Amyotrophic Lateral Sclerosis Disease Progression and Mitochondrial Dysfunction
10.13471/j.cnki.j.sun.yat-sen.univ（med.sci）.2022.0502
- VernacularTitle:肌萎缩侧索硬化症疾病进展与线粒体功能紊乱
- Author:
Zhi-long ZHENG
1
;
Xing GUO
1
Author Information
1. School of Basic Medicine， Nanjing Medical University， Nanjing 211166， China
- Publication Type:Review
- Keywords:
amyotrophic lateral sclerosis;
mitochondrial dysfunction;
motor neurons;
energy metabolism
- From:
Journal of Sun Yat-sen University(Medical Sciences)
2022;43(5):697-704
- CountryChina
- Language:Chinese
-
Abstract:
Amyotrophic lateral sclerosis （ALS） is one of typical neurodegenerative diseases characterized by progressive degeneration of motor neurons in the brain and/or spinal cord. A large number of ALS pathogenic genes have been screened out by DNA sequencing and broadened our scope with the occurrence of ALS. However， the downstream signaling pathways of these genes leading to the progression of ALS disease remains unclear. With the continuous progress of basic research， it has been found that mitochondrial damage， abnormal mitochondrial dynamics， and mitophagy play important roles in the pathogenesis of neurodegenerative diseases such as ALS. In this review， we mainly discussed the possible mechanism of mitochondrial dysfunction caused by pathogenic genes of ALS， in order to emphasize the importance of mitochondrial dysfunction in the development of ALS.
