Fragmentation of the Golgi apparatus of the anterior horn cells in patients with familial amyotrophic lateral sclerosis with SOD1 mutations and posterior column involvement
Fragmentation of the Golgi apparatus of the anterior horn cells in patients with familial amyotrophic lateral sclerosis with SOD1 mutations and posterior column involvement
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DOI：
10.1016/s0022-510x(00)00265-3
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发表时间：
2000-03-15
影响因子：
4.4
通讯作者：
Hirano, A
中科院分区：
文献类型：
--
作者：
Fujita, Y;Okamoto, K;Hirano, A
应用免疫组织化学方法对3例伴有后柱受累的家族性肌萎缩侧索硬化症（FALS）患者脊髓前角细胞的高尔基体（GA）进行了研究。大的运动神经元的前下摆显着减少的数量和10个剩余的14个大的运动神经元显示碎片和GA的元素的数量减少。GA的碎片是相同的，以前报道的运动神经元的脊髓和运动皮层的患者散发性ALS和转基因小鼠表达的G93 A突变的基因编码的铜/锌超氧化物歧化酶几个月前瘫痪。这是第一次报告的碎裂GA的前角细胞的患者与后柱参与的FALS。这些结果表明，GA是散发性和FALS神经元变性的共同靶点。(C)2000 Elsevier Science B. V.保留所有权利。
The Golgi apparatus (GA) of the anterior horn cells in the spinal cord was examined by immunohistological methods with an antibody against the MG-160 protein, a conserved intrinsic membrane sialoglycoprotein of the medial cisternae of the GA, in three patients with familial amyotrophic lateral sclerosis (FALS) with posterior column involvement. Large motor neurons in the anterior hems were markedly reduced in number and 10 of total 14 remaining large motor neurons showed fragmentation and a reduction in the number of the elements of the GA. The fragmentation of the GA was identical to that previously reported in motor neurons of the spinal cord and motor cortex from patients with sporadic ALS and in transgenic mice expressing the G93A mutation of the gene encoding the Cu/Zn superoxide dismutase months before the onset of paralysis. This is the first report of fragmented GA of the anterior horn cells in patients with FALS with posterior column involvement. The findings suggest that the GA is a common target in the neuronal degeneration in sporadic and FALS. (C) 2000 Elsevier Science B.V. All rights reserved.