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TDP-43参与肌萎缩侧索硬化症的病理机制研究进展

黄茂鑫,廉宏想

International Journal of Pathology and Clinical Medicine（2021）

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Abstract
肌萎缩侧索硬化症(amyotrophic lateral sclerosis,ALS)是以选择性上、下运动神经元损害为特征的慢性进行性神经系统变性疾病,其发病机制尚不明确.TDP-43(transactive response DNA-binding protein-43)在细胞质中形成包涵体是ALS的特征性病理标志之一.病理性TDP-43通过影响细胞内多种生理过程介导了神经退变的发生,如TDP-43可损害线粒体功能,引发内质网应激(endoplasmic reticulum stress,ER stress),TDP-43的病理性聚集与应激颗粒(stress granule,SG)有密切关系,TDP-43可与细胞内降解途径相互作用产生细胞保护或细胞毒性效应等.
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