Alpha-synuclein-based models of Parkinson’s disease

Parkinson’s disease is a progressive neurodegenerative disorder mainly
characterized by the loss of dopaminergic neurons from the substantia nigra pars
compacta and the presence, in the affected brain regions, of protein inclusions
called ‘Lewy bodies’. Most cases are sporadic, but mutations in several genes,
including SNCA, which encodes α-synuclein, are associated with disease
development. A myriad of α-synuclein-based models for studying Parkinson’s
disease have been generated over the last two decades through different
methodologies. Collectively, these models offer new opportunities to elucidate
the mechanisms underlying the relentless progression of protein aggregation and
neurodegeneration in Parkinson’s. The present, non-exhaustive review focuses on
mammalian models and the main strategies that are currently available, including
transgenesis, viral vector gene delivery and the recently developed ‘prion-like’
models.

Copyright © 2016 Elsevier Masson SAS. All rights reserved.

 

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