A Protein Misfolding Shaking Amplification-based method for the spontaneous generation of hundreds of bona fide prions.

ABSTRACT: Prion diseases are a group of rapidly progressing neurodegenerative disorders caused by the misfolding of the endogenous prion protein (PrP^C) into a pathogenic form (PrP^Sc). This process, despite being the central event underlying these disorders, remains largely unknown at a molecular level, precluding the prediction of new potential outbreaks or interspecies transmission incidents. In this work, we present a method to generate bona fide recombinant prions de novo, allowing a comprehensive analysis of protein misfolding across a wide range of prion proteins from mammalian species. We study more than 380 different prion proteins from mammals and classify them according to their spontaneous misfolding propensity and their conformational variability. This study aims to

SUBMITTER: Erana H 

PROVIDER: S-EPMC10923866 | biostudies-literature | 2024 Mar

REPOSITORIES: biostudies-literature