Structural attributes of mammalian prion infectivity: Insights from studies with synthetic prions.

ABSTRACT: Prion diseases are neurodegenerative disorders that affect many mammalian species. Mammalian prion proteins (PrPs) can misfold into many different aggregates. However, only a small subpopulation of these structures is infectious. One of the major unresolved questions in prion research is identifying which specific structural features of these misfolded protein aggregates are important for prion infectivity in vivo Previously, two types of proteinase K-resistant, self-propagating aggregates were generated from the recombinant mouse prion protein in the presence of identical cofactors. Although these two aggregates appear biochemically very similar, they have dramatically different biological properties, with one of them being highly infectious and the other one lacking any infectivit

SUBMITTER: Li Q 

PROVIDER: S-EPMC6290147 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature