Publications

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the structure of the infectious prion protein and its propagation.the prion diseases, which include creutzfeldt-jakob disease in humans, chronic wasting disease in cervids (i.e., deer, elk, moose, and reindeer), bovine spongiform encephalopathy in cattle, as well as sheep and goat scrapie, are caused by the conversion of the cellular prion protein (prp(c)) into a disease-causing conformer (prp(sc)). prp(c) is a regular, gpi-anchored protein that is expressed on the cell surface of neurons and many other cell types. the structure of prp(c) is well studied, base ...201728838667
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
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
the structure of mammalian prions and their aggregates.prion diseases, such as creutzfeldt-jakob disease in humans, bovine spongiform encephalopathy in cattle, chronic wasting disease in cervids (i.e., deer, elk, moose, and reindeer), and sheep scrapie, are caused by the misfolding of the cellular prion protein (prp(c)) into a disease-causing conformer (prp(sc)). prp(c) is a normal, gpi-anchored protein that is expressed on the surface of neurons and other cell types. the structure of prp(c) is well understood, based on studies of recombinant prp, w ...201728109330
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
chronic wasting disease.chronic wasting disease (cwd) is a prion disease of free-ranging and farmed ungulates (deer, elk, and moose) in north america and south korea. first described by the late e.s. williams and colleagues in northern colorado and southern wyoming in the 1970s, cwd has increased tremendously both in numerical and geographical distribution, reaching prevalence rates as high as 50% in free-ranging and >90% in captive deer herds in certain areas of usa and canada. cwd is certainly the most contagious pri ...201121598099
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