Synthetic α-synuclein fibrils replicate in mice causing MSA-like pathology.

ABSTRACT: Multiple-system atrophy (MSA) is a rapidly progressive neurodegenerative disease of unknown cause, typically affecting individuals aged 50-60 years and leading to death within a decade^1-3. It is characterized by glial cytoplasmic inclusions (GCIs) composed of fibrillar α-synuclein (aSyn)^4-8, the formation of which shows parallels with prion propagation^9,10. While fibrils extracted from brains of individuals with MSA have been structurally characterized¹¹, their ability to replicate in a protein-only manner has been questioned¹², and their ability to induce GCIs in vivo remains unexplored. By contrast, the synthetic fibril strain 1B^13,14, assembled from recombinant human aSyn, self-replicates in vitro and induces GCIs in mice

SUBMITTER: Burger D 

PROVIDER: S-EPMC12695662 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature