Disease-, region- and cell type specific diversity of α-synuclein carboxy terminal truncations in synucleinopathies.

ABSTRACT: Synucleinopathies, including Parkinson's disease (PD), Lewy body dementia (LBD), Alzheimer's disease with amygdala restricted Lewy bodies (AD/ALB), and multiple system atrophy (MSA) comprise a spectrum of neurodegenerative disorders characterized by the presence of distinct pathological α-synuclein (αSyn) inclusions. Experimental and pathological studies support the notion that αSyn aggregates contribute to cellular demise and dysfunction with disease progression associated with a prion-like spread of αSyn aggregates via conformational templating. The initiating event(s) and factors that contribute to diverse forms of synucleinopathies remain poorly understood. A major post-translational modification of αSyn associated with pathological inclusions is a diverse array of specific truncations

SUBMITTER: Hass EW 

PROVIDER: S-EPMC8403399 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature