Wong Yvette C, Krainc Dimitri
Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Nat Med. 2017 Feb 7;23(2):1-13. doi: 10.1038/nm.4269.
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.
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。