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Origin of endocochlear potential studied with mouse model for human heredetary nonsyndromic deafness.

Research Project

Project/Area Number 12480253
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Laboratory animal science
Research InstitutionRIKEN

Principal Investigator

MINOWA Osamu  RIKEN mutation phenotype exploration team Senior research scientist, マウス変異探索研究チーム, 上級研究員 (00181967)

Co-Investigator(Kenkyū-buntansha) YAO Ryouji  Cancer Institute Department of cell biology Member Associate, 癌研究所, 研究員 (80291095)
Project Period (FY) 2000 – 2001
Project Status Completed (Fiscal Year 2001)
Budget Amount *help
¥16,500,000 (Direct Cost: ¥16,500,000)
Fiscal Year 2001: ¥5,300,000 (Direct Cost: ¥5,300,000)
Fiscal Year 2000: ¥11,200,000 (Direct Cost: ¥11,200,000)
KeywordsEndocochlear Potential / targeted Gene Knock out / Conditional Gene Targeting / Connexin 26 / Nonsyndromic Deafness / DFNB1 / DFNA3 / 聴性脳幹反応 / 標的遺伝子組替え
Research Abstract

The endocochlear potential (EP) is remarkable electrostatic potential observed between lymph of cochlear duct and that of neighboring duct scala tympani in inner ear. This electro-physiological character is known as specific for higher vertebrate especially in mammalian species and to be essential for their cochlear function. About the origin, the process of establishment, and ways of maintenance of EP, however, are still little known.
Recently, Gap junction component connexin 26 (Cx26) is thought to have pivotal role in maintenance of EP as well as transcription factor Brn-4 does. Cx26 is also known as responsive gene for human nonsyndromic deafness DFNB1 and DFNA3. To better understand the mechanism of emergence of EP and to investigate the roles of Cx26 in conjunction with Brn-4, we introduced conditional gene targeting technology using cre-lfcxP system to produce "Cx26-conditional-KO" mice with "silent" alleles of Cx26 those were assumed to work normally in vivo and then induced deletion of Cx26 gene in spatio-temporally dependent manner.
By crossing Cx26-conditional-KO mice with PO-Cre transgenic mice, we attempted to eliminate Cx26 gene from whole inner ear tissue cells in their offspring mice of adult age. Auditory brainstem response measurement showed that Cx26-Silent-Allele : O/P0-Cre+ mice were profound deafness whereas Cx26-Silent-Allele:E/PO-Cre+ mice were normal. These results indicate that, like in the case of human nonsyndromic deafness, Cx26 gene is essential for mouse inner ear function and suggest Cx26-Silent-Allele:E/PO-Cre+ mouse could be a model for human DFNB1.

Report

(3 results)
  • 2001 Annual Research Report   Final Research Report Summary
  • 2000 Annual Research Report
  • Research Products

    (22 results)

All Other

All Publications (22 results)

  • [Publications] O.Minowa et al.: "Mmh/Oggl gene inactivation results in accumulation of 8-hydroxyguanine in mice"Proc. Natl. Acad. Sci. USA. 97(8). 4156-4161 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] H.Beppu, O.Minowa et al.: "BMP Type II receptor is required for gastrulation and early development of mouse embryos"Dev. Biol.. 221(1). 249-258 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] H.Saegusa, O.Minowa et al.: "Altered pain responses in mice lacking alpha 1E subunit of the voltage-dependent Ca2+ channel"Proc. Natl. Acad. Sci. USA. 97(11). 6132-6137 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Y.Yoshida, O.Minowa et al.: "Negative regulation of BMP/Smad signaling by Tob in osteoblasts"Cell. 103. 1085-1097 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] K.Tobiume, O.Minowa et al.: "ASK1 is required for sustained activations of JNK/p38 MAP kinases and apoptosis"EMBO Rep.. 2(3). 222-228 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] AP.Xia, O.Minowa et al.: "Late-onset hearing loss in a mouse model of DFN3 non-syndromic deafness : morphologic and immunohistochemical analyses"Hear Res.. 166(1):. 150-158 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] O. Minowa et al.: "Mmh/Oggl gene inactivation results in accumulation of 8-hydroxyguanine in mice"Proc.Natl. Acad. Sci. USA. 97(8). 4156-4161 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] H. Beppu, O. Minowa et al: "BMP Type II receptor is required for gastrulation and early development of mouse embryos"Dev. Biol.. 221(1). 249-258 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] H. Saegusa, O. Minowa et al: "Altered pain responses in mice lacking alpha 1E subunit of the voltage-dependent Ca2+ channel"Proc.Natl. Acad. Sci. USA. 97(11). 6132-6137 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Y.Yoshida, O. Minowa et al: "Negative regulation of BMP/Smad signaling by Tob in osteoblasts"Cell. 103. 1085-1097 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] K.Tobiume, O. Minowa et al.: "ASK1 is required for sustained activations of JNK/p38 MAP kinases and apoptosis"EMBO Rep.. 2(3). 222-228 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] AP.Xia, O ,Minowa et al.: "Late-onset hearing loss in a mouse model of DFN3 non-syndromic deafness : morphologic and immunohistochemical analyses"Hear Res.. 166(1). 150-158 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] O.Minowa et al.: "Mmh/Ogg1 gene inactivation results in accumulation of 8-hydroxyguanine in mice"Proc. Natl. Acad. Sci. USA. 97(8). 4156-4161 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] H.Beppu, O.Minowa et al.: "BMP Type II receptor is required for gastrulation and early development of mouse embryos"Developmental Biology. 221(1). 249-258 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] H.Saegusa, O.Minowa et al.: "Altered pain responses in mice lacking alpha 1E subunit of the voltage-dependent Ca2+ channel"Proc. Natl. Acad. Sci. USA. 97(11). 6132-6137 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] Y.Yoshida, O.Minowa et al.: "Negative regulation of BMP/Smad signaling by Tob in osteoblasts"Cell. 103. 1085-1097 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] T.Nagai, O.Minowa et al.: "Zic2 regulates the kinetics of neurulation"Proc. Natl. Acad. Sci. USA. 97(4). 1618-1623 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] K.Tobiume, O.Minowa et al.: "ASK1 is required for sustained activations of JNK/p38 MAP kinases and apoptosis."EMBO Rep.. 2(3). 222-228 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] O.Minowa et al.: "Mmh/Oggl gene inactivation results in accumulation of 8-hydroxyguanine in mice."Proc.Natl.Acad.Sci.USA. 79・8. 4156-4161 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] H.Beppu,O.Minowa et al.: "BMP Type II receptor is required for gastrulation and early development of mouse embryos."Dev.Biol.. 221・1. 249-258 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] H.Saegusa,O.Minowa et al: "Altered pain responses in mice lacking alpha 1E subunit of the voltage-dependent Ca2+ channel."Proc.Natl.Acad.Sci.USA. 97・11. 6132-6137 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] Y.Yoshida,O.Minowa et al: "Negative regulation of BMP/Smad signaling by Tob in osteoblasts."Cell. 103・. 1085-1097 (2000)

    • Related Report
      2000 Annual Research Report

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Published: 2000-04-01   Modified: 2016-04-21  

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