Comprehensive research on pathomechanism therapy of ALS and

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With transgenic mice of mutant SOD1, survival signals such as PI3K, Akt, bcl2 were found to be decreased from early period of presymptomatic stage. VEGF induction after hypox is was greatly reduced in this transgenic mice model. Furthermore, reduction of glutamatergic AMPA receptor is also involved in the pathogenesis of the disease.For development of in vivo treatment for ALS, intrathecal injection of IGF-1 was newly developed for experimental mice and human patients. With this therapy, motor neurons were well preserved with retention of survival signals. Furthermore, intrathecal injection of IGF-1 also preserved clinical symptoms. of human ALS patients for 9 month of treatment. Thus this, project was effectively achieved this year with successful results.