Genetic and epigenetic control of the efficiency and fidelity of cross-species prion transmission.

ABSTRACT: Self-perpetuating amyloid-based protein isoforms (prions) transmit neurodegenerative diseases in mammals and phenotypic traits in yeast. Although mechanisms that control species specificity of prion transmission are poorly understood, studies of closely related orthologues of yeast prion protein Sup35 demonstrate that cross-species prion transmission is modulated by both genetic (specific sequence elements) and epigenetic (prion variants, or 'strains') factors. Depending on the prion variant, the species barrier could be controlled at the level of either heterologous co-aggregation or conversion of the aggregate-associated heterologous protein into a prion polymer. Sequence divergence influences cross-species transmission of different prion variants in opposing ways. The ability of a heter

SUBMITTER: Chen B 

PROVIDER: S-EPMC3025758 | biostudies-literature | 2010 Jun

REPOSITORIES: biostudies-literature