[Animal models of amyotrophic lateral sclerosis].
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.
Yamanaka
Koji
K
Yamanaka Research Unit, RIKEN Brain Science Institute.
D000690:Amyotrophic Lateral Sclerosis; D000818:Animals; D004195:Disease Models, Animal; D051379:Mice; D008822:Mice, Transgenic