Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome.

ABSTRACT: Human transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia. GSS is a genetically determined TSE caused by a range of mutations within the prion protein (PrP) gene. Several animal models, based on the expression of PrPs carrying mutations analogous to human heritable prion diseases, support that mutations might predispose PrP to spontaneously misfold. An adapted Protein Misfolding Cyclic Amplification methodology based on the use of human recombinant PrP (recPMCA) generated different self-propagating misfolded proteins spontaneously. These were characterized biochemically and structurally, and the one partiall

SUBMITTER: Elezgarai SR 

PROVIDER: S-EPMC5575253 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature