Article

Review Article

Exp Neurobiol 2016; 25(5): 233-240

Published online October 31, 2016

https://doi.org/10.5607/en.2016.25.5.233

© The Korean Society for Brain and Neural Sciences

Astrocytes and Microglia as Non-cell Autonomous Players in the Pathogenesis of ALS

Junghee Lee1,2*, Seung Jae Hyeon3, Hyeonjoo Im3, Hyun Ryu2, Yunha Kim3 and Hoon Ryu1,2,3*

1Veterans Affairs Boston Healthcare System, Boston, MA 02130, 2Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA, 3Center for Neuromedicine, Brain Science Institute, Korea Institute of Science and Technology, Seoul 04535, Korea

Correspondence to: *To whom correspondence should be addressed.
TEL: 1-857-364-6034, 5910, FAX: 1-857-364-4540
Junghee Lee e-mail: Junghee@bu.edu, Hoon Ryu hoonryu@bu.edu

Received: August 4, 2016; Revised: August 20, 2016; Accepted: August 22, 2016

Abstract

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.

Keywords: amyotrophic lateral sclerosis, astrocyte, microglia, motor neuron, non-cell autonomous toxicity