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运动神经元变性死亡的内质网相关机制
作者:宋彬彬  陈峥  宋岳涛  李保英  高茂龙  刘向国  杨璇  于佳 
单位:北京中医药大学附属北京老年医院 老年病临床与康复研究所, 北京 100095
关键词:内质网应激 未折叠蛋白反应 运动神经元 肌萎缩侧索硬化症 综述 
分类号:R744.8
出版年·卷·期(页码):2018·46·第三期(334-340)
摘要:

肌萎缩侧索硬化症(amyotrophic lateral sclerosis,ALS)是最常见的运动神经元病,选择性累及上、下运动神经元,当运动神经元中出现错误折叠或未折叠蛋白大量积聚或钙紊乱可引起内质网应激和未折叠蛋白反应(unfolded protein response,UPR)。一定程度的内质网应激/UPR可以保护细胞,而ALS中出现持续强烈的内质网应激/UPR会引起CHOP/GADD153(C/EBP homologous protein/growth arrest-and DNA damage inducible gene 153)、c-Jun氨基末端激酶和caspase12介导的细胞凋亡。作者将对内质网生理结构和功能、ALS中出现内质网应激/UPR及其引起运动神经元变性死亡的相关机制进行综述,以期更好地认识内质网应激与ALS的关系。

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