Abstract:
:The transmissible spongiform encephalopathies (TSEs) invariably result in fatal neurodegeneration and accumulation of PrP, an abnormal form of the host prion protein PrP, 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 PrP 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 PrP folding pattern.
journal_name
Neuroreportjournal_title
Neuroreportauthors
O'Rourke KI,Spraker TR,Zhuang D,Greenlee JJ,Gidlewski TE,Hamir ANdoi
10.1097/WNR.0b013e3282f1ca2fsubject
Has Abstractpub_date
2007-12-03 00:00:00pages
1935-8issue
18eissn
0959-4965issn
1473-558Xpii
00001756-200712030-00012journal_volume
18pub_type
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