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2018 ; 8
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Cell Biology and Pathophysiology of ?-Synuclein
#MMPMID28108534
Burré J
; Sharma M
; Südhof TC
Cold Spring Harb Perspect Med
2018[Mar]; 8
(3
): ä PMID28108534
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?-Synuclein is an abundant neuronal protein that is highly enriched in
presynaptic nerve terminals. Genetics and neuropathology studies link ?-synuclein
to Parkinson's disease (PD) and other neurodegenerative disorders. Accumulation
of misfolded oligomers and larger aggregates of ?-synuclein defines multiple
neurodegenerative diseases called synucleinopathies, but the mechanisms by which
?-synuclein acts in neurodegeneration are unknown. Moreover, the normal cellular
function of ?-synuclein remains debated. In this perspective, we review the
structural characteristics of ?-synuclein, its developmental expression pattern,
its cellular and subcellular localization, and its function in neurons. We also
discuss recent progress on secretion of ?-synuclein, which may contribute to its
interneuronal spread in a prion-like fashion, and describe the neurotoxic effects
of ?-synuclein that are thought to be responsible for its role in
neurodegeneration.
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