Understanding the key features of the spontaneous formation of bona fide prions through a novel methodology that enables their swift and consistent generation.

ABSTRACT: Among transmissible spongiform encephalopathies or prion diseases affecting humans, sporadic forms such as sporadic Creutzfeldt-Jakob disease are the vast majority. Unlike genetic or acquired forms of the disease, these idiopathic forms occur seemingly due to a random event of spontaneous misfolding of the cellular PrP (PrP^C) into the pathogenic isoform (PrP^Sc). Currently, the molecular mechanisms that trigger and drive this event, which occurs in approximately one individual per million each year, remain completely unknown. Modelling this phenomenon in experimental settings is highly challenging due to its sporadic and rare occurrence. Previous attempts to model spontaneous prion misfolding in vitro have not been fully successful, as the spontaneous formation of prio

SUBMITTER: Erana H 

PROVIDER: S-EPMC10486007 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature