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从发病到进展:突触核蛋白病中α-突触核蛋白的时间谱

From onset to advancement: the temporal spectrum of α-synuclein in synucleinopathies.

作者信息

Wiseman James A, Reddy Kreesan, Dieriks Birger Victor

机构信息

Department of Anatomy and Medical Imaging, University of Auckland, Auckland, New Zealand; Centre for Brain Research, University of Auckland, Auckland 1023, New Zealand; Brain and Mind Centre & Faculty of Medicine and Health School of Medical Sciences, The University of Sydney, Sydney, NSW 2050, Australia.

Department of Anatomy and Medical Imaging, University of Auckland, Auckland, New Zealand; Centre for Brain Research, University of Auckland, Auckland 1023, New Zealand; Brain and Mind Centre & Faculty of Medicine and Health School of Medical Sciences, The University of Sydney, Sydney, NSW 2050, Australia.

出版信息

Ageing Res Rev. 2025 Feb;104:102640. doi: 10.1016/j.arr.2024.102640. Epub 2024 Dec 10.

Abstract

This review provides an in-depth analysis of the complex role of alpha-synuclein (α-Syn) in the development of α-synucleinopathies, with a particular focus on its structural diversity and the resulting clinical variability. The ability of α-Syn to form different strains or polymorphs and undergo various post-translational modifications significantly contributes to the wide range of symptoms observed in disorders such as Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), as well as in lesser-known non-classical α-synucleinopathies. The interaction between genetic predispositions and environmental factors further complicates α-synucleinopathic disease pathogenesis, influencing the disease-specific onset and progression. Despite their common pathological hallmark of α-Syn accumulation, the clinical presentation and progression of α-synucleinopathies differ significantly, posing challenges for diagnosis and treatment. The intricacies of α-Syn pathology highlight the critical need for a deeper understanding of its biological functions and interactions within the neuronal environment to develop targeted therapeutic strategies. The precise point at which α-Syn aggregation transitions from being a byproduct of initial disease triggers to an active and independent driver of disease progression - through the propagation and acceleration of pathogenic processes - remains unclear. By examining the role of α-Syn across various contexts, we illuminate its dual role as both a marker and a mediator of disease, offering insights that could lead to innovative approaches for managing α-synucleinopathies.

摘要

本综述深入分析了α-突触核蛋白(α-Syn)在α-突触核蛋白病发展过程中的复杂作用,特别关注其结构多样性以及由此产生的临床变异性。α-Syn形成不同菌株或多形体以及经历各种翻译后修饰的能力,显著促成了在帕金森病(PD)、路易体痴呆(DLB)和多系统萎缩(MSA)等疾病以及鲜为人知的非经典α-突触核蛋白病中观察到的广泛症状。遗传易感性与环境因素之间的相互作用进一步使α-突触核蛋白病的发病机制复杂化,影响疾病特异性的发病和进展。尽管α-突触核蛋白病具有α-Syn积累这一共同的病理特征,但其临床表现和进展却有显著差异,给诊断和治疗带来了挑战。α-Syn病理学错综复杂,凸显了深入了解其在神经元环境中的生物学功能和相互作用以制定靶向治疗策略的迫切需求。α-Syn聚集从初始疾病触发因素的副产品转变为通过致病过程的传播和加速成为疾病进展的活跃且独立驱动因素的确切时间点仍不清楚。通过研究α-Syn在各种情况下的作用,我们阐明了其作为疾病标志物和介质的双重作用,并提供了可能带来管理α-突触核蛋白病创新方法的见解。

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