von Bohlen Und Halbach Oliver
Interdisciplinary Center for Neurosciences, Department of Neuroanatomy, University of Heidelberg, Im Neuenheimer Feld 307, 69120 Heidelberg, Germany.
Cell Tissue Res. 2004 Oct;318(1):163-74. doi: 10.1007/s00441-004-0921-7. Epub 2004 Jul 24.
Parkinson's disease (PD) is one of the most common neurodegenerative motor disorders, marked by chronic progressive loss of neurons in the substantia nigra. It has long been believed that PD is caused by environmental factors. The discovery of genetic factors involved in PD has improved the understanding of the pathology of the disease. The first gene found to be mutated in PD encodes for the presynaptic protein alpha-synuclein. alpha-Synuclein is a major component of Lewy bodies and Lewy neurites, which represent the morphological hallmarks of the disease. The mechanisms by which alpha-synuclein is involved in nigral cell death remain poorly understood. Moreover, the factors triggering the formation of alpha-synuclein-positive inclusion bodies remain enigmatic. Indeed, even the normal cellular functions of alpha-synuclein and of the other synucleins (beta-synuclein and gamma-synuclein) are still unknown. Several lines of evidence suggest that they play a role in the regulation of vesicular turnover under normal nonpathological conditions.
帕金森病（PD）是最常见的神经退行性运动障碍之一，其特征是黑质中神经元的慢性进行性丧失。长期以来，人们一直认为帕金森病是由环境因素引起的。参与帕金森病的遗传因素的发现增进了对该疾病病理学的理解。第一个被发现与帕金森病相关的突变基因编码突触前蛋白α-突触核蛋白。α-突触核蛋白是路易小体和路易神经突的主要成分，而路易小体和路易神经突是该疾病的形态学标志。α-突触核蛋白参与黑质细胞死亡的机制仍知之甚少。此外，触发α-突触核蛋白阳性包涵体形成的因素仍然是个谜。事实上，即使是α-突触核蛋白以及其他突触核蛋白（β-突触核蛋白和γ-突触核蛋白）的正常细胞功能也仍然未知。有几条证据表明，它们在正常非病理条件下的囊泡周转调节中发挥作用。
