Lee Junghee, Hyeon Seung Jae, Im Hyeonjoo, Ryu Hyun, Kim Yunha, Ryu Hoon
Veterans Affairs Boston Healthcare System, Boston, MA 02130, USA.; Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA.
Center for Neuromedicine, Brain Science Institute, Korea Institute of Science and Technology, Seoul 04535, Korea.
Exp Neurobiol. 2016 Oct;25(5):233-240. doi: 10.5607/en.2016.25.5.233. Epub 2016 Oct 20.
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder that leads to a progressive muscle wasting and paralysis. The pathological phenotypes are featured by severe motor neuron death and glial activation in the lumbar spinal cord. Proposed ALS pathogenic mechanisms include glutamate cytotoxicity, inflammatory pathway, oxidative stress, and protein aggregation. However, the exact mechanisms of ALS pathogenesis are not fully understood yet. Recently, a growing body of evidence provides a novel insight on the importance of glial cells in relation to the motor neuronal damage via the non-cell autonomous pathway. Accordingly, the aim of the current paper is to overview the role of astrocytes and microglia in the pathogenesis of ALS and to better understand the disease mechanism of ALS.
肌萎缩侧索硬化症(ALS)是一种毁灭性的神经退行性疾病,会导致进行性肌肉萎缩和瘫痪。其病理表型的特征是腰脊髓中严重的运动神经元死亡和胶质细胞激活。提出的ALS致病机制包括谷氨酸细胞毒性、炎症途径、氧化应激和蛋白质聚集。然而,ALS发病的确切机制尚未完全了解。最近,越来越多的证据为胶质细胞通过非细胞自主途径在运动神经元损伤中的重要性提供了新的见解。因此,本文的目的是概述星形胶质细胞和小胶质细胞在ALS发病机制中的作用,并更好地理解ALS的疾病机制。