the first canadian indigenous case of bovine spongiform encephalopathy (bse) has molecular characteristics for prion protein that are similar to those of bse in the united kingdom but differ from those of chronic wasting disease in captive elk and deer.brain tissue from a case of bovine spongiform encephalopathy (bse) from alberta was subjected to a western immunoblotting technique to ascertain the molecular profile of any disease-specific, abnormal prion protein, that is, prion protein that is protease-resistant (prp(res)). this technique can discriminate between isolates from bse, ovine scrapie, and sheep experimentally infected with bse. isolates of brain tissue from the bse case in alberta, 3 farmed elk with chronic wasting disease (cwd) f ...200415532881
prion gene sequence variation within diverse groups of u.s. sheep, beef cattle, and deer.prions are proteins that play a central role in transmissible spongiform encephalopathies in a variety of mammals. among the most notable prion disorders in ungulates are scrapie in sheep, bovine spongiform encephalopathy in cattle, and chronic wasting disease in deer. single nucleotide polymorphisms in the sheep prion gene ( prnp) have been correlated with susceptibility to natural scrapie in some populations. similar correlations have not been reported in cattle or deer; however, characterizat ...200314722726
rapid detection of cwd prp: comparison of tests designed for the detection of bse or scrapie.chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse) mainly affecting cervids in north america. the accumulation of an abnormal form of host-encoded prion protein (prp(cwd) ) in the cns and lymphoid tissues is characteristic of the disease and known to be caused by pathogenic prion proteins (prp(res) ), which are thought to be transmitted mainly by contact with body fluids, such like saliva. species known to be naturally infected by cwd include rocky mountain elk (cer ...201122212828
chronic wasting disease.chronic wasting disease (cwd) is a unique transmissible spongiform encephalopathy (tse) of mule deer (odocoileus hemionus), white-tailed deer (o. virginianus), and rocky mountain elk (cervus elaphus nelsoni). the natural history of cwd is incompletely understood, but it differs from scrapie and bovine spongiform encephalopathy (bse) by virtue of its occurrence in nondomestic and free-ranging species. cwd has many features in common with scrapie, including early widespread distribution of disease ...200516145200
chronic wasting disease: fingerprinting the culprit in risk assessments.transmissible spongiform encephalopathies (prion diseases) in animals may be associated with a zoonotic risk potential for humans as shown by the occurrence of variant creutzfeldt-jakob disease in the wake of the bovine spongiform encephalopathy epidemic. thus, the increasing exposure of humans in north america to cervid prions of chronic wasting disease (cwd) in elk and deer has prompted comprehensive risk assessments. the susceptibility of humans to cwd infections is currently under investigat ...201522453172
molecular mechanisms of chronic wasting disease prion propagation.prion disease epidemics, which have been unpredictable recurrences, are of significant concern for animal and human health. examples include kuru, once the leading cause of death among the fore people in papua new guinea and caused by mortuary feasting; bovine spongiform encephalopathy (bse) and its subsequent transmission to humans in the form of variant creutzfeldt-jakob disease (vcjd), and repeated examples of large-scale prion disease epidemics in animals caused by contaminated vaccines. the ...201728193766
mother to offspring transmission of chronic wasting disease in reeves' muntjac deer.the horizontal transmission of prion diseases has been well characterized in bovine spongiform encephalopathy (bse), chronic wasting disease (cwd) of deer and elk and scrapie of sheep, and has been regarded as the primary mode of transmission. few studies have monitored the possibility of vertical transmission occurring within an infected mother during pregnancy. to study the potential for and pathway of vertical transmission of cwd in the native cervid species, we used a small cervid model-the ...201323977159
mucosal immunization with an attenuated salmonella vaccine partially protects white-tailed deer from chronic wasting disease.prion disease is a unique category of illness, affecting both animals and humans, in which the underlying pathogenesis is related to a conformational change of a normal, self-protein called prp(c) (c for cellular) to a pathological and infectious conformer known as prp(sc) (sc for scrapie). bovine spongiform encephalopathy (bse), a prion disease believed to have arisen from feeding cattle with prion contaminated meat and bone meal products, crossed the species barrier to infect humans. chronic w ...201525539804
insights into chronic wasting disease and bovine spongiform encephalopathy species barriers by use of real-time conversion.the propensity for transspecies prion transmission is related to the structural characteristics of the enciphering and new host prp, although the exact mechanism remains incompletely understood. the effects of variability in prion protein on cross-species prion transmission have been studied with animal bioassays, but the influence of prion protein structure versus that of host cofactors (e.g., cellular constituents, trafficking, and innate immune interactions) remains difficult to dissect. to i ...201526157118
assessment of the prpc amino-terminal domain in prion species barriers.chronic wasting disease (cwd) in cervids and bovine spongiform encephalopathy (bse) in cattle are prion diseases that are caused by the same protein-misfolding mechanism, but they appear to pose different risks to humans. we are interested in understanding the differences between the species barriers of cwd and bse. we used real-time, quaking-induced conversion (rt-quic) to model the central molecular event in prion disease, the templated misfolding of the normal prion protein, prp(c), to a path ...201627654299
susceptibility of cattle to first-passage intracerebral inoculation with chronic wasting disease agent from white-tailed deer.fourteen, 3-month-old calves were intracerebrally inoculated with the agent of chronic wasting disease (cwd) from white-tailed deer (cwdwtd) to compare the clinical signs and neuropathologic findings with those of certain other transmissible spongiform encephalopathies (tse, prion diseases) that have been shown to be experimentally transmissible to cattle (sheep scrapie, cwd of mule deer [cwdmd], bovine spongiform encephalopathy [bse], and transmissible mink encephalopathy). two uninoculated cal ...200717606510
chronic wasting disease of cervids.chronic wasting disease (cwd) has recently emerged in north america as an important prion disease of captive and free-ranging cervids (species in the deer family). cwd is the only recognized transmissible spongiform encephalopathy (tse) affecting free-ranging species. three cervid species, mule deer (odocoileus hemionus), white-tailed deer (o. virginianus), and rocky mountain elk (cervus elaphus nelsoni), are the only known natural hosts of cwd. endemic cwd is well established in southern wyomin ...200415148993
chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein.chronic wasting disease (cwd) is a prion disease that affects free-ranging and captive cervids, including mule deer, white-tailed deer, rocky mountain elk and moose. cwd-infected cervids have been reported in 14 usa states, two canadian provinces and in south korea. the possibility of a zoonotic transmission of cwd prions via diet is of particular concern in north america where hunting of cervids is a popular sport. to investigate the potential public health risks posed by cwd prions, we have in ...201020610667
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