⍺-Synuclein Structural Diversity and the Cellular Environment in ⍺-Synuclein Transmission Models and Humans.

ABSTRACT: Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA) are termed synucleinopathies, disorders that are characterized by the intracellular aggregation of the protein ɑ-synuclein. The cellular tropism of synuclein pathology in these syndromes is notably distinct since in the Lewy disorders, PD and DLB, ɑSyn forms aggregates in neurons whereas in MSA ɑSyn forms aggregates in oligodendrocytes. Studies examining ɑSyn pathology in experimental models and in human brain have now identified fibrillar ɑSyn with unique but distinct molecular signatures, suggesting that the structure of these ɑSyn fibrils might be closely tied to their cellular ontogeny. In contrast to the native structural heterogeneity of ɑSyn in vitro, the conformational landscape of fibrilla

SUBMITTER: Peelaerts W 

PROVIDER: S-EPMC10119367 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature