Roles of glial cells in motor neuron degeneration

Koji Yamanaka

Department of Neuroscience and Pathobiology, Research Institute of Environmental Medicine, Nagoya University

Pathomechanism of amyotrophic lateral sclerosis (ALS) is not restricted to the motor neurons but attributed to the abnormal interactions of neurons and surrounding glial cells. Researches using chimeric mice and cell-type specific gene ablation in inherited SOD1-ALS mice led to the concept of non-cell autonomous neurodegeneration. Toxicity of mutant SOD1 protein in motor neurons and neighboring glial cells, microglia and astrocytes, determines disease onset and progression, respectively. This review article discusses the involvement of microglia and astrocytes in inherited ALS mouse models and sporadic ALS. Understanding the cell-type specific pathomechanisms of motor neuron diseases could lead to future therapeutic strategies for ALS and could be potentially applied to other neurodegenerative diseases.

Address correspondence to Dr. Koji Yamanaka, Department of Neuroscience and Pathobiology, Research Institute of Environmental Medicine, Nagoya University (Furo-cho, Chikusa-ku, Nagoya, Aichi 464-8601, Japan)