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Spiral ganglion degeneration in a mouse model of auditory neuropathy spectrum disorder

Research Project

Project/Area Number 24791819
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Otorhinolaryngology
Research Institution独立行政法人国立病院機構(東京医療センター臨床研究センター)

Principal Investigator

NAMBA Kazunori  独立行政法人国立病院機構(東京医療センター臨床研究センター), 聴覚・平衡覚研究部, 研究員 (60425684)

Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2013: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2012: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
KeywordsOTOF / Otoferlin / Auditory Neuropathy / ANSD / 蝸牛神経節 / Spiral Ganglion / 神経細胞 / neurodegeneration / 蝸牛 / BDNF / TUNEL / 国際情報交換, 対象国:米国,フランス
Outline of Final Research Achievements

Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder in which sound enters the inner ear normally but the transmission of signals from the inner ear to the brain is impaired. In this study, we compared the number of spiral ganglion (SG) neurons between otoferlin deficient (OTOF-/-) and normal mice in developmental stage.
Comparing to normal SG, the number of afferent type-1 neuron for OTOF-/- was apoptically decreased from post-natal day 7, and it over 50% in 12 weeks of age. However, a decrease was not seen in efferent type-II neuron.
Because the outer hair cell is mainly projected by efferent nerve, these data suit the definition of ANSD, and it is useful as the animal model of disease of ANSD.

Report

(4 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • 2012 Research-status Report
  • Research Products

    (2 results)

All 2013

All Presentation (2 results)

  • [Presentation] Auditory Neuropathyの特徴を示すOTOF遺伝子欠損マウスを用いた蝸牛神経低形成の新規モデル2013

    • Author(s)
      難波一徳、藤井正人、加我君孝、松永達雄
    • Organizer
      日本生化学会
    • Place of Presentation
      パシフィコ横浜
    • Related Report
      2013 Research-status Report
  • [Presentation] Auditory Neuropathyの特徴を示すOTOF遺伝子欠損マウスを用いた蝸牛神経低形成の新規モデル2013

    • Author(s)
      難波一徳、藤井正人、加我君孝、松永達雄
    • Organizer
      第86回日本生化学会大会
    • Place of Presentation
      パシフィコ横浜
    • Related Report
      2012 Research-status Report

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Published: 2013-05-31   Modified: 2019-07-29  

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