Elk with a long incubation prion disease phenotype have a unique PrPd profile.

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

Neuroreport

journal_title

Neuroreport

authors

O'Rourke KI,Spraker TR,Zhuang D,Greenlee JJ,Gidlewski TE,Hamir AN

doi

10.1097/WNR.0b013e3282f1ca2f

subject

Has Abstract

pub_date

2007-12-03 00:00:00

pages

1935-8

issue

18

eissn

0959-4965

issn

1473-558X

pii

00001756-200712030-00012

journal_volume

18

pub_type

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