clinical observations on four cases of scrapie in goats. 19921557871
[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
molecular biology and pathology of scrapie and the prion diseases of humans.scrapie and bovine spongiform encephalopathy of animals and creutzfeldt-jakob and gerstmann-sträussler-scheinker diseases of humans are transmissible and genetic neurodegenerative diseases caused by prions. infectious prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein which is encoded by a chromosomal gene. an as yet unidentified post-translational process converts the cellular prion protein into an abnormal isoform. scrapie neuropathology, incubat ...19911669719
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
experimental transmission of scrapie to cattle. 19901971338
bovine spongiform encephalopathy--a new disease transmissable to humans?current concerns about the cattle disease bovine spongiform encephalopathy do not appear to take cognisance of the parallel with scrapie, the similar/identical disease of sheep/goats. this has existed for 200 years, and clearly involved a longstanding consumption of meat/offal from affected animals, but apparently without consequential human disease. a summary is given of the characteristics of the human and animal spongiform encephalopathy diseases and their causative agents. the conclusion of ...19902233424
the genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved on reisolation in mice.263k is the most widely used strain of agent in scrapie research because it produces very short incubation periods in golden hamsters and exceptionally high infectivity titres in clinically affected brain. 263k is also remarkable in having a very low pathogenicity for mice. evidence is presented that 263k originated as a mutant that was strongly selected on passage in hamsters. seven new passage lines have been established in hamsters using well characterized strains of mouse scrapie representin ...19892504883
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
classic genetics of scrapie.many years ago, observations of natural scrapie revealed differences in clinical manifestations and in the areas of the brain that showed the most intense histopathological changes. in experimental work with scrapie in mice two fundamental points were established in early studies: (1) a mouse gene, termed sinc for scrapie incubation, affects the length of the incubation period. (2) in a single strain of mice, incubation periods of different "strains" of scrapie can differ by as much as 3-4 fold, ...19892690115
immunostaining of scrapie cerebral amyloid plaques with antisera raised to scrapie-associated fibrils (saf).brain sections from 16 different mouse scrapie models were immunostained with antisera to scrapie-associated fibrils (saf) from three experimental scrapie sources (hamster 263k, mouse me7 and mouse 22l). these models involved seven strains of scrapie injected intracerebrally or intraperitoneally into a range of inbred mouse strains, producing a wide variety of neuropathological changes. the only brain structures which were positively immunostained were amyloid plaque cores in those models in whi ...19883221978
the relative susceptibility of sheep, goats and mice to two types of the goat scrapie agent. 19664163198
[visna virus-like particles in cultures of choroid plexus cells of a goat with symptoms of visna infection]. 19744363195
further observations on the production of scrapie in sheep by oral dosing with foetal membranes from scrapie-affected sheep. 19744425935
scrapie: a prototype slow infection. 19724622954
naturally-occurring scrapie in goats. 19744859427
transmission and characterization of the agents of spongiform virus encephalopathies: kuru, creutzfeldt-jakob disease, scrapie and mink encephalopathy. 19715000225
[new observations on the occurrence of a rna-dependent dna-polymerase]. 19715102738
scrapie--eradication and field trial study of the natural disease. 19695392186
the unsuccessful dialysis of scrapie agent from goat brain and spleen. 19705498566
glycosidase histochemistry in normal and scrapie mice, rats, sheep and goats. 19685690819
scrapie agent contains a hydrophobic protein.the scrapie agent causes a degenerative nervous system disorder of sheep and goats. considerable evidence indicates that the scrapie agent contains a protein that is necessary for infectivity [prusiner, s. b., groth, d. f., cochran, s. p., masiarz, f. r., mckinley, m. p. & martinez, h. m. (1980) biochemistry 19, 4883-4891], but direct demonstration of a protein moiety has been hampered by lack of sufficiently purified preparations. employing preparations of the scrapie agent enriched 100- to 100 ...19816273882
virologic and neurohistologic findings in dairy goats affected with natural scrapie.virologic and neurohistologic findings in three dairy goats that became affected with scrapie while living with naturally infected suffolk sheep were essentially like those in affected sheep. virus, detected by mouse inoculation, was widespread in non-neural sites, particularly in lymphatic tissues and intestine. im most sites, titers of virus ranged from 3.0 to 3.5 log10 mouse intracerebral ld50/30 mg of tissue. virus was in nervous tissue in much higher titer. ranging from 5.1 to 5.8 log10, th ...19806767304
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
[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
scrapie control in the united states. 19938236771
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
biochemical typing of scrapie strains. 19979121579
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
