| Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2013: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2012: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2011: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
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| Research Abstract |
We identified novel mutations of optineurin (OPTN) as a cause of amyotrophic lateral sclerosis (ALS), and OPTN is involved in various molecular mechanisms. We created OPTN knockout mice and knock-in mouse, and established mouse embryonic fibroblasts and ES cells for experiments with cultured cell lines. At this moment, we have not obtained a pathological phenotype, but pathological analysis revealed neuronal loss of the anterior horn of the spinal cord. There was not any significant change in the autophagy-associated molecules in the analysis using the MEF. However, it is indicated that OPTN plays an important role in cellular organelle regulation. We are aiming at revealing the detail of pathogenesis caused by OPTN mutation by iPS-derived neuron induced from the patient's fibroblast.
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