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α-突触核蛋白在帕金森病中的作用。

α-Synuclein in Parkinson's disease.

机构信息

Laboratory of Neurodegenerative Diseases, Biomedical Research Foundation of the Academy of Athens, and Second Department of Neurology, University of Athens Medical School, Athens 11527, Greece.

出版信息

Cold Spring Harb Perspect Med. 2012 Feb;2(2):a009399. doi: 10.1101/cshperspect.a009399.

Abstract

α-Synuclein is a presynaptic neuronal protein that is linked genetically and neuropathologically to Parkinson's disease (PD). α-Synuclein may contribute to PD pathogenesis in a number of ways, but it is generally thought that its aberrant soluble oligomeric conformations, termed protofibrils, are the toxic species that mediate disruption of cellular homeostasis and neuronal death, through effects on various intracellular targets, including synaptic function. Furthermore, secreted α-synuclein may exert deleterious effects on neighboring cells, including seeding of aggregation, thus possibly contributing to disease propagation. Although the extent to which α-synuclein is involved in all cases of PD is not clear, targeting the toxic functions conferred by this protein when it is dysregulated may lead to novel therapeutic strategies not only in PD, but also in other neurodegenerative conditions, termed synucleinopathies.

摘要

α-突触核蛋白是一种突触前神经元蛋白,它在遗传和神经病理学上与帕金森病(PD)有关。α-突触核蛋白可能通过多种方式导致 PD 的发病机制,但一般认为,其异常可溶性寡聚构象,称为原纤维,是介导细胞内稳态破坏和神经元死亡的毒性物质,通过对各种细胞内靶点的影响,包括突触功能。此外,分泌的α-突触核蛋白可能对邻近细胞产生有害影响,包括聚集的传播,从而可能导致疾病的传播。虽然 α-突触核蛋白在所有 PD 病例中的参与程度尚不清楚,但当这种蛋白质失调时,针对其毒性功能可能会导致新的治疗策略,不仅在 PD 中,而且在其他神经退行性疾病中,也称为突触核蛋白病。

相似文献

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α-Synuclein in Parkinson's disease.α-突触核蛋白在帕金森病中的作用。
Cold Spring Harb Perspect Med. 2012 Feb;2(2):a009399. doi: 10.1101/cshperspect.a009399.

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In vivo demonstration that alpha-synuclein oligomers are toxic.体内实验证明α-突触核蛋白寡聚体具有毒性。
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