Wakabayashi K, Takahashi H
Department of Neuropathology, Hirosaki University School of Medicine.
Nihon Rinsho. 2000 Oct;58(10):2022-7.
The presence of Lewy bodies(LBs) in the substantia nigra and other subcortical nuclei is a diagnostic hallmark of Parkinson's disease(PD). Recently, two mutations in the alpha-synuclein gene in families with autosomal dominant PD were identified. Subsequent immunocytochemical studies showed that antibodies to alpha-synuclein detect all of the LBs and Lewy neurites in the brains of patients with PD. Immunoelectron microscopy revealed that the reaction product is localized within abnormal filamentous structures. Moreover, alpha-synuclein is aggregated and fibrillated in vitro. More recently, a novel protein that associates with alpha-synuclein, called synphilin-1, has been reported to be present in LBs. These findings suggest that both alpha-synuclein and synphilin-1 are precise molecular compositions of LBs.
黑质及其他皮质下核团中路易小体（LB）的存在是帕金森病（PD）的诊断标志。最近，在常染色体显性遗传PD家族中发现了α-突触核蛋白基因的两个突变。随后的免疫细胞化学研究表明，针对α-突触核蛋白的抗体可检测出PD患者大脑中的所有路易小体和路易神经突。免疫电子显微镜显示反应产物定位于异常丝状结构内。此外，α-突触核蛋白在体外会聚集并形成纤维。最近，据报道一种与α-突触核蛋白相关的新型蛋白质——突触结合蛋白-1存在于路易小体中。这些发现表明α-突触核蛋白和突触结合蛋白-1都是路易小体精确的分子组成成分。