the shortest known prion protein gene allele occurs in goats, has only three octapeptide repeats and is non-pathogenic.the prion protein (prp) gene modulates the incidence and incubation periods of transmissible spongiform encephalopathies of sheep, goats, mice and man. here, a new caprine prp allele encoding the shortest naturally occurring prp protein so far described is reported. this variant contains only three instead of the usual five copies of a short peptide repeat [pro-gln/his-gly-gly-gly-(gly)-trpgly-gln] characteristic of prp, with an additional trp to gly substitution in codon 102. fifteen out of 111 ...19989880037
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 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
astrocytosis and proliferating cell nuclear antigen expression in brains of scrapie-infected hamsters.scrapie is a neurodegenerative disease in sheep and goats. neuropathological examination shows astrocytosis. one issue is whether the astrocytosis seen in scrapie is a function of an increase in reactivity of individual cells, or whether there is actual replication of astrocytes. we used double-label immunohistochemistry for proliferating cell nuclear antigen (pcna) and for glial fibrillary acidic protein (gfap) to determine the mitotic state of cells and to confirm their identity as astrocytes. ...199810344795
a survey of more than 11 years of neurologic diseases of ruminants with special reference to transmissible spongiform encephalopathies (tses) in greece.the first cases of scrapie were detected in greece in a flock of sheep in october 1986. all the animals of the affected flock and all sheep in two flocks that were in contact were killed and buried. a systematic investigation of all available cases with signs indicating a neurological disease started in sheep and goats in late 1986, as well as in cattle in 1989. the investigation was based on clinical examination, necropsy or macroscopical examination of the brain and viscera, and histological e ...200010861199
clinical signs and diagnosis of scrapie in italy: a comparative study in sheep and goats.this article reports the clinical signs of scrapie occurring in sheep-goat flocks in italy. the research, carried out on over 500 goats and 550 sheep, yielded an interesting clinical picture, especially of goat scrapie. the affected animals, aged between 2 and 8 years, showed neurological signs that could be classified into three categories: changes in mental status, abnormalities of movement and changes in sensation. some general clinical signs were also observed. the clinical suspicion was con ...200111515309
bse: a consequence of cattle feeding with glycated molecules host-unknown?although there is much evidence supporting a prion contribution in the pathogenesis of transmissible spongiform encephalopathies, a novel point of view as to the induction of the diseases can be proposed. it is hypothesized that neurodegenerative diseases, such as scrapie in sheep and goats and bovine spongiform encephalopathy in cattle (bse), originate from the consumption of glycated proteins contained in their feed. these components are obtained during a high-temperature glycation process.200212027519
sheep/human comparative map in a chromosome region involved in scrapie incubation time shows multiple breakpoints between human chromosomes 14 and 15 and sheep chromosomes 7 and 18.a chromosome region involved in scrapie incubation time was identified on sheep chromosome 18 (oar18). since oar18 (and oar7) share conserved chromosome segments with human chromosomes hsa14 and hsa15, a dense map of type i markers was constructed by fish mapping of bacterial artificial chromosomes containing genes located on these human chromosomes. in this study, we used the complete human sequence information (gene positions in megabases, mb) to locate approximately one gene every 2 mb on hsa ...200212296519
scrapie and chronic wasting disease.scrapie and cwd share many features. there are marked similarities in the clinical presentations, the lesions, and the pathogenesis of these diseases, and some similarities in the epidemiology. extrapolation from the scrapie model of tse disease to cwd--which occurs in three different species, and should not be considered to be uniform in their response--may be erroneous, however. such differences may influence diagnostics (e.g., the amount and distribution of prpc in these different species), p ...200312733429
[cytopathological changes in human and animal brain in prion diseases].the study of brain histological sections of humans and animals afflicted by prion diseases has shown that neuronal vacuolization began in and intensely spread from the distal portions of dendrites. two types of neuronal cell body death were demonstrated, including cytolysis and pyknosis, the latter being usually associated with dendrite vacuolization. the involvement of major glial types in the pathogenesis of prion diseases was established, which argues against the concept of astrocytosis in fa ...200312741105
risk management of the transmissible spongiform encephalopathies in north north american free trade agreement partners, canada, the united states of america (usa) and mexico apply independent but harmonised transmissible spongiform encephalopathy (tse) risk management strategies in observance of office international des epizooties guidelines. the divergence between bovine spongiform encephalopathy (bse) risk management approaches in north american and europe reflects comparatively reduced external and internal bse risks in north america. the external quarantine and ...200312793780
transmission by contact of scrapie in mice. 196414198336
distribution of the scrapie agent in the tissues of experimentally inoculated goats. 196214484423
