Accumulation of alpha-synuclein is a cytopathological feature common to Parkinson's disease and multiple system atrophy

α-突触核蛋白是帕金森病和路易体痴呆中路易体的主要成分，也是多系统萎缩中神经胶质细胞质内含物的主要成分。最近，一种新的蛋白质称为synphilin-1已被确定，与α-突触核蛋白，它已被报道，共同转染的哺乳动物细胞中的嗜酸性细胞质包涵体类似Lewy体的嗜酸性细胞核蛋白和synphilin-1。免疫细胞化学和超微结构的调查，现在已经进行了各种神经退行性疾病的患者使用抗突触亲蛋白-1抗体的大脑。这些抗体免疫染色的神经元在整个大脑的控制对象的点状图案。在帕金森病中，在脑干中观察到的大多数路易体对synphilin-1呈阳性。这些路易体在其中央核心显示强染色，但其周边部分仅弱染色或未染色。α-突触核蛋白阳性的苍白小体和路易神经突为synphilin-1阴性。在路易体痴呆中，一小部分皮质路易体被抗突触亲蛋白-1免疫标记。在多系统萎缩中，许多胶质细胞胞浆包涵体为synphilin-1阳性。免疫电子显微镜显示，反应产物是本地化的丝状和环状结构的路易体。在路易体病和多系统萎缩以外的神经退行性疾病中，各种神经元和神经胶质包涵体均为synphilin-1阴性。这些发现表明，突触亲蛋白-1的异常积累是特定的脑病变，其中α-突触核蛋白是一个主要组成部分。

Alpha-Synuclein is a major component of Lewy bodies in Parkinson's disease and dementia with Lewy bodies, as well as of glial cytoplasmic inclusions in multiple system atrophy. Recently, a novel protein called synphilin-1 has been identified that associates with alpha-synuclein, and it has been reported that co-transfection of both alphasynuclein and synphilin-1 in mammalian cells yielded eosinophilic cytoplasmic inclusions resembling Lewy bodies. Immunocytochemical and ultrastructural investigations have now been performed on the brain of patients with various neurodegenerative disorders using anti-synphilin-1 antibodies. These antibodies immunostained the neuropil in a punctate pattern throughout the brain of control subjects. In Parkinson's disease, most Lewy bodies observed in the brainstem were positive for synphilin-1. These Lewy bodies showed intense staining in their central cores, but their peripheral portions were only weakly stained or unstained. Pale bodies and Lewy neurites, which were positive for alpha-synuclein, were synphilin-1-negative. In dementia with Lewy bodies, a small fraction of cortical Lewy bodies were immunolabeled by anti-synphilin-1. In multiple system atrophy, numerous glial cytoplasmic inclusions were positive for synphilin-1. Immunoelectron microscopy revealed that the reaction product was localized within filamentous and circular structures in Lewy bodies. Various neuronal and glial inclusions in neurodegenerative disorders other than Lewy body disease and multiple system atrophy were synphilin-1-negative. These findings suggest that abnormal accumulation of synphilin-1 is specific for brain lesions in which alpha-synuclein is a major component.