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Clarification of the mechanism of the cochlear nerve degeneration in an animal model of deafness

Research Project

Project/Area Number 11671669
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Otorhinolaryngology
Research InstitutionTohoku University

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)

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)
Project Period (FY) 1999 – 2000
Project Status Completed (Fiscal Year 2000)
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)
KeywordsDeafness 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
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.

Report

(3 results)
  • 2000 Annual Research Report   Final Research Report Summary
  • 1999 Annual Research Report
  • Research Products

    (22 results)

All 2000 1999 Other

All Journal Article (10 results) Publications (12 results)

  • [Journal Article] Cisplatin-induced apoptosis2000

    • Author(s)
      Ara SA et al.
    • Journal Title

      Hear Res 141

      Pages: 28-38

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Charocteuzation of novel and identified2000

    • Author(s)
      Oshima et al.
    • Journal Title

      BBRC 273

      Pages: 84-89

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Expression of connerin312000

    • Author(s)
      Yra A-P et al.
    • Journal Title

      Neuro Report 11

      Pages: 2449-2453

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Cisplatin-induced apoptosis cell death in Mongolian gerbil cochlea.2000

    • Author(s)
      Ara SA, Ikeda K^*, Oshima T, Suzuki M, Kawase T, Kikuchi T, Takasaka K.
    • Journal Title

      Hear Res 141

      Pages: 28-38

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Characterization of novel and identified genes in guinea pig organ of Corti.2000

    • Author(s)
      Oshima T^*, Nakajima T, Wada H, Ikeda. K, Takasaka T.
    • Journal Title

      Biochem Biophys Res Com 273

      Pages: 84-89

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Expression of connexin 31 in the developing mouse cochlea.2000

    • Author(s)
      Xia An-Ping^*, Ikeda K, Katori Y, Oshima T, Kikuchi T, Takasaka T.
    • Journal Title

      Neuro Report 11

      Pages: 2449-2453

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Expression and localization of Na-H1999

    • Author(s)
      Goto S
    • Journal Title

      Hear Res 128

      Pages: 89-96

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Altered cochlear tihrocytes1999

    • Author(s)
      Minoua O et al.
    • Journal Title

      Science 285

      Pages: 1408-1411

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Expression and localization of the Na+-H+exchanger in the guinea pig cochlea.1999

    • Author(s)
      Goto S, Oshima T, Ikeda, K^*, Takasaka T
    • Journal Title

      Hear Res 128

      Pages: 89-96

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Journal Article] Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness.1999

    • Author(s)
      Minowa O#,, Ikeda K#, Sugitani Y, Oshima T, Nakai S, Katori Y, Suzuki M, Furukawa M, Kawase T, Zheng Y, Ogura M, Asada Y, Watanabe K, Yamanaka H, Gotoh S, Nishi-Takeshima M, Hamada H, Sugimoto T, Kikuchi T, Takasaka T, Noda T^*.
    • Journal Title

      Science 285(#:equal contributors)

      Pages: 1408-1411

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2000 Final Research Report Summary
  • [Publications] Goto S: "Expression and localization of Na-H"Hear Res. 128. 89-96 (1999)

    • Related Report
      2000 Annual Research Report
  • [Publications] Minoua O et al: "Altered cochlear fibrocytes"Science. 285. 1408-1411 (1999)

    • Related Report
      2000 Annual Research Report
  • [Publications] Ara SA et al: "Cisplatin-induced apoptosis"Hear Res. 141. 28-38 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] Oshina et al: "Characterization of novel and identified"BBRC. 273. 84-89 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] Xra A-P et al: "Expression of connexin 31"NeuroReport. 11. 2449-2453 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] Xra A-P et al: "Expression of connexin 30"Brain Res. (印刷中).

    • Related Report
      2000 Annual Research Report
  • [Publications] Kudo et al.: "New Common mutations in the GJB2"Am J Med Genet. 90. 141-145 (2000)

    • Related Report
      1999 Annual Research Report
  • [Publications] Minowa et al.: "Altered cochlear fibrocytes in a mouse model"Science. 285. 1408-1411 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Oshima et al.: "Hearing loss with a mitochondrial gene"Laryngoscope. 109. 334-338 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Goto et al.: "Expression and localization of the Na^+-H^+ exchager"Hear Res. 128. 89-86 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Hirano et al.: "Prognosis of sudden deafness"ANL. 26. 111-115 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Ogura et al.: "Profound Hearing Loss Attributable to cochlear Nerve"Laryngoscope. 109. 1820-1824 (1999)

    • Related Report
      1999 Annual Research Report

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Published: 1999-04-01   Modified: 2016-04-21  

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