BRAIN and NERVE Shinkei Kenkyu no Shinpo Volume 59, Issue 10 (October 2007)

ALS and Microglia―a Player for Non-cell-autonomous Neuron Death Koji Yamanaka 1 , Hirofumi Yamashita 1 1Yamanaka Research Unit, RIKEN Brain Science Institute Keyword: amyotrophic lateral sclerosis (ALS) , SOD1 , microglia , astrocyte , neurodegeneration pp.1163-1170
Published Date 2007/10/1
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 Dominant mutation in the gene of superoxide dismutase 1 (SOD1) leads to amyotrophic lateral sclerosis (ALS), an adult-onset progressive fatal motor neuron disease. Recent progress in research on ALS has been made by the use of transgenic mouse model of familial ALS, which expresses mutant form of SOD1 and recapitulates the phenotype and pathology of motor neuron disease. There is accumulating evidence indicating non-cell-autonomous motor neuron death in ALS mouse model. Using the mice carrying deletable mutant SOD1 transgene by the action of Cre recombinase, we have demonstrated that diminishing mutant SOD1 toxicity within microglia significantly slowed disease progression of ALS, indicating the active role of microglia in disease progression of ALS. In this paper, we review the recent advance of ALS research focusing on the role of glial cells in ALS and discuss its prospect.

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BRAIN and NERVE-神経研究の進歩
59巻10号 (2007年10月)
電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院