Effects of a naturally occurring amino acid substitution in bovine PrP: a model for inherited prion disease in a natural host species.

ABSTRACT:

Objective

The most common hereditary prion disease is human Creutzfeldt-Jakob disease (CJD), associated with a mutation in the prion gene resulting in a glutamic acid to lysine substitution at position 200 (E200K) in the prion protein. Models of E200K CJD in transgenic mice have proven interesting but have limitations including inconsistencies in disease presentation, requirement for mixed species chimeric protein constructs, and the relatively short life span and time to disease onset in rodents. These factors limit research on the mechanism by which the mutation drives disease development. Therefore, our objective was to provide the first assessment of cattle carrying the homologous mutation, E211K, as a system for investigating longer-term disease mechanisms. The E211K substitut

SUBMITTER: Vrentas CE 

PROVIDER: S-EPMC5738711 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature