jakob disease.creutzfeldt-jakob disease (cjd) is a rare but important fatal, dementing illness. a number of types of cjd are identified, each with distinct clinical features. characteristic mri changes have been described recently. sporadic cjd, the commonest type, is found worldwide, and causes hyperintensity of the putamen and caudate nuclei. in the recently described variant cjd, which affects younger patients and has been linked to bovine spongiform encephalopathy (bse) in cattle, a highly characteristic ...200111688725
the shifting biology of prions.transmissible spongiform encephalopathies (tses), or prion diseases, are rare fatal neurodegenerative diseases of humans and animals. although some tses, like scrapie in sheep, have been known to exist for centuries, bovine spongiform encephalopathy (bse) was recognized only 15 years ago. new variant creutzfeldt-jakob disease (nvcjd) of humans is probably caused by consumption of bse-infected materials. the nature of the infectious agent is not fully elucidated, but substantial evidence suggests ...200111690621
elevation of apolipoprotein e in the csf of cattle affected by bse.the cerebrospinal fluid (csf) of patients suffering from creutzfeldt-jakob disease (cjd) display two unique polypeptide chains by two-dimensional polyacrylamide gel electrophoresis (2-d page). in the absence of a well-defined ante-mortem diagnostic test for bovine spongiform encephalopathy (bse), spinal fluid samples of eight normal cows and eight cows known to carry bse by post-mortem histological analysis were investigated to verify if equivalent polypeptides were present. proteins with simila ...19979369204
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 ...19948040393
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 ...19902301106
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