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Establishment and characterization of mouse models for hearing loss based on Molecular interaction

Research Project

Project/Area Number 20300147
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Laboratory animal science
Research InstitutionTokyo University of Agriculture

Principal Investigator

KIKKAWA Yoshiaki  Tokyo University of Agriculture, 生物産業学部, 准教授 (20280787)

Co-Investigator(Kenkyū-buntansha) SHITARA Hiroshi  (財)東京都医学研究機構, 東京都臨床医学総合研究所, 研究員 (90321885)
NOGUCHI Yoshihiro  東京医科歯科大学, 医学部付属病院, 講師 (50282752)
Research Collaborator STEVE Brown  英国MRC, Mammalian Genetics Unit, 教授
奥村 和弘  東京農業大学, 大学院, 博士後期課程
Project Period (FY) 2008 – 2010
Project Status Completed (Fiscal Year 2010)
Budget Amount *help
¥19,630,000 (Direct Cost: ¥15,100,000、Indirect Cost: ¥4,530,000)
Fiscal Year 2010: ¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2009: ¥5,460,000 (Direct Cost: ¥4,200,000、Indirect Cost: ¥1,260,000)
Fiscal Year 2008: ¥9,100,000 (Direct Cost: ¥7,000,000、Indirect Cost: ¥2,100,000)
Keywords育種遺伝 / 分子間相互作用 / 難聴 / マウス / 有毛細胞 / Stereocilia / 難聴発症機構 / ヒト難聴モデルマウス / ミオシンVI / カドヘリン23 / 加齢性難聴 / 修飾遺伝子 / ゲルソリン / DBA / 2J / 4.1蛋白質 / Sans / Whirlin / Myosin VI / マウス亜種間交配
Research Abstract

To establish new mouse models for deafness and identify molecular mechanism of hearing impairment, we carried out positional cloning approach, expression analysis of the inner ear hair cells and protein-protein interaction studies among the deafness associated-proteins. 1) We identified that responsible gene of two new spontaneous mutants is myosin VI. 2) 4.1B and gelsolin may be members of the myosin XV-whirlin complex that is important for stereocilia maturation. 3) Phenotypic data of double homozygotes between Sans and Whrlin mutants suggest that the function of SANS interacts with WHRLIN and the interaction play regulation of planar cell polarity in stereocilia of the hair cells.

Report

(4 results)
  • 2010 Annual Research Report   Final Research Report ( PDF )
  • 2009 Annual Research Report
  • 2008 Annual Research Report
  • Research Products

    (47 results)

All 2010 2009 2008 Other

All Journal Article (7 results) (of which Peer Reviewed: 7 results) Presentation (37 results) Book (1 results) Remarks (2 results)

  • [Journal Article] Protein 4.1 expression in the developing hair cells of the mouse inner ear.2010

    • Author(s)
      Okumura K., Mochizuki E., Yokohama M., Yamakawa H., Shitara H., Mburu P., Yonekawa H., Brown S.D.M., Kikkawa Y.
    • Journal Title

      Brain Res. 1307

      Pages: 53-62

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Phenotypic and expression analysis of a novel spontaneous Myosin VI null mutant mouse.2010

    • Author(s)
      Mochizuki E., Okumura K., Ishikawa M., Yoshimoto S., Yamaguchi J., Seki Y., Wada K., Yokohama M., Ushiki T., Tokano H., Ishii R., Shitara H., Taya C., Kitamura K., Yonekawa H., Kikkawa Y.
    • Journal Title

      Exp.Anim. 59

      Pages: 57-71

    • NAID

      10027852619

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Gelsolin plays a role in the actin polymerization complex of hair cell stereocilia.2010

    • Author(s)
      Mburu P., Romero M., Hilton H., Parker A., Townsend S., Kikkawa Y., Brown S.D.M.
    • Journal Title

      PLoS One 5

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Gelsolin plays a role in the actin polymerization complex of hair cell stereocilia2010

    • Author(s)
      Mburu P.
    • Journal Title

      PLoS One

      Volume: 5

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Phenotypic and expression analysis of a novel spontaneous Myosin VI null mutant mouse2010

    • Author(s)
      Eiji Mochizuki, Kazuhiro Okumura, Masashi Ishikawa, Sachi Yoshimoto, Junya Yamaguchi, Yuta Seki, Kenta Wada, Michinari Yokohama, Tatsuo Ushiki, Hisashi Tokano, Rie Ishii, Hiroshi Shitara, Choji Taya, Ken Kitamura, Hiromichi Yonekawa, Yoshiaki Kikkawa
    • Journal Title

      Exp. Anim. 59

      Pages: 57-71

    • NAID

      10027852619

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Protein 4.1 expression in the developing hair cells of the mouse inner ear2010

    • Author(s)
      Kazuhiro Okumura, Eiji Mochizuki, Michinari Yokohama, Hisao Yamakawa, Hiroshi Shitara, Philomena Mburu, Hiromichi Yonekawa, Steve D.M.Brown, Yoshiaki Kikkawa
    • Journal Title

      Brain Res. 1307

      Pages: 53-62

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A novel hairless mouse model on an atopic dermatitis-prone genetic background generated by receptor-mediated transgenesis.2008

    • Author(s)
      Toyoyuki Takada
    • Journal Title

      Transgenic Res. 17

      Pages: 1155-1162

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Presentation] Gelsolin plays a role in the actin polymerization complex of hair cell stereocilia2010

    • Author(s)
      Mburu P.
    • Organizer
      24^<rd> International Mammalian Genome Conference
    • Place of Presentation
      Heraklion, Crete, Greece
    • Year and Date
      2010-10-19
    • Related Report
      2010 Final Research Report
  • [Presentation] 新規自然発症ミオシンVI欠損マウスの表現型および発現解析2010

    • Author(s)
      尾崎真央
    • Organizer
      日本遺伝学会第82回大会
    • Place of Presentation
      北海道大学
    • Year and Date
      2010-09-20
    • Related Report
      2010 Final Research Report
  • [Presentation] マウスの早発性難聴発症に関する第5番染色体上の複数の感受性遺伝子2010

    • Author(s)
      鈴木沙理
    • Organizer
      日本遺伝学会第82回大会
    • Place of Presentation
      北海道大学
    • Year and Date
      2010-09-20
    • Related Report
      2010 Final Research Report
  • [Presentation] SansおよびWhirlinの2重変異マウスは内耳感覚毛表面の感覚毛極性異常を示す2010

    • Author(s)
      吉川欣亮
    • Organizer
      日本遺伝学会第82回大会
    • Place of Presentation
      北海道大学
    • Year and Date
      2010-09-20
    • Related Report
      2010 Final Research Report
  • [Presentation] 近交系マウスDBA2/Jの早発性難聴に関与するCdh23と相互作用する修飾遺伝子の同定2010

    • Author(s)
      鈴木沙理
    • Organizer
      第57回日本実験動物学会総会
    • Place of Presentation
      京都テルサ
    • Year and Date
      2010-05-13
    • Related Report
      2010 Final Research Report
  • [Presentation] マウスを用いた加齢性難聴の遺伝学的解析2010

    • Author(s)
      吉川欣亮
    • Organizer
      国立遺伝学研究所研究会「Mouse Forward Geneticsの新潮流」
    • Place of Presentation
      国立遺伝学研究所
    • Year and Date
      2010-04-23
    • Related Report
      2010 Final Research Report
  • [Presentation] マウスを用いた加齢性難聴の遺伝学的解析2010

    • Author(s)
      吉川欣亮
    • Organizer
      国立遺伝学研究所研究会「Mouse Forward Geneticsの新潮流」
    • Place of Presentation
      国立遺伝学研究所(三島)
    • Year and Date
      2010-04-23
    • Related Report
      2010 Annual Research Report
  • [Presentation] Gelsolin plays a role in the actin polymerization complex of hair cell stereocilia2010

    • Author(s)
      Mburu P.
    • Organizer
      24^<th> International Mammalian Genome Conference
    • Place of Presentation
      Heraklion, Crete, Greece
    • Related Report
      2010 Annual Research Report
  • [Presentation] 新規自然発症ミオシンVI欠損マウスの表現型および発現解析2010

    • Author(s)
      尾崎真央
    • Organizer
      日本遺伝学会第82回大会
    • Place of Presentation
      北海道大学(札幌)
    • Related Report
      2010 Annual Research Report
  • [Presentation] マウスの早発性難聴発症に関する第5番染色体上の複数の感受性遺伝子2010

    • Author(s)
      鈴木沙理
    • Organizer
      日本遺伝学会第82回大会
    • Place of Presentation
      北海道大学(札幌)
    • Related Report
      2010 Annual Research Report
  • [Presentation] SansおよびWhirlinの2重変異マウスは内耳感覚毛表面の感覚毛極性異常を示す2010

    • Author(s)
      吉川欣亮
    • Organizer
      日本遺伝学会第82回大会
    • Place of Presentation
      北海道大学(札幌)
    • Related Report
      2010 Annual Research Report
  • [Presentation] 近交系マウスDBA2/Jの早発性難聴に関与するCdh23と相互作用する修飾遺伝子の同定2010

    • Author(s)
      鈴木沙理
    • Organizer
      第57回日本実験動物学会総会
    • Place of Presentation
      京都テルサ(京都)
    • Related Report
      2010 Annual Research Report
  • [Presentation] ヒト難聴モデルマウスを用いた加齢性難聴の感受性遺伝子の同定および内耳表現型解析2010

    • Author(s)
      吉本沙智
    • Organizer
      第57回日本実験動物学会総会
    • Place of Presentation
      京都テルサ(京都)
    • Related Report
      2010 Annual Research Report
  • [Presentation] Whirlin and Sans functions in planar cell polarity regulation at the apical surface of inner ear hair cells.2009

    • Author(s)
      Okumura K.
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜
    • Year and Date
      2009-12-11
    • Related Report
      2010 Final Research Report
  • [Presentation] Whirlin and Sans functions in planar cell polarity regulation at the apical surface of inner ear hair cells.2009

    • Author(s)
      Kazuhiro Okumura, Yuki Miyasaka, Yasuhiro Oshiba, Yoshihiro Noguchi, Ken Kitamura, Philomena Mburu, Steve D.M.Brown, Hiromichi Yonekawa, Yoshiaki Kikkawa
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜(横浜)
    • Year and Date
      2009-12-11
    • Related Report
      2009 Annual Research Report
  • [Presentation] Mapping of quantitative trait loci for early onset hearing loss in DBA/2J mice.2009

    • Author(s)
      Suzuki S.
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜
    • Year and Date
      2009-12-10
    • Related Report
      2010 Final Research Report
  • [Presentation] Mapping of quantitative trait loci for early onset hearing loss in DBA/2J mice.2009

    • Author(s)
      Sari Suzuki, Takuya Ueda, Masashi Ishikawa, Kazuhiro Okumura, Osamu Minowa, Shigeharu Wakana, Hiromichi Yonekawa, Yoshiaki Kikkawa
    • Organizer
      第32回日本分子生物学会年会
    • Place of Presentation
      パシフィコ横浜(横浜)
    • Year and Date
      2009-12-10
    • Related Report
      2009 Annual Research Report
  • [Presentation] Significant correlation between hearing defect and double hetero-/homozygosty for mouse mutant in WHIRLIN and SANS.2009

    • Author(s)
      吉川欣亮
    • Organizer
      23^<rd> International Mammalian Genome Conference
    • Place of Presentation
      La Jolla, California
    • Year and Date
      2009-11-02
    • Related Report
      2010 Final Research Report 2009 Annual Research Report
  • [Presentation] Difference in hearing ability among Ednrb down-regulated JF1 micecoincides with melanocyte survival of cochlear stria vascularis2009

    • Author(s)
      Kazuhiro Okumura, Yuki Miyasaka, Tetsuo Kunieda, Tsuyoshi Koide, Toshihiko Shiroishi, Hiromichi Yonekawa, Yoshiaki Kikkawa
    • Organizer
      23^<rd> International Mammalian Genome Conference
    • Place of Presentation
      La Jolla, California
    • Year and Date
      2009-11-02
    • Related Report
      2009 Annual Research Report
  • [Presentation] 脊椎動物の内耳有毛細胞におけるMyosin VIの発現分布2009

    • Author(s)
      吉川欣亮
    • Organizer
      日本遺伝学会第81回大会
    • Place of Presentation
      信州大学
    • Year and Date
      2009-09-17
    • Related Report
      2010 Final Research Report
  • [Presentation] マウスEdnrb突然変異体JF1の聴力個体差は蝸牛管血管条のメラノサイト生存分布と一致する2009

    • Author(s)
      宮坂勇輝, 奥村和弘, 上原重之、 山本博章, 小出剛, 城石俊彦, 米川博通, 吉川欣亮
    • Organizer
      日本遺伝学会第81回大会
    • Place of Presentation
      信州大学理学部(松本)
    • Year and Date
      2009-09-17
    • Related Report
      2009 Annual Research Report
  • [Presentation] 脊椎動物の内耳有毛細胞におけるMyosin VIの発現分布2009

    • Author(s)
      吉川欣亮、松原創、米川博通、望月英志
    • Organizer
      日本遺伝学会第81回大会
    • Place of Presentation
      信州大学理学部(松本)
    • Year and Date
      2009-09-17
    • Related Report
      2009 Annual Research Report
  • [Presentation] 加齢性難聴の原因となるカドヘリン23多型と相互作用するQTLs.2009

    • Author(s)
      植田卓也
    • Organizer
      第56回日本実験動物学会総会
    • Place of Presentation
      大宮ソニックホール
    • Year and Date
      2009-05-15
    • Related Report
      2010 Final Research Report
  • [Presentation] JF1マウスの聴力および聴覚組織形態の個体差2009

    • Author(s)
      奥村和弘
    • Organizer
      第56回日本実験動物学会総会
    • Place of Presentation
      大宮ソニックホール
    • Year and Date
      2009-05-15
    • Related Report
      2010 Final Research Report
  • [Presentation] 加齢性難聴の原因となるカドヘリン23多型と相互作用するQTLs2009

    • Author(s)
      植田卓也, 石川昌志, 吉本沙智, 奥村和弘, 鈴木沙理, 尾崎真央, 美野輪治, 若菜茂晴, 米川博通, 吉川欣亮
    • Organizer
      第56回日本実験動物学会総会
    • Place of Presentation
      大宮ソニックホール(大宮)
    • Year and Date
      2009-05-15
    • Related Report
      2009 Annual Research Report
  • [Presentation] JF1マウスの聴力および聴覚組織形態の個体差2009

    • Author(s)
      奥村和弘, 宮坂勇輝, 松島芳文, 赤松紀子, 小出剛, 城石俊彦, 米川博通, 吉川欣亮
    • Organizer
      第56回日本実験動物学会総会
    • Place of Presentation
      大宮ソニックホール(大宮)
    • Year and Date
      2009-05-15
    • Related Report
      2009 Annual Research Report
  • [Presentation] 内耳感覚毛形成に関与するスカフォールド蛋白質SANSおよびWhirlinの相互作用2008

    • Author(s)
      奥村和弘
    • Organizer
      BMB2008
    • Place of Presentation
      神戸ポートアイランド
    • Year and Date
      2008-12-10
    • Related Report
      2010 Final Research Report
  • [Presentation] 脊椎動物の内耳形成時における非筋肉性ミオシン蛋白質の機能2008

    • Author(s)
      望月英志
    • Organizer
      BMB2008
    • Place of Presentation
      神戸ポートアイランド
    • Year and Date
      2008-12-10
    • Related Report
      2010 Final Research Report
  • [Presentation] 近交系マウスDBA/2Jの加齢に伴う難聴発症原因遺伝子のマッピング2008

    • Author(s)
      石川昌志
    • Organizer
      BMB2008
    • Place of Presentation
      神戸ポートアイランド
    • Year and Date
      2008-12-10
    • Related Report
      2010 Final Research Report
  • [Presentation] マウス内耳有毛細胞発生過程におけるMyosin VIの機能2008

    • Author(s)
      望月英志
    • Organizer
      日本実験動物科学技術2008
    • Place of Presentation
      仙台国際センター
    • Year and Date
      2008-05-15
    • Related Report
      2010 Final Research Report
  • [Presentation] 内耳感覚毛形成異常マウスを用いた4.1タンパク質の免疫組織化学的解析2008

    • Author(s)
      奥村和弘
    • Organizer
      日本実験動物科学技術2008
    • Place of Presentation
      仙台国際センター
    • Year and Date
      2008-05-15
    • Related Report
      2010 Final Research Report
  • [Presentation] マウス内耳有毛細胞発生過程におけるMyosin VIの機能2008

    • Author(s)
      望月英志
    • Organizer
      日本実験動物科学技術2008
    • Place of Presentation
      仙台
    • Related Report
      2008 Annual Research Report
  • [Presentation] 内耳感覚毛形成異常マウスを用いた4.1タンパク質の免疫組織化学的解析2008

    • Author(s)
      奥村和弘
    • Organizer
      日本実験動物科学技術2008
    • Place of Presentation
      仙台
    • Related Report
      2008 Annual Research Report
  • [Presentation] 実験用マウス近交系のミトコンドリアDNA多型2008

    • Author(s)
      吉川欣亮
    • Organizer
      日本実験動物科学技術2008
    • Place of Presentation
      仙台
    • Related Report
      2008 Annual Research Report
  • [Presentation] 内耳感覚毛形成に関与するスカフォールド蛋白質SANSおよびWhirlinの相互作用2008

    • Author(s)
      奥村和弘
    • Organizer
      BMB2008
    • Place of Presentation
      神戸
    • Related Report
      2008 Annual Research Report
  • [Presentation] 脊椎動物の内耳形成時における非筋肉性ミオシン蛋白質の機能2008

    • Author(s)
      望月英志
    • Organizer
      BMB2008
    • Place of Presentation
      神戸
    • Related Report
      2008 Annual Research Report
  • [Presentation] 近交系マウスDBA/2Jの加齢に伴う難聴発症原因遺伝子のマッピング2008

    • Author(s)
      石川昌志
    • Organizer
      BMB2008
    • Place of Presentation
      神戸
    • Related Report
      2008 Annual Research Report
  • [Book] 感覚器 聴覚;脳・神経系;疾患モデルマウス:臓器・疾患別解析・実験法;生命科学研究の戦略:疾患モデルマウスと表現型

    • Author(s)
      吉川欣亮・鈴木沙理・野口佳裕
    • Publisher
      中山書店(印刷中)
    • Related Report
      2010 Final Research Report
  • [Remarks] ホームページ等

    • URL

      http://nodai.cc-town.net/laboratory/single.php?id=115

    • Related Report
      2010 Final Research Report
  • [Remarks]

    • URL

      http://nodai.cc-town.net/laboratory/single.php?id=115

    • Related Report
      2009 Annual Research Report

URL: 

Published: 2008-04-01   Modified: 2016-04-21  

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