Common coupling protein of amyotrophic lateral sclerosis and spinocerebellar degeneration

本研究的目的是阐明肌萎缩侧索硬化症和脊髓小脑变性的共同偶联蛋白。ATXN蛋白和TDP-43蛋白可以相互结合。我们用组织病理学证明了这一点。(1)肌萎缩性侧索硬化症患者的CAG重复数均在正常范围内。(2)抗atxn蛋白抗体免疫染色未发现ALS病理细胞内包涵体。ALS合并痴呆患者仅在局部齿状回胞浆内包涵体表达一种ataxin-2。(3) SCA2脊髓前角中TDP-43的免疫组化显示出三种不同的表达模式：“核内”、“核内内含物”和“细胞质”。这些结果表明，神经细胞中TDP-43蛋白的易位可能在SCD细胞死亡中起重要作用。

The aim of this study is clarify the common coupling protein of amyotrophic lateral sclerosis and spinocerebellar degeneration. The ATXN protein and TDP-43 protein may be combined each other. We prove this histopathologically.(1) We measured the number of the CAG repeat in the patient of amyotrophic lateral sclerosis, but it was in the normal range. (2) Pathological intracellular inclusions in ALS were not found by immunostaining of an anti-ATXN protein antibody. Only an ataxin-2 expressed in the intracytoplasmic inclusion body of the dentate gyrus of the part in case of ALS with dementia. (3) Immunohistochemistry for TDP-43 in an anterior horn of the spinal cord in SCA2 has demonstrated three different expression patterns: 'intranuclear' 'nuclear with inclusions' and 'cytoplasmic'. These results show that the translocation of the TDP-43 protein in a nerve cell may play an important role of the cell death of SCD.