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和脊髓小脑变性共同的偶联蛋白。ATXN蛋白和TDP-43蛋白可以彼此组合。我们在组织病理学上证明了这一点。(1)我们检测了肌萎缩侧索硬化患者的CAG重复序列，但在正常范围内。(2)通过抗ATXN蛋白抗体的免疫染色未发现ALS中的病理性细胞内包涵体。在ALS伴痴呆的病例中，仅在齿状回的胞浆内包涵体中表达共济失调蛋白-2。(3)免疫组织化学TDP-43在SCA 2脊髓前角已证明了三种不同的表达模式：“核内”，“核与夹杂物”和“细胞质”。这些结果表明TDP-43蛋白在神经细胞中的移位可能在SCD的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.