Publications

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 ...	2017	28838667
high prevalence of prion protein genotype associated with resistance to chronic wasting disease in one alberta woodland caribou population.	chronic wasting disease (cwd) is a prion disease found in deer, elk and moose in north america and since recently, wild reindeer in norway. caribou are at-risk to encounter cwd in areas such as alberta, canada, where the disease spreads toward caribou habitats. cwd susceptibility is modulated by species-specific polymorphisms in the prion protein gene (prnp). we sequenced prnp of woodland caribou from 9 albertan populations. in one population (chinchaga) a significantly higher frequency of the 1 ...	2017	28350512
de novo generation of a unique cervid prion strain using protein misfolding cyclic amplification.	substantial evidence supports the hypothesis that prions are misfolded, infectious, insoluble, and protease-resistant proteins (prp(res)) devoid of instructional nucleic acid that cause transmissible spongiform encephalopathies (tses). protein misfolding cyclic amplification (pmca) has provided additional evidence that prpres acts as a template that can convert the normal cellular prion protein (prp(c)) present in uninfected normal brain homogenate (nbh) into the infectious misfolded prp(res) is ...	2017	28144628
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 ...	2017	28109330
horizontal transmission of chronic wasting disease in reindeer.	we challenged reindeer by the intracranial route with the agent of chronic wasting disease sourced from white-tailed deer, mule deer, or elk and tested for horizontal transmission to naive reindeer. reindeer were susceptible to chronic wasting disease regardless of source species. horizontal transmission occurred through direct contact or indirectly through the environment.	2016	27869594
early and non-invasive detection of chronic wasting disease prions in elk feces by real-time quaking induced conversion.	chronic wasting disease (cwd) is a fatal prion disease of wild and captive cervids in north america. prions are infectious agents composed of a misfolded version of a host-encoded protein, termed prpsc. infected cervids excrete and secrete prions, contributing to lateral transmission. geographical distribution is expanding and case numbers in wild cervids are increasing. recently, the first european cases of cwd have been reported in a wild reindeer and two moose from norway. therefore, methods ...	2016	27829062
first case of chronic wasting disease in europe in a norwegian free-ranging reindeer.	chronic wasting disease (cwd) is a fatal contagious prion disease in cervids that is enzootic in some areas in north america. the disease has been found in deer, elk and moose in the usa and canada, and in south korea following the importation of infected animals. here we report the first case of cwd in europe, in a norwegian free-ranging reindeer in southern norway. the origin of the disease is unknown. until now a low number of cervids, and among them a few reindeer, have been tested for cwd i ...	2016	27641251
caribou consumption in northern canadian communities.	chronic wasting disease (cwd) is a transmissible spongiform encephalopathy (tse) found in both farmed and wild deer, elk, and moose in the united states and canada. surveillance efforts in north america identified the geographical distribution of the disease and mechanisms underlying distribution, although the possibility of transmission to other cervids, including caribou, and noncervids, including humans, is not well understood. because of the documented importance of caribou (rangifer tarandu ...	2016	27556568
potential role of soil properties in the spread of cwd in western canada.	chronic wasting disease (cwd) is a horizontally transmissible prion disease of free ranging deer, elk and moose. recent experimental transmission studies indicate caribou are also susceptible to the disease. cwd is present in southeast alberta and southern saskatchewan. this cwd-endemic region is expanding, threatening manitoba and areas of northern alberta and saskatchewan, home to caribou. soil can serve as a stable reservoir for infectious prion proteins; prions bound to soil particles remain ...	2016	24618673
experimental oral transmission of chronic wasting disease to reindeer (rangifer tarandus tarandus).	chronic wasting disease (cwd), a transmissible spongiform encephalopathy of cervids, remains prevalent in north american elk, white-tailed deer and mule deer. a natural case of cwd in reindeer (rangifer tarandus tarandus) has not been reported despite potential habitat overlap with cwd-infected deer or elk herds. this study investigates the experimental transmission of cwd from elk or white-tailed deer to reindeer by the oral route of inoculation. ante-mortem testing of the three reindeer expose ...	2012	22723928
the role of genetics in chronic wasting disease of north american cervids.	chronic wasting disease (cwd) is a major concern for the management of north american cervid populations. this fatal prion disease has led to declines in populations which have high cwd prevalence and areas with both high and low infection rates have experienced economic losses in wildlife recreation and fears of potential spill-over into livestock or humans. research from human and veterinary medicine has established that the prion protein gene (prnp) encodes the protein responsible for transmi ...	2012	22460693
prion protein genes in caribou from alaska.	prion protein genes were sequenced in free-ranging alaska caribou (rangifer tarandus grantii). caribou prion alleles are identical or nearly so to those of wapiti, white-tailed deer, and mule deer. five single-nucleotide polymorphisms were detected with substitutions at residues 2 (v-->m), 129 (g-->s), 138 (s-->n), 146 (n-->n), and 169 (v-->m). the 138n codon had been previously reported only in prion pseudogenes of other cervids. in caribou, the 138s and 138n alleles are present at frequencies ...	2007	17495306
screening for chronic wasting disease in caribou in northern quebec.		2002	12497968