Department of Neurology, Teikyo University Chiba Medical Center, Ichihara, Japan.
Acta Neurol Scand. 2010 Nov;122(5):367-72. doi: 10.1111/j.1600-0404.2010.01321.x.
Transactivation-responsive DNA-binding protein-43 (TDP-43) was identified as a major component of the ubiquitin-positive inclusions in sporadic amyotrophic lateral sclerosis (ALS). However, there has been no study of TDP-43 in ALS skin. The present study investigates TDP-43 in ALS skin.
We made a quantitative immunohistochemical study of the expression of TDP-43 in the skin from 15 patients with ALS and 15 control subjects.
The proportion of TDP-43-positive (TDP-43+) cells in the epidermis in ALS patients was significantly higher (P < 0.001) than in controls. There was a significant positive relationship (r = 0.62, P < 0.02) between the proportion and duration of illness in ALS patients. The optical density of TDP-43+ cells in the epidermis in ALS patients is markedly stronger (P < 0.001) than in controls. There was a significant positive relation (r = 0.72, P < 0.01) between the immunoreactivity and duration of illness in ALS patients.
These data suggest that changes of TDP-43 in ALS skin are likely to be related to the disease process and that metabolic alterations of TDP-43 may take place in the skin of patients with ALS.
转激活反应 DNA 结合蛋白 43（TDP-43）被鉴定为散发性肌萎缩侧索硬化症（ALS）中泛素阳性包涵体的主要成分。然而，目前还没有关于 ALS 皮肤中 TDP-43 的研究。本研究调查了 ALS 皮肤中的 TDP-43。
我们对 15 例 ALS 患者和 15 例对照者的皮肤进行了 TDP-43 的定量免疫组织化学研究。
ALS 患者表皮中 TDP-43 阳性（TDP-43+）细胞的比例明显高于对照组（P < 0.001）。ALS 患者中，比例与疾病持续时间之间存在显著正相关（r = 0.62，P < 0.02）。ALS 患者表皮中 TDP-43+细胞的光密度明显强于对照组（P < 0.001）。ALS 患者的免疫反应活性与疾病持续时间之间存在显著正相关（r = 0.72，P < 0.01）。
这些数据表明，ALS 皮肤中 TDP-43 的变化可能与疾病过程有关，并且 TDP-43 的代谢改变可能发生在 ALS 患者的皮肤中。
