Strain specific resistance to murine scrapie associated with a naturally occurring human prion protein polymorphism at residue 171.

ABSTRACT: Transmissible spongiform encephalopathies (TSE) or prion diseases are neurodegenerative disorders associated with conversion of normal host prion protein (PrP) to a misfolded, protease-resistant form (PrPres). Genetic variations of prion protein in humans and animals can alter susceptibility to both familial and infectious prion diseases. The N171S PrP polymorphism is found mainly in humans of African descent, but its low incidence has precluded study of its possible influence on prion disease. Similar to previous experiments of others, for laboratory studies we created a transgenic model expressing the mouse PrP homolog, PrP-170S, of human PrP-171S. Since PrP polymorphisms can vary in their effects on different TSE diseases, we tested these mice with four different strains of mouse-adapte

SUBMITTER: Striebel JF 

PROVIDER: S-EPMC3182929 | biostudies-literature | 2011 Sep

REPOSITORIES: biostudies-literature