Journal
NEUROREPORT
Volume 18, Issue 18, Pages 1935-1938Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/WNR.0b013e3282f1ca2f
Keywords
cervid; chronic wasting disease; Creutzfeldt-Jakob disease; glycoform profile; prion protein; Rocky Mountain elk; Western blot
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The transmissible spongiform encephalopathies (TSEs) invariably result in fatal neurodegeneration and accumulation of PrPd, an abnormal form of the host prion protein PrPc, encoded by the PRNP gene. A naturally occurring polymorphism (methionine/valine) at PRNP codon 129 is associated with variation in relative disease susceptibility, incubation time, clinical presentation, neuropathology, and/or PrPd biochemical characteristics in a range of human TSEs. A methionine/leucine polymorphism at the corresponding site in the Rocky Mountain elk PRNP gene is associated with variation in relative susceptibility and incubation time in the cervid TSE chronic wasting disease. We now report that elk lacking the predisposing 132-methionine allele develop chronic wasting disease after a long incubation period and display a novel PrPd folding pattern.
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