Multiple system atrophy: α-Synuclein strains at the neuron-oligodendrocyte crossroad.

ABSTRACT: The aberrant accumulation of α-Synuclein within oligodendrocytes is an enigmatic, pathological feature specific to Multiple system atrophy (MSA). Since the characterization of the disease in 1969, decades of research have focused on unravelling the pathogenic processes that lead to the formation of oligodendroglial cytoplasmic inclusions. The discovery of aggregated α-Synuclein (α-Syn) being the primary constituent of glial cytoplasmic inclusions has spurred several lines of research investigating the relationship between the pathogenic accumulation of the protein and oligodendrocytes. Recent developments have identified the ability of α-Syn to form conformationally distinct "strains" with varying behavioral characteristics and toxicities. Such "strains" are potentially disease-specific, p

SUBMITTER: Reddy K 

PROVIDER: S-EPMC9701437 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature