BRAIN and NERVE－神経研究の進歩71巻11号

2019年11月発行

増大特集 ALS2019

ALSの病理

著者： 吉田眞理¹

所属機関： ¹愛知医科大学加齢医科学研究所

ページ範囲：P.1152 - P.1168

文献概要

筋萎縮性側索硬化症（ALS）は病理学的に上位運動ニューロン（UMN）と下位運動ニューロン（LMN）がさまざまな程度の組合せで障害される疾患である。孤発性ALSのLMNではTDP-43の核内局在が消失し，細胞質に異常に凝集して封入体を形成する。TDP-43の封入体は運動ニューロン系を超えて神経細胞とグリア細胞に分布する。病変の進展機序に，TDP-43のプリオン病様蛋白伝播仮説が提唱されている。

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