evaluation of rapid tests for the diagnosis of transmissible spongiform encephalopathies in sheep and accordance with eu regulation 999/2001, rapid tests already adopted for bovine spongiform encephalopathy (bse; prionics check western, platelia-bse and enfer tse) are to be applied in all european countries to a sub-population of over 18-month-old slaughtered or dead sheep and goats to improve scrapie surveillance and to determine the possible presence of bse in sheep; however, the three tests have thus far been evaluated only for bse and no official data are available about their performance ...200415067554
experimental transmission of sheep scrapie by intracerebral and oral routes to genetically susceptible suffolk sheep in the united states.scrapie is a naturally occurring fatal neurodegenerative disease of sheep and goats. susceptibility to the disease is partly dependent on the genetic makeup of the host. this study documents clinicopathological findings and the distribution of abnormal prion proteins (prpres) by immunohistochemical and western blot techniques, in tissues of genetically susceptible sheep inoculated with us sheep scrapie agents. four-month-old suffolk lambs (qq or hq at codon 171) were inoculated (5 intracerebrall ...200515690944
[swiss scrapie surveillance. i. clinical aspects of neurological diseases in sheep and goats].small ruminants infected with scrapie show a large range of often unspecific clinical symptoms. the most-often described signs, locomotion, sensibility and behavioural disorders and emaciation, rarely occur together, and cases have been described in which only one of those signs was detectable.thus, formulating a well-circumscribed definition of a clinical suspect case is difficult. most animals with cns-effecting diseases such as listeriosis, polioencephalomacia, cerebrospinal nematidiasis and ...200516259408
comparison of immunohistochemistry and two rapid tests for detection of abnormal prion protein in different brain regions of sheep with typical of the "gold standard" techniques for postmortem confirmation of scrapie diagnosis in sheep and goats is immunohistochemical examination of brain tissue. active surveillance for scrapie is mainly performed by rapid diagnostic tests on the basis of postmortem immunochemical detection of prion protein (prp) in the obex tissue. the aim of this study was to determine the performance of 2 rapid tests, prionics-check lia (a chemiluminescence sandwich enzyme-linked immunosorbent assay) and prionics ...200516312240
identification of an allelic variant of the goat prp gene associated with resistance to scrapie.the association between prp gene variations and scrapie susceptibility was studied in a single herd of ionica breed goats. the entire herd comprised 100 animals, 11 of which were clinically affected and showed pathological prion protein (prp(sc)) deposition in both their central nervous system (cns) and lymphoreticular system (lrs). among asymptomatic goats, nine harboured prp(sc) in both cns and lrs, 19 showed prp(sc) only at the lrs level and 61 animals had no prp(sc) deposition. genetic analy ...200616603543
histopathological and immunohistochemical features of natural goat scrapie.histopathological and immunohistochemical examinations were performed on the brain and spinal cord of 37 goats from two greek herds in which scrapie had been reported. of the 37 animals, 18 were from a herd consisting only of goats and 19 were from a herd of goats mixed with sheep. the goats studied were grouped on the basis of the presence or absence of clinical signs. distinctive lesions and prp(sc) (prp, prion protein) deposition were found in the central nervous system (cns) of eight clinica ...200616997004
il-1 family members as candidate genes modulating scrapie susceptibility in sheep: localization, partial characterization, and expression.scrapie (sc) is a transmissible spongiform encephalopathy (tse) in sheep and goats. susceptibility to this neurodegenerative disease is controlled mainly by point mutations at the prnp locus. other genes, apart from prnp, have been reported to modulate resistance/susceptibility to sc. on the basis of several studies on alzheimer's disease and different tse models, and of requirement for correct homeostasis of cytokines in brain, il1b and il1rn were chosen as putative positional and functional ca ...200717242860
atypical scrapie in a swiss goat and implications for transmissible spongiform encephalopathy surveillance.different types of transmissible spongiform encephalopathies (tses) affect sheep and goats. in addition to the classical form of scrapie, both species are susceptible to experimental infections with the bovine spongiform encephalopathy (bse) agent, and in recent years atypical scrapie cases have been reported in sheep from different european countries. atypical scrapie in sheep is characterized by distinct histopathologic lesions and molecular characteristics of the abnormal scrapie prion protei ...200717459826
scrapie in goats. 200717873273
rapid typing of transmissible spongiform encephalopathy strains with differential elisa.the bovine spongiform encephalopathy (bse) agent has been transmitted to humans, leading to variant creutzfeldt-jakob disease. sheep and goats can be experimentally infected by bse and have been potentially exposed to natural bse; however, whether bse can be transmitted to small ruminants is not known. based on the particular biochemical properties of the abnormal prion protein (prpsc) associated with bse, and particularly the increased degradation induced by proteinase k in the n terminal part ...200818394279
