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Cell Biology and Pathophysiology of α-Synuclein.

TitleCell Biology and Pathophysiology of α-Synuclein.
Publication TypeJournal Article
Year of Publication2018
AuthorsBurré J, Sharma M, Südhof TC
JournalCold Spring Harb Perspect Med
Date Published2018 03 01
Keywordsalpha-Synuclein, Animals, Humans, Mice, Mice, Transgenic, Neurons, Parkinson Disease, Synapses

α-Synuclein is an abundant neuronal protein that is highly enriched in presynaptic nerve terminals. Genetics and neuropathology studies link α-synuclein to Parkinson's disease (PD) and other neurodegenerative disorders. Accumulation of misfolded oligomers and larger aggregates of α-synuclein defines multiple neurodegenerative diseases called synucleinopathies, but the mechanisms by which α-synuclein acts in neurodegeneration are unknown. Moreover, the normal cellular function of α-synuclein remains debated. In this perspective, we review the structural characteristics of α-synuclein, its developmental expression pattern, its cellular and subcellular localization, and its function in neurons. We also discuss recent progress on secretion of α-synuclein, which may contribute to its interneuronal spread in a prion-like fashion, and describe the neurotoxic effects of α-synuclein that are thought to be responsible for its role in neurodegeneration.

Alternate JournalCold Spring Harb Perspect Med
PubMed ID28108534
PubMed Central IDPMC5519445
Grant ListP50 NS094733 / NS / NINDS NIH HHS / United States
R01 AG052505 / AG / NIA NIH HHS / United States
R01 NS095988 / NS / NINDS NIH HHS / United States