| Project/Area Number |
11671669
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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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 | Tohoku University |
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Principal Investigator |
IKEDA Katsuhisa Tohoku University, Graduate school of Med., Associate Professor, 大学院・医学系研究科, 助教授 (70159614)
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| Co-Investigator(Kenkyū-buntansha) |
KAWASE Tetsuaki Tohoku University, Hospital, Lecture, 医学部附属病院, 講師 (50169728)
OSHIMA Takeshi Tohoku University, Graduate school of Med., Research Associate, 大学院・医学系研究科, 助手 (40241608)
吉田 尚弘 東北大学, 医学部・附属病院, 助手 (90291260)
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| Project Period (FY) |
1999 – 2000
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| Project Status |
Completed (Fiscal Year 2000)
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| Budget Amount *help |
¥2,100,000 (Direct Cost: ¥2,100,000)
Fiscal Year 2000: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1999: ¥1,300,000 (Direct Cost: ¥1,300,000)
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| Keywords | Deafness gene / Mutation / Brn / GJB2 / Konckout mice / Gene transfer / Endocochlear potential / K recysling / Brain / 転写因子 / 難聴 / ABR / 内リンパ電位 / 線維細胞 / K^+イオン / Brn-3.1 / ノックアウトマウス / 蝸牛神経 / 内リンパ静止電位 |
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| Research Abstract |
DFN3, an X-linked nonsyndromic mixed deafness is caused by mutations in BRN gene, which encodes a POU transcription factor gene. By gene targeting technology Brn-deficient mice were created and found to exhibit profound deafness. No gross morphological changes were observed in the conductive ossicles or cochlea, although there was a drastic reduction in endocochlear potential (EP). Electron microscopy revealed severe ultrastructural alterations in cochlear spiral ligament fibrocytes. These findings suggest that these fibrocytes, which are mesenchymal in origin and have been postulated to function in K^+ homeostasis, may play a critical role in auditory function and show a major cause of the hereditary deafness.
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