Dickson D W
Department of Pathology, Mayo Clinic, Jacksonville, Florida 32224, USA.
Curr Opin Neurol. 2001 Aug;14(4):423-32. doi: 10.1097/00019052-200108000-00001.
alpha-Synuclein has risen to prominence during the past 5 years because of its association with several neurodegenerative diseases that have come to be known as the synucleinopathies. The clinical phenotype of the synucleinopathies is variable, with the most common being parkinsonism, autonomic dysfunction, and dementia. Progress has been made in clinical, neuropathologic and biochemical characterization of the synucleinopathies and their differentiation from other neurodegenerative disorders. At the molecular level, the synucleinopathies have conformational and post-translational modifications of synuclein that favor its fibrillization and aggregation in inclusions in neurons and glia. Whether inclusion body formation is an adaptive response or is directly related to degeneration of neuronal and glial cells is a topic of current research.
在过去5年里，α-突触核蛋白因其与几种神经退行性疾病（即突触核蛋白病）相关而备受关注。突触核蛋白病的临床表型多样，最常见的是帕金森综合征、自主神经功能障碍和痴呆。在突触核蛋白病的临床、神经病理学和生化特征以及它们与其他神经退行性疾病的鉴别方面已经取得了进展。在分子水平上，突触核蛋白病存在突触核蛋白的构象和翻译后修饰，这有利于其在神经元和神经胶质细胞的包涵体中形成纤维和聚集。包涵体形成是一种适应性反应还是与神经元和神经胶质细胞的变性直接相关，是当前研究的一个课题。
