Further Characterization of Glycoform-Selective Prions of Variably Protease-Sensitive Prionopathy.

ABSTRACT: Prion is an infectious protein (PrP^Sc) that is derived from a cellular glycoprotein (PrP^C) through a conformational transition and associated with a group of prion diseases in animals and humans. Characterization of proteinase K (PK)-resistant PrP^Sc by western blotting has been critical to diagnosis and understanding of prion diseases including Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker (GSS) disease in humans. However, formation as well as biochemical and biological properties of the glycoform-selective PrP^Sc in variably protease-sensitive prionopathy (VPSPr) remain poorly understood. Here we reveal that formation of the ladder-like PrP^Sc in VPSPr is a PK-dependent two-step process, which is enhanced by basic p

SUBMITTER: Zhang W 

PROVIDER: S-EPMC8146342 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature