Brain and nerve = Shinkei kenkyū no shinpo
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Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder characterized by the death of upper and lower motor neurons. Approximately 20% of familial ALS cases are caused by mutations in the superoxide dismutase 1 (SOD1) gene. We generated rats that express a human SOD1 transgene with two different ALS-associated mutations and found that these rats develop remarkable motor neuron degeneration and paralysis. ⋯ These projects have been supported by the "Super Special Consortium for Supporting the Development of Cutting-edge Medical Care" (tokku), a special program organized by the Cabinet Office of the Japanese government (research representative: Hideyuki Okano, M. D., Ph. D., Professor at Keio University).