Rinsho Shinkeigaku (Clinical Neurology)

The 48th Annual Meeting of the Japanese Society of Neurology

Animal models of amyotrophic lateral sclerosis

Koji Yamanaka, M.D., Ph.D.

Yamanaka Research Unit, RIKEN Brain Science Institute

Dominant mutation in the gene of superoxide dismutase 1 (SOD1) leads amyotrophic lateral sclerosis (ALS), an adult-onset progressive fatal motor neuron disease. Recent research progress in 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. In this symposium, I review the recent advance of ALS research focusing on the development of animal models, the role of glial cells in ALS, and therapeutic intervention of rodent models and discuss their prospect.

(CLINICA NEUROL, 47: 934|937, 2007)
key words: ALS, SOD1, transgenic mouse, non cell-autonomous neuron death, microglia

(Received: 16-May-07)