Alpha-synuclein-based models of Parkinson's disease

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• 作者：B. Dehay^a ; ^b ; ^{benjamin.dehay@u-bordeaux.fr" class="auth_mail" title="E-mail the corresponding author} ; P.-O. Fernagut^a ; ^b
• 关键词：Alpha-synuclein ; Parkinson ; Animal models ; Neurodegeneration ; Synucleinopathy
• 刊名：Revue Neurologique
• 出版年：2016
• 出版时间：June-July 2016
• 年：2016
• 卷：172
• 期：6-7
• 页码：371-378
• 全文大小：425 K

文摘

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.