Budget Amount *help |
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2009: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2008: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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Research Abstract |
Waardenburg syndrome, an auditory-pigmentary syndrome, is caused by abnormal differentiation in melanocyte-related gene such as endothelin receptor B (Ednrb). In this study, the aim is an elucidation of hearing loss with pigmentation disorder and its therapeutic development. Ednrb-deficient mice (Ednrb-KO) were deafness and had a defect of melanocyte in inner ear and decrease of spiral ganglion cells. The hearing losses with neurodegeneration of spiral ganglion cells were improved by introducing an Ednrb transgene under control of the dopamine beta-hydroxylase promoter.
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