Small ruminant nor98 prions share biochemical features with human gerstmann-straussler-scheinker disease and variably protease-sensitive prionopathy.

ABSTRACT: Prion diseases are classically characterized by the accumulation of pathological prion protein (PrP(Sc)) with the protease resistant C-terminal fragment (PrP(res)) of 27-30 kDa. However, in both humans and animals, prion diseases with atypical biochemical features, characterized by PK-resistant PrP internal fragments (PrP(res)) cleaved at both the N and C termini, have been described. In this study we performed a detailed comparison of the biochemical features of PrP(Sc) from atypical prion diseases including human Gerstmann-Sträussler-Scheinker disease (GSS) and variably protease-sensitive prionopathy (VPSPr) and in small ruminant Nor98 or atypical scrapie. The kinetics of PrP(res) production and its cleavage sites after PK digestion were analyzed, along with the PrP(Sc) conformational st

SUBMITTER: Pirisinu L 

PROVIDER: S-EPMC3691246 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature