Wakabayashi Koichi, Tanji Kunikazu, Mori Fumiaki, Takahashi Hitoshi
Department of Neuropathology, Institute of Brain Science, Hirosaki University School of Medicine, Hirosaki, Japan.
Neuropathology. 2007 Oct;27(5):494-506. doi: 10.1111/j.1440-1789.2007.00803.x.
The histological hallmark of Parkinson's disease (PD) is the presence of fibrillar aggregates called Lewy bodies (LBs). LB formation has been considered to be a marker for neuronal degeneration, because neuronal loss is found in the predilection sites for LBs. To date, more than 70 molecules have been identified in LBs, in which alpha-synuclein is a major constituent of LB fibrils. Alpha-synuclein immunohistochemistry reveals that diffuse cytoplasmic staining develops into pale bodies via compaction, and that LBs arise from the peripheral portion of pale bodies. This alpha-synuclein abnormality is found in 10% of pigmented neurons in the substantia nigra and more than 50% of those in the locus ceruleus in PD. Recent studies have suggested that oligomers and protofibrils of alpha-synuclein are cytotoxic, and that LBs may represent a cytoprotective mechanism in PD.
帕金森病（PD）的组织学特征是存在称为路易小体（LBs）的纤维状聚集体。路易小体的形成一直被认为是神经元变性的标志，因为在路易小体的好发部位发现了神经元丢失。迄今为止，已在路易小体中鉴定出70多种分子，其中α-突触核蛋白是路易小体纤维的主要成分。α-突触核蛋白免疫组织化学显示，弥漫性细胞质染色通过压实发展为浅色小体，并且路易小体起源于浅色小体的周边部分。在帕金森病中，这种α-突触核蛋白异常在黑质中10%的色素神经元以及蓝斑中超过50%的色素神经元中被发现。最近的研究表明，α-突触核蛋白的寡聚体和原纤维具有细胞毒性，并且路易小体可能代表帕金森病中的一种细胞保护机制。
