The participation of p47, which interacts with TDP-43 as a causative protein of ALS, in ALS pathology

TDP-43是肌萎缩侧索硬化症的致病蛋白之一，与肌萎缩侧索硬化症的发病机制密切相关。然而，TDP-43引起运动神经元变性的机制尚不清楚。本研究的代表人物(I)确定P47是一种新的与TDP-43相互作用的蛋白。此外，我还澄清了ALS连锁突变体TDP-43蛋白的半衰期比野生型要长。肌萎缩侧索硬化症的早期发病与家族性肌萎缩侧索硬化症连锁突变TDP-43蛋白的稳定性增加有关。

TDP-43 is one of a causative protein of amyotrophic lateral sclerosis(ALS), and generally related with the ALS pathology. The mechanism of motor neuron degeneration by TDP-43, however, is still unknown.The representative of this study(I) identified p47 as a novel protein interacted with TDP-43.I elucidated that p47 interacts with not ALS-linked TDP-43 mutant proteins but wild-type. In addition, I clarified that the half-life of ALS-linked mutant TDP-43 protein is longer than that of wild-type. Early onset of ALS correlates with increased stability of familial ALS-linked mutant TDP-43 proteins.