线粒体功能障碍在肌萎缩侧索硬化发病中作用的研究进展

doi:10.16352/j.issn.1001-6325.2023.02.332

基础医学与临床 ›› 2023, Vol. 43 ›› Issue (2): 332-335.doi: 10.16352/j.issn.1001-6325.2023.02.332

线粒体功能障碍在肌萎缩侧索硬化发病中作用的研究进展

刘立洋^1,3, 柳青², 刘雅萍¹, 张学^1*

1.中国医学科学院基础医学研究所北京协和医学院基础学院医学遗传学系,北京 100005;
2.中国医学科学院北京协和医学院北京协和医院神经科,北京 100730;
3.中国医学科学院北京协和医学院临床医学专业试点班,北京 100730

收稿日期:2022-10-04 修回日期:2022-11-22 出版日期:2023-02-05 发布日期:2023-02-02
通讯作者: ^*xuezhang@pumc.edu.cn
基金资助:
中国医学科学院医学与健康科技创新工程(2021-I2M-1-018)

Progress in the study of mitochondrial dysfunction in pathogenesis of amyotrophic lateral sclerosis

LIU Liyang^1,3, LIU Qing², LIU Yaping¹, ZHANG Xue^1*

1. Department of Medical Genetics, Institute of Basic Medical Sciences CAMS, School of Basic Medicine PUMC, Beijing 100005;
2. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730;
3. 4+4 Medical Doctor Program, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

Received:2022-10-04 Revised:2022-11-22 Online:2023-02-05 Published:2023-02-02
Contact: ^*xuezhang@pumc.edu.cn

摘要/Abstract

摘要： 线粒体功能障碍在肌萎缩侧索硬化(ALS)的发病中有着重要作用。线粒体功能障碍导致严重的氧化应激损伤和细胞钙稳态失衡,以及被异常释放的线粒体DNA激活炎性反应,这些可能是造成ALS患者运动神经元死亡的重要原因。线粒体自噬作为线粒体质量控制的最后环节,其功能的减弱或过度增强都会促进ALS的发展。

关键词: 肌萎缩侧索硬化, 线粒体功能障碍

Abstract: Mitochondrial dysfunction plays an important role in the pathogenesis of amyotrophic lateral sclerosis(ALS). The mitochondrial dysfunction will cause severe oxidative stress and disruption in calcium homeostasis. The abnormally released mitochondrial DNA will induce cellular inflammation. These phenomena may explain the loss of motor neurons in ALS patients. As a quality control process, either abnormal increased or decreased mitophagy flux will speed up the pathogenic development of ALS.

Key words: amyotrophic lateral sclerosis, mitochondrial dysfunction

中图分类号:

R741.02

刘立洋, 柳青, 刘雅萍, 张学. 线粒体功能障碍在肌萎缩侧索硬化发病中作用的研究进展[J]. 基础医学与临床, 2023, 43(2): 332-335.

LIU Liyang, LIU Qing, LIU Yaping, ZHANG Xue. Progress in the study of mitochondrial dysfunction in pathogenesis of amyotrophic lateral sclerosis[J]. Basic & Clinical Medicine, 2023, 43(2): 332-335.

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线粒体功能障碍在肌萎缩侧索硬化发病中作用的研究进展

Progress in the study of mitochondrial dysfunction in pathogenesis of amyotrophic lateral sclerosis

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