MSA prions exhibit remarkable stability and resistance to inactivation.

ABSTRACT: In multiple system atrophy (MSA), progressive neurodegeneration results from the protein α-synuclein misfolding into a self-templating prion conformation that spreads throughout the brain. MSA prions are transmissible to transgenic (Tg) mice expressing mutated human α-synuclein (TgM83^+/-), inducing neurological disease following intracranial inoculation with brain homogenate from deceased patient samples. Noting the similarities between α-synuclein prions and PrP scrapie (PrP^Sc) prions responsible for Creutzfeldt-Jakob disease (CJD), we investigated MSA transmission under conditions known to result in PrP^Sc transmission. When peripherally exposed to MSA via the peritoneal cavity, hind leg muscle, and tongue, TgM83^+/- mice developed neurological s

SUBMITTER: Woerman AL 

PROVIDER: S-EPMC5756500 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature