| Project/Area Number |
14370539
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Otorhinolaryngology
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| Research Institution | Tokyo Medical and Dental University |
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Principal Investigator |
KITAMURA Ken Tokyo Medical and Dental University, Dept of Otolaryngology, Graduate School Professor, 大学院・医歯学総合研究科, 教授 (90010470)
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| Co-Investigator(Kenkyū-buntansha) |
TSUNODA Atsunobu Tokyo Medical and Dental University, Dept of Otolaryngology, Associate Professor, 大学院・医歯学総合研究科, 助教授 (00280983)
KOUDA Hiroko Tokyo Medical and Dental University, Dept of Otolaryngology Faculty, 大学院・医歯学総合研究科, 助手 (80334423)
YASHIMA Takatoshi Tokyo Medical and Dental University, Dept of Otolaryngology Faculty, 医学部付属病院, 助手 (50372438)
SUMI Takurou Tokyo Medical and Dental University, Dept of Otolaryngology Faculty, 医学部付属病院, 助手 (20361701)
鵜澤 正道 東京医科歯科大学, 医学部附属病院, 助手 (10361700)
岡村 洋沖 東京医科歯科大学, 大学院・医歯学総合研究科, 教授 (50244372)
野口 佳裕 東京医科歯科大学, 大学院・医歯学総合研究科, 講師 (50282752)
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| Project Period (FY) |
2002 – 2004
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| Project Status |
Completed (Fiscal Year 2004)
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| Budget Amount *help |
¥4,000,000 (Direct Cost: ¥4,000,000)
Fiscal Year 2004: ¥1,300,000 (Direct Cost: ¥1,300,000)
Fiscal Year 2003: ¥1,300,000 (Direct Cost: ¥1,300,000)
Fiscal Year 2002: ¥1,400,000 (Direct Cost: ¥1,400,000)
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| Keywords | Gene / Genome / Neurology / Brain・Nerve / Cranial Nerve / 難聴 / ホメオボックス遺伝子 / 内耳奇形マウス / 転写因子 / Scaffold protein / Six1 / Scaffold / protein |
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| Research Abstract |
The purpose of the present research is to analyze the mechanism of deafness using the knockout mouse and patients with hereditary hearing loss. The experimental animals are mice with mutation of molecular motor and homeobox genes, respectively. The mouse with mutation of the leptin gene was also studied. We identified mutations of EYA1 gene in patients with Branchio-Oto syndrome. EYA1 genes are known to function in association with homeobox genes such as SIX1 gene. The present study demonstrated that six1 gene plays a key role in developing and differentiation of the otic vesicle. Six1 knock out mice showed no development of the inner ear, whose phenotype is quite different from human with mutation of SIX1. These phenotype-genotype difference between mouse and human is prerequisite for understanding the gene function. Jackson shaker mouse is demonstrated to have mutation of the sans, which works as a mechanosensory transduction channel in accordance with molecular motor. Leptin knockout mouse is obese and suffers from diabetes mellitus. They become deaf younger than wild animal. Light microscopic findings of the inner ear showed no abnormality in hair cells, spiral ganglion cells, and stria vascularis, even though their hearing level was already below the normal level.
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