伴有广泛空泡形成和透明包涵体的家族性肌萎缩侧索硬化症
Familial amyotrophic lateral sclerosis with widespread vacuolation and hyaline inclusions.
作者信息
Sasaki S, Ohsawa Y, Yamane K, Sakuma H, Shibata N, Nakano R, Kikugawa K, Mizutani T, Tsuji S, Iwata M
机构信息
Department of Neurology, Tokyo Women's Medical College, Japan.
出版信息
Neurology. 1998 Sep;51(3):871-3. doi: 10.1212/wnl.51.3.871.
This report presents a familial amyotrophic lateral sclerosis (FALS) patient with widespread vacuoles and hyaline inclusions strongly immunostained with the anti-superoxide dismutase (SOD1) antibody. The overall pathologic similarity between our non-SOD1-linked FALS patient and transgenic mice expressing a mutated human SOD1 gene suggests that common pathogenetic mechanisms other than an SOD1 mutation exist in the development of these diseases.
本报告介绍了一名家族性肌萎缩侧索硬化症（FALS）患者，其有广泛的空泡和透明包涵体，用抗超氧化物歧化酶（SOD1）抗体进行免疫染色时呈强阳性。我们的非SOD1相关FALS患者与表达突变型人类SOD1基因的转基因小鼠在总体病理上具有相似性，这表明在这些疾病的发展过程中存在除SOD1突变之外的共同致病机制。
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