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神经退行性变中的α-突触核蛋白毒性:机制与治疗策略

α-synuclein toxicity in neurodegeneration: mechanism and therapeutic strategies.

作者信息

Wong Yvette C, Krainc Dimitri

机构信息

Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

出版信息

Nat Med. 2017 Feb 7;23(2):1-13. doi: 10.1038/nm.4269.

Abstract

Alterations in α-synuclein dosage lead to familial Parkinson's disease (PD), and its accumulation results in synucleinopathies that include PD, dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). Furthermore, α-synuclein contributes to the fibrilization of amyloid-b and tau, two key proteins in Alzheimer's disease, which suggests a central role for α-synuclein toxicity in neurodegeneration. Recent studies of factors contributing to α-synuclein toxicity and its disruption of downstream cellular pathways have expanded our understanding of disease pathogenesis in synucleinopathies. In this Review, we discuss these emerging themes, including the contributions of aging, selective vulnerability and non-cell-autonomous factors such as α-synuclein cell-to-cell propagation and neuroinflammation. Finally, we summarize recent efforts toward the development of targeted therapies for PD and related synucleinopathies.

摘要

α-突触核蛋白剂量的改变会导致家族性帕金森病(PD),其积累会导致突触核蛋白病,包括PD、路易体痴呆(DLB)和多系统萎缩(MSA)。此外,α-突触核蛋白会促进淀粉样蛋白-β和tau蛋白(阿尔茨海默病中的两种关键蛋白)的纤维化,这表明α-突触核蛋白毒性在神经退行性变中起核心作用。最近对导致α-突触核蛋白毒性及其对下游细胞通路破坏的因素的研究,扩展了我们对突触核蛋白病发病机制的理解。在本综述中,我们讨论这些新出现的主题,包括衰老、选择性易损性以及非细胞自主性因素(如α-突触核蛋白的细胞间传播和神经炎症)的作用。最后,我们总结了近期针对PD及相关突触核蛋白病开发靶向治疗方法的努力。

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本文引用的文献

2
Pathological α-synuclein transmission initiated by binding lymphocyte-activation gene 3.
Science. 2016 Sep 30;353(6307). doi: 10.1126/science.aah3374.
3
Molecular and Biological Compatibility with Host Alpha-Synuclein Influences Fibril Pathogenicity.
Cell Rep. 2016 Sep 20;16(12):3373-3387. doi: 10.1016/j.celrep.2016.08.053.
4
Lysosomal trafficking defects link Parkinson's disease with Gaucher's disease.
Mov Disord. 2016 Nov;31(11):1610-1618. doi: 10.1002/mds.26802. Epub 2016 Sep 13.
5
Tunneling nanotubes spread fibrillar α-synuclein by intercellular trafficking of lysosomes.
EMBO J. 2016 Oct 4;35(19):2120-2138. doi: 10.15252/embj.201593411. Epub 2016 Aug 22.
6
9
G2019S-LRRK2 Expression Augments α-Synuclein Sequestration into Inclusions in Neurons.
J Neurosci. 2016 Jul 13;36(28):7415-27. doi: 10.1523/JNEUROSCI.3642-15.2016.
10
α-Synuclein binds to TOM20 and inhibits mitochondrial protein import in Parkinson's disease.
Sci Transl Med. 2016 Jun 8;8(342):342ra78. doi: 10.1126/scitranslmed.aaf3634.

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