Lewy-MSA hybrid fold drives distinct neuronal α-synuclein pathology.

ABSTRACT: The ordered assembly of α-synuclein protein into filaments encoded by SNCA characterizes neurodegenerative diseases called synucleinopathies. Lewy body disease (LBD) shows predominantly neuronal α-synuclein pathology and multiple system atrophy (MSA) predominantly oligodendrocytic α-synuclein pathology affecting subcortical brain structures. Based on cryo-electron microscopy, it was reported that structures of α-synuclein filaments from LBD differ from MSA and juvenile onset synucleinopathy (JOS) caused by a 21-nucleotide duplication in the second exon of one allele of SNCA gene^1-3. Importantly, a rare subtype of MSA, called atypical MSA⁴ shows abundant neuronal argyrophilic α-synuclein inclusions in the limbic system. Current concepts indicate that disease ent

SUBMITTER: Enomoto M 

PROVIDER: S-EPMC11601602 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature