Postnatal Developmental Expression in Gjb2 Transgenic Mice of the Organ of Corti

• Project/Area Number: 20890219
• Research Category: Grant-in-Aid for Young Scientists (Start-up)
• Allocation Type: Single-year Grants
• Research Field: Otorhinolaryngology
• Research Institution: Juntendo University
• Principal Investigator: INOSHITA Ayako Juntendo University, 医学部, 助教 (00514762)
• Project Period (FY): 2008 – 2009
• Project Status: Completed (Fiscal Year 2009)
• Budget Amount: ¥3,302,000 (Direct Cost: ¥2,540,000、Indirect Cost: ¥762,000); Fiscal Year 2009: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2008: ¥1,742,000 (Direct Cost: ¥1,340,000、Indirect Cost: ¥402,000)
• Keywords: GJB2遺伝子変異 / コネキシン26 / 先天性難聴 / コルチ器 / アポトーシス / GER(内側隆起) / GJB2遺伝子 / 内耳 / コルチトンネル / モデルマウス

Research Abstract

A strikingly high proportion of congenital bilateral nonsyndromic sensorineural deafness cases among Japanese have been linked to mutations in the GJB2 coding for the connexin26. We aimed to evaluate the postnatal development of the organ of Corti in GJB2 mutations model mice. The mice showed incomplete development of the cochlear pillar cells and delayed apoptosis in GER. The present findings contribute strongly to one of basic remedies for the congenital deafness caused by Gjb2 mutation.

Research Products

• [Journal Article] Dominant negative connexin26 mutation R75W causing severe hearing loss influences normal programmed cell death in postnatal organ of Corti (2010)
  • Author(s): Ayako Inoshita, Kazusaku Kamiya, Takashi Iizuka, Masayuki Furukawa, Katsuhisa Ikeda
  • Journal Title: American Journal of Pathology (in press)
  • Peer Reviewed
• [Journal Article] Cochlear outer hair cells in a dominant-negative connexin26 mutant mouse preserve non-linear capacitance in spite of impaired distortion product otoacoustic emission (2009)
  • Author(s): A. MINEKAWA, T. ABE, A. INOSHITA, T. IIZUKA, S. KAKEHATA, Y. NARUI, T. KOIKE, K. KAMIYA, H. OKAMURA, H. SHINKAWA, K. IKEDA
  • Journal Title: Neuroscience 164 Pages: 1312-1319
• [Journal Article] Postnatal development of the organ of Corti in dominant-negative GJB2 transgenic mice (2008)
  • Author(s): A. INOSHITA, T. IIZUKA, H. OKAMURA, A. MINEKAWA, K. KOJIMA, M. FURUKAWA, T. KUSUNOKI, K. IKEDA
  • Journal Title: Neuroscience 156 Pages: 1039-1047
• [Journal Article] Postnatal development of the organ of Corti in dominant-negative Gjb2 transgenic mice. (2008)
  • Author(s): Ayako Inoshita, et al.
  • Journal Title: Neuroscience 156 Pages: 1039-1047
• [Presentation] Postnatal development of the organ of Corti in dominant-negative GJB2 transgenic mice (2009)
  • Author(s): Ayako Inoshita
  • Organizer: 32rd ARO Mid Winter Meeting
  • Place of Presentation: アメリカ, フェニックス
• [Presentation] Postnatal apoptosis of GER in dominant-negative Gjb2 transgenic mice (2009)
  • Author(s): Ayako Inoshita
  • Organizer: 7th Molecular Biology of Hearing and Deafness
  • Place of Presentation: アメリカ, ボストン
• [Presentation] Postnatal apoptosis of GER in dominant-negative Gjb2 transgenic mice (2009)
  • Author(s): Ayako Inoshita
  • Organizer: 7^<th> Molecular Biology of Hearing and Deafness
  • Place of Presentation: アメリカ、ボストン
• [Presentation] Gjb2遺伝子優性阻害変異マウスの発育段階におけるコルチ器の構造と微細構造 (2008)
  • Author(s): 井下綾子
  • Organizer: 第109回日本耳鼻咽喉科学会総会
  • Place of Presentation: 大阪
• [Presentation] Gjb2遺伝子優性阻害変異マウスの発育段階におけるコルチ器の構造と微細構造 (2008)
  • Author(s): 井下綾子
  • Organizer: 第18回日本耳科学会総会
  • Place of Presentation: 神戸
• [Presentation] 難聴遺伝子Gjb2優性阻害変異マウスの発育過程における解析と評価 (2008)
  • Author(s): 井下 綾子
  • Organizer: 日本耳鼻咽喉科学会総会・学術講演会
  • Place of Presentation: 大阪府大阪市
• [Remarks]
  • URL: http://cme.ucsd.edu/mbad/index.html