Divergent prion strain evolution driven by PrP^C expression level in transgenic mice.

ABSTRACT: Prions induce a fatal neurodegenerative disease in infected host brain based on the refolding and aggregation of the host-encoded prion protein PrP^C into PrP^Sc. Structurally distinct PrP^Sc conformers can give rise to multiple prion strains. Constrained interactions between PrP^C and different PrP^Sc strains can in turn lead to certain PrP^Sc (sub)populations being selected for cross-species transmission, or even produce mutation-like events. By contrast, prion strains are generally conserved when transmitted within the same species, or to transgenic mice expressing homologous PrP^C. Here, we compare the strain properties of a representative sheep scrapie isolate transmitted to a panel of transgenic mouse lines express

SUBMITTER: Le Dur A 

PROVIDER: S-EPMC5264111 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature