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Morphological analysis and gene therapy targeted the vestibular organ with Connexin 26 related hereditary deafness

Research Project

Project/Area Number 26462567
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Otorhinolaryngology
Research InstitutionJuntendo University

Principal Investigator

OKADA Hiroko  順天堂大学, 医学部, 助教 (20433774)

Co-Investigator(Kenkyū-buntansha) 神谷 和作  順天堂大学, 医学部, 准教授 (10374159)
飯塚 崇  順天堂大学, 医学部, 非常勤助教 (40372932)
Project Period (FY) 2014-04-01 – 2017-03-31
Project Status Completed (Fiscal Year 2016)
Budget Amount *help
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2016: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Keywords遺伝性難聴 / GJB2 / コネキシン26 / 前庭 / トランスジェニックマウス / 遺伝子治療 / アデノ随伴ウィルス / 前庭機能 / Gjb2
Outline of Final Research Achievements

We analyzed the morphological and functional development of the vestibular organ in Cx26 transgenic mice between 0 days after birth (P0) and P140, which was compared with that of littermate control mice (non-Tg). The gross structure of the inner ear in non-transgenic and transgenic mice revealed no hydrops, no defects, no degeneration in either the cochlea or the vestibule throughout the postnatal period. Light microscopic observations in the sensory epithelium revealed normally developed and matured utricle macula, saccular macula, and ampulla in the transgenic mice, which were very similar to those of the nontransgenic mouse. The present study clearly demonstrated that postnatal development and maturation in the vestibular organ were morphologically and functionally completed in Cx26 transgenic mice. Furthermore, we examined the gene transfer with adeno associated virus (AAV) via semicircular canal and successfully delivered AAV into the vestibular cells.

Report

(4 results)
  • 2016 Annual Research Report   Final Research Report ( PDF )
  • 2015 Research-status Report
  • 2014 Research-status Report
  • Research Products

    (20 results)

All 2016 2015

All Journal Article (6 results) (of which Peer Reviewed: 5 results,  Open Access: 5 results,  Acknowledgement Compliant: 2 results) Presentation (14 results) (of which Int'l Joint Research: 10 results)

  • [Journal Article] Surgical Management of Myringosclerosis over an Entire Perforated Tympanic Membrane by Simple Underlay Myringoplasty.2016

    • Author(s)
      Furukawa M, Hayashi C, Narabayashi O, Kasai M, Okada H, Haruyama T, Minekawa A, Iizuka T, Ikeda K.
    • Journal Title

      Int J Otolaryngol.

      Volume: 2016 Pages: 2894932-2894932

    • DOI

      10.1155/2016/2894932

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Postnatal Development and Maturation of the Vestibular Organ in Dominant-Negative Connexin 26 Transgenic Mouse.2015

    • Author(s)
      Hiroko Okada, Kazusaku Kamiya., Takashi Iizuka and Katsuhisa Ikeda.
    • Journal Title

      J Otol Rhinol

      Volume: S1 Pages: 37-40

    • DOI

      10.4172/2324-8785.s1-009

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Deformation of the Outer Hair Cells and the Accumulation of Caveolin-2 in Connexin 26 Deficient Mice.2015

    • Author(s)
      Anzai, T., Fukunaga, I., Hatakeyama, K., Fujimoto, A., Kobayashi, K., Nishikawa, A., Aoki, T., Noda, T., Minowa, O., Ikeda, K., and Kamiya, K
    • Journal Title

      PloS one

      Volume: 10 Issue: 10 Pages: e0141258-e0141258

    • DOI

      10.1371/journal.pone.0141258

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Differentiation of iPS Cells to Cochlear Cells are Regulated Depending on the Part of Co-cultured Organs.2015

    • Author(s)
      Kazusaku Kamiya, Keiko Karasawa, Kazuma Kobayashi, Asuka Miwa and Katsuhisa Ikeda.
    • Journal Title

      J Otol Rhinol

      Volume: S1 Pages: 34-36

    • DOI

      10.4172/2324-8785.s1-008

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness.2015

    • Author(s)
      Izuka T., Kamiya K., Gotoh S., Sugitani Y., Suzuki M., Noda T., Minowa O., Ikeda K.
    • Journal Title

      Hum. Mol.Genet.

      Volume: 24)13) Issue: 13 Pages: 3651-3661

    • DOI

      10.1093/hmg/ddv109

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Connexin26 regulates assembly and maintenance of cochlear gap junction macromolecular complex for normal hearing2015

    • Author(s)
      Kazusaku Kamiya, Ichiro Fukunaga, Kaori Hatakeyama and Katsuhisa Ikeda
    • Journal Title

      AIP Conference Proceedings

      Volume: 1703 Pages: 030018-030018

    • DOI

      10.1063/1.4939333

    • Related Report
      2015 Research-status Report
    • Acknowledgement Compliant
  • [Presentation] GJB2変異遺伝性難聴に対する細胞治療・遺伝子治療法の開発2016

    • Author(s)
      神谷 和作
    • Organizer
      第15回日本再生医療学会総会
    • Place of Presentation
      大阪国際会議場(大阪市)
    • Year and Date
      2016-03-17
    • Related Report
      2015 Research-status Report
  • [Presentation] iPS細胞からのConnexin26発現細胞への分化誘導と細胞間ギャップ結合プラークの構築2016

    • Author(s)
      福永一朗、畠山佳欧里、 青木徹、藤本あゆみ、 西川貴菜、美野輪治、 池田 勝久、神谷和作
    • Organizer
      第15回日本再生医療学会総会
    • Place of Presentation
      大阪国際会議場(大阪市)
    • Year and Date
      2016-03-17
    • Related Report
      2015 Research-status Report
  • [Presentation] コネキシン26欠損マウスからのiPS細胞の樹立2016

    • Author(s)
      青木徹、福永一朗、 畠山佳欧里、藤本あゆみ、西川貴菜、美野輪治、 池田勝久、神谷和作
    • Organizer
      第15回日本再生医療学会総会
    • Place of Presentation
      大阪国際会議場(大阪市)
    • Year and Date
      2016-03-17
    • Related Report
      2015 Research-status Report
  • [Presentation] 内耳フィーダー細胞を用いたiPS細胞の分化誘導によるCx26ギャップ結合形成細胞の樹立2016

    • Author(s)
      藤本あゆみ、福永一朗、 畠山佳欧里、青木徹、 西川貴菜、池田勝久、 神谷 和作
    • Organizer
      第15回日本再生医療学会総会
    • Place of Presentation
      大阪国際会議場(大阪市)
    • Year and Date
      2016-03-17
    • Related Report
      2015 Research-status Report
  • [Presentation] Restoration og Cochlear Gap Junction for GJB22016

    • Author(s)
      Kazusaku Kamiya, Ichiro Fukunaga
    • Organizer
      Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
    • Place of Presentation
      Manchester Grand Hyatt, San Diego, CA, USA
    • Year and Date
      2016-02-20
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Deformation of the Outer Hair Cells and the Accumulation of Caveolin-2 in Connexin 26-Deficient Mice2016

    • Author(s)
      Takashi Anzai, Kazusaku Kamiya, Katsuhisa Ikeda
    • Organizer
      Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
    • Place of Presentation
      Manchester Grand Hyatt, San Diego, CA, USA
    • Year and Date
      2016-02-20
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Generation of induced pluripoten stem cells form connexin26 conditional knock out mouce2016

    • Author(s)
      Toru Aoki, Katsuhisa Ikeda, Kazusaku Kamiya
    • Organizer
      Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
    • Place of Presentation
      Manchester Grand Hyatt, San Diego, CA, USA
    • Year and Date
      2016-02-20
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Differentiation of mouse iPS cell into Cx26-positive cell and formation of inter cellular Cx26-gap junction plaque2016

    • Author(s)
      Ichiro Fukunaga, Kaori Hatakeyama,Toru Aoki, Atena Nishikawa, Ayumi Fujimoto, Katsuhisa Ikeda, Kazusaku Kamiya
    • Organizer
      Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
    • Place of Presentation
      Manchester Grand Hyatt, San Diego, CA, USA
    • Year and Date
      2016-02-20
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Induction of iPS cell differentiation into Connexin26 positive cells with gap junction plaques using cochlear feeder cell2016

    • Author(s)
      Ayumi Fujimoto, Ichiro Fukunaga, Kaori Hatakeyama, Toru Aoki, Atena Nishikawa, Katsuhisa Ikeda, Kazusaku Kamiya
    • Organizer
      Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
    • Place of Presentation
      Manchester Grand Hyatt, San Diego, CA, USA
    • Year and Date
      2016-02-20
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Various cochlear gap junction plaque reproduced in human cell line2016

    • Author(s)
      Kaori Hatakeyama, Katsuhisa Ikeda, Kazusaku Kamiya
    • Organizer
      Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
    • Place of Presentation
      Manchester Grand Hyatt, San Diego, CA, USA
    • Year and Date
      2016-02-20
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Cochlear gap junction plaque, stabilized macromolecular complex composed of specific connexins2015

    • Author(s)
      Kazusaku Kamiya, Ichiro Fukunaga, Kaori Hatakeyama, Toru Aoki, Ayumi Fujimoto, Atena Nishikawa ,Takashi Anzai, Osamu Minowa, Katsuhisa Ikeda
    • Organizer
      52nd Inner Ear Biology Workshop
    • Place of Presentation
      Campus of the Catholic University of Rome, Italy
    • Year and Date
      2015-09-12
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Caveolins accumulates at the organ of Corti in GJB2 associated deafness2015

    • Author(s)
      Takashi Anzai, Kazusaku Kamiya, Katsuhisa Ikeda
    • Organizer
      52nd Inner Ear Biology Workshop
    • Place of Presentation
      Campus of the Catholic University of Rome, Italy
    • Year and Date
      2015-09-12
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Reproduction of various types of cochlear gap junction plaques in human cell line2015

    • Author(s)
      Kaori Hatakeyama, Katsuhisa Ikeda, Kazusaku Kamiya
    • Organizer
      52nd Inner Ear Biology Workshop
    • Place of Presentation
      Campus of the Catholic University of Rome, Italy
    • Year and Date
      2015-09-12
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Differentiation of mouse iPS cell into Cx26-positive cell and formation of inter cellular Cx26-gap junction plaque2015

    • Author(s)
      Ichiro Fukunaga, Kaori Hatakeyama,Toru Aoki, Atena Nishikawa, Ayumi Fujimoto, Osamu Minowa, Katsuhisa Ikeda, Kazusaku Kamiya
    • Organizer
      52nd Inner Ear Biology Workshop
    • Place of Presentation
      Campus of the Catholic University of Rome, Italy
    • Year and Date
      2015-09-12
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research

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Published: 2014-04-04   Modified: 2018-03-22  

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