Development of a methodology for large-scale production of prions for biological and structural studies.

ABSTRACT: Prion diseases are a group of infectious neurodegenerative diseases produced by the conversion of the normal prion protein (PrP^C) into the disease-associated form (PrP^Sc). Extensive evidence indicate that the main or sole component of the infectious agent is PrP^Sc, which can replicate in affected individuals in the absence of nucleic acids. However, the mechanism of PrP^C-to-PrP^Sc conversion remains elusive, which has been attributed to the lack of sufficient structural information of infectious PrP^Sc and a reliable system to study prion replication in vitro. In this article we adapted the Protein Misfolding Cyclic Amplification (PMCA) technology for rapid and efficient generation of highly infectious prions in large-s

SUBMITTER: Concha-Marambio L 

PROVIDER: S-EPMC10449461 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature