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α-突触核蛋白改变果蝇中突变型亨廷顿蛋白的聚集及神经毒性。

α-Synuclein modifies mutant huntingtin aggregation and neurotoxicity in Drosophila.

作者信息

Poças Gonçalo M, Branco-Santos Joana, Herrera Federico, Outeiro Tiago Fleming, Domingos Pedro M

机构信息

Instituto de Tecnologia Química e Biológica, Universidade Nova de Lisboa, Av. da República, Oeiras 2780-157, Portugal.

Instituto de Tecnologia Química e Biológica, Universidade Nova de Lisboa, Av. da República, Oeiras 2780-157, Portugal, Cell and Molecular Neuroscience Unit, Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Av. Prof. Egas Moniz, Lisboa 1649-029, Portugal.

出版信息

Hum Mol Genet. 2015 Apr 1;24(7):1898-907. doi: 10.1093/hmg/ddu606. Epub 2014 Dec 1.

Abstract

Protein misfolding and aggregation is a major hallmark of neurodegenerative disorders such as Alzheimer's disease (AD), Parkinson's disease (PD) and Huntington's disease (HD). Until recently, the consensus was that each aggregation-prone protein was characteristic of each disorder [α-synuclein (α-syn)/PD, mutant huntingtin (Htt)/HD, Tau and amyloid beta peptide/AD]. However, growing evidence indicates that aggregation-prone proteins can actually co-aggregate and modify each other's behavior and toxicity, suggesting that this process may also contribute to the overlap in clinical symptoms across different diseases. Here, we show that α-syn and mutant Htt co-aggregate in vivo when co-expressed in Drosophila and produce a synergistic age-dependent increase in neurotoxicity associated to a decline in motor function and life span. Altogether, our results suggest that the co-existence of α-syn and Htt in the same neuronal cells worsens aggregation-related neuropathologies and accelerates disease progression.

摘要

蛋白质错误折叠和聚集是神经退行性疾病(如阿尔茨海默病(AD)、帕金森病(PD)和亨廷顿舞蹈病(HD))的主要标志。直到最近,人们的共识是,每种易于聚集的蛋白质都是每种疾病的特征[α-突触核蛋白(α-syn)/PD、突变型亨廷顿蛋白(Htt)/HD、 Tau蛋白和淀粉样β肽/AD]。然而,越来越多的证据表明,易于聚集的蛋白质实际上可以共同聚集并相互改变行为和毒性,这表明这个过程可能也导致了不同疾病临床症状的重叠。在这里,我们表明,当在果蝇中共表达时,α-syn和突变型Htt在体内共同聚集,并产生与运动功能和寿命下降相关的协同年龄依赖性神经毒性增加。总之,我们的结果表明,α-syn和Htt在同一神经元细胞中的共存会加剧与聚集相关的神经病理学,并加速疾病进展。

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