serial passage of sheep scrapie inoculum in suffolk sheep.scrapie is a naturally occurring fatal neurodegenerative disease of sheep and goats. susceptibility to the disease is partly dependent upon the genetic makeup of the host. in a recent study, it was shown that sheep intracerebrally inoculated with a us scrapie agent (no. 13-7) developed scrapie and survived for an average of 19 months post inoculation. in the present study, when this scrapie inoculum was further passaged for 3 successive generations, the survival time was reduced by approximately ...200919112113
field performance of two rapid screening tests in active surveillance of transmissible spongiform encephalopathies in small ruminants.recently, screening tests for monitoring the prevalence of transmissible spongiform encephalopathies specifically in sheep and goats became available. although most countries require comprehensive test validation prior to approval, little is known about their performance under normal operating conditions. switzerland was one of the first countries to implement 2 of these tests, an enzyme-linked immunosorbent assay (elisa) and a western blot, in a 1-year active surveillance program. slaughtered a ...200919139507
identification of seven haplotypes of the caprine prp gene at codons 127, 142, 154, 211, 222 and 240 in french alpine and saanen breeds and their association with classical sheep, susceptibility to scrapie is mainly influenced by polymorphisms of the prp gene. in goats, there are to date few data related to scrapie susceptibility association with prp gene polymorphisms. in this study, we first investigated prp gene polymorphisms of the french alpine and saanen breeds. based on prp gene open reading frame sequencing of artificial insemination bucks (n=404), six encoding mutations were identified at codons 127, 142, 154, 211, 222 and 240. however, only seven haplo ...200919218225
prnp haplotype distribution in moroccan goats.susceptibility/resistance to scrapie in sheep and goats is influenced by host prion protein gene (prnp) genotype. in this study, we report the analysis of prion protein gene polymorphisms in 137 goats of two moroccan populations: d'man and chaouni. we found seven previously described amino acid polymorphisms at codons 37, 127, 137, 142, 154, 222 and 240, as well as three known silent mutations. in addition, we identified three new allelic variants: 101r and 139s in d'man goats and 145d in d'man ...200919397523
characterization of a us sheep scrapie isolate with short incubation time.scrapie is a naturally occurring fatal neurodegenerative disease of sheep and goats. susceptibility to the disease is partly dependent upon the genetic makeup of the host. in a previous study it was shown that sheep intracerebrally inoculated with us scrapie inoculum (no. 13-7) developed terminal disease within an average of 19 months. we have since produced an inoculum, no. x124 from pooled brains of us-origin sheep scrapie, that results in incubations nearly threefold shorter. the present stud ...200919605918
identification of atypical scrapie in canadian sheep.scrapie, a transmissible spongiform encephalopathy of sheep and goats, exists in most small ruminant-producing countries of the world. a novel form of this disease was recently recognized and is known by various names, including nor98, nor98-like, and atypical scrapie. differing from classic scrapie in epidemiology, histopathology, and biochemical characteristics, atypical scrapie cases have been identified throughout europe and in the united states. enhanced scrapie surveillance efforts recentl ...201020453215
prpsc spreading patterns in the brain of sheep linked to different prion types.abstract: scrapie in sheep and goats has been known for more than 250 years and belongs nowadays to the so-called prion diseases that also include e.g. bovine spongiform encephalopathy in cattle (bse) and creutzfeldt-jakob disease in humans. according to the prion hypothesis, the pathological isoform (prpsc) of the cellular prion protein (prpc) comprises the essential, if not exclusive, component of the transmissible agent. currently, two types of scrapie disease are known - classical and atypic ...201121324114
molecular discrimination of sheep bovine spongiform encephalopathy from scrapie.sheep ch1641-like transmissible spongiform encephalopathy isolates have shown molecular similarities to bovine spongiform encephalopathy (bse) isolates. we report that the prion protein prpsc from sheep bse is extremely resistant to denaturation. this feature, combined with the n-terminal prpsc cleavage, allowed differentiation of classical scrapie, including ch1641-like, from natural goat bse and experimental sheep bse.201121470463
experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle.prion diseases or transmissible spongiform encephalopathies (tses) of animals include scrapie of sheep and goats; transmissible mink encephalopathy (tme); chronic wasting disease (cwd) of deer, elk and moose; and bovine spongiform encephalopathy (bse) of cattle. the emergence of bse and its spread to human beings in the form of variant creutzfeldt-jakob disease (vcjd) resulted in interest in susceptibility of cattle to cwd, tme and scrapie. experimental cross-species transmission of tse agents p ...201121908269
immunophenotype of cells within cervine rectoanal mucosa-associated lymphoid tissue and mesenteric lymph nodes.rectoanal mucosa-associated lymphoid tissue (ramalt) is a part of the lymphoid system that can be sampled easily in live animals, especially ruminants. ramalt biopsy is useful for the diagnosis of transmissible spongiform encephalopathies, including scrapie in sheep and goats and chronic wasting disease (cwd) in cervids. diagnosis is reliant on detection of abnormal prion protein (prp(d)), which is associated with lymphoid follicles. for enzyme linked immunosorbent assays (elisas) detecting prp( ...201122000034
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