Alpha and Beta Synucleins: From Pathophysiology to Clinical Application as Biomarkers.

Alpha and Beta Synucleins: From Pathophysiology to Clinical Application as Biomarkers.
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DOI:
10.1002/mds.28941
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发表时间:
2022-04
期刊:
影响因子:
8.6
通讯作者:
Parnetti, Lucilla
Parnetti, Lucilla
中科院分区:
医学1区
文献类型:
--
作者:
Barba, Lorenzo;Paolini Paoletti, Federico;Bellomo, Giovanni;Gaetani, Lorenzo;Halbgebauer, Steffen;Oeckl, Patrick;Otto, Markus;Parnetti, Lucilla

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突触核蛋白家族包括三种神经元蛋白,分别为α -突触核蛋白、β -突触核蛋白和γ -突触核蛋白,它们具有特殊的结构特征。α‐突触核蛋白被广泛认为是帕金森病(PD)和其他突触核蛋白病(即路易体痴呆和多系统萎缩)病理生理学中的关键蛋白。β -突触核蛋白和γ -突触核蛋白在生理功能和对人类疾病的潜在贡献方面的作用尚不清楚。α‐突触核蛋白作为突触核蛋白病的潜在生物标志物在脑脊液(CSF)和血液中被广泛研究。最近,β -突触核蛋白也引起了人们的高度关注,其脑脊液和血液水平似乎反映了突触损伤和神经退行性变,而与突触核蛋白病的存在无关。本文就突触核蛋白的病理生理作用作一综述。由于γ - synuclein在突触核蛋白病领域的研究很少,其病理生理作用也远未明确,因此我们将重点放在α - synuclein和β - synuclein在PD中的相互作用上。我们还讨论了α -突触核蛋白和β -突触核蛋白作为潜在生物标志物的作用,以改善突触核蛋白病的诊断特征,从而强调它们在疾病修饰治疗的临床试验中的潜在应用。©2022作者。由Wiley期刊有限责任公司代表国际帕金森和运动障碍协会出版的《运动障碍》4月信息图:α和β突触核蛋白:从病理生理学到作为生物标志物的临床应用
The synuclein family includes three neuronal proteins, named α‐synuclein, β‐synuclein, and γ‐synuclein, that have peculiar structural features. α‐synuclein is largely known for being a key protein in the pathophysiology of Parkinson's disease (PD) and other synucleinopathies, namely, dementia with Lewy bodies and multisystem atrophy. The role of β‐synuclein and γ‐synuclein is less well understood in terms of physiological functions and potential contribution to human diseases. α‐synuclein has been investigated extensively in both cerebrospinal fluid (CSF) and blood as a potential biomarker for synucleinopathies. Recently, great attention has been also paid to β‐synuclein, whose CSF and blood levels seem to reflect synaptic damage and neurodegeneration independent of the presence of synucleinopathy. In this review, we aim to provide an overview on the pathophysiological roles of the synucleins. Because γ‐synuclein has been poorly investigated in the field of synucleinopathy and its pathophysiological roles are far from being clear, we focus on the interactions between α‐synuclein and β‐synuclein in PD. We also discuss the role of α‐synuclein and β‐synuclein as potential biomarkers to improve the diagnostic characterization of synucleinopathies, thus highlighting their potential application in clinical trials for disease‐modifying therapies. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society April Infographic : Alpha and Beta Synucleins: From Pathophysiology to Clinical Application as Biomarkers
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