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伴路易体痴呆的路易体的主要成分，也是多系统萎缩的胶质细胞质包涵体的主要成分。最近，一种名为synphilin-1的新蛋白被发现与α -突触核蛋白相关，并且据报道，α -突触核蛋白和synphilin-1在哺乳动物细胞中共同转染产生了类似路易小体的嗜酸性细胞质包涵体。免疫细胞化学和超微结构的研究已经在各种神经退行性疾病患者的大脑中使用抗突触蛋白-1抗体进行。这些抗体在对照组的整个大脑中以点状模式对神经细胞进行免疫染色。在帕金森病中，脑干中观察到的大多数路易小体均阳性。路易小体中心核呈强烈染色，外周部分呈弱染色或无染色。-突触核蛋白阳性的苍白体和路易神经突为突触蛋白-1阴性。在伴有路易小体的痴呆中，一小部分皮层路易小体被抗突触蛋白-1免疫标记。在多系统萎缩中，许多胶质细胞质包涵体对突触蛋白-1呈阳性。免疫电镜显示反应产物定位于路易小体的丝状和圆形结构内。除路易体病和多系统萎缩外，神经退行性疾病的各种神经元和胶质包涵体均为突触蛋白-1阴性。这些发现表明突触蛋白-1的异常积累是脑病变的特异性，而α -突触核蛋白是脑病变的主要成分。

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.