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Exploration of phenomena indicative of mitochondrial dysfunction and its applications

Research Project

Project/Area Number 18H03180
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Review Section Basic Section 59040:Nutrition science and health science-related
Research InstitutionKyushu University

Principal Investigator

Matsushima Yuichi  九州大学, 医学研究院, 助教 (20571342)

Co-Investigator(Kenkyū-buntansha) 瀬戸山 大樹  九州大学, 大学病院, 助教 (30550850)
相原 正宗  九州大学, 大学病院, 臨床検査技師 (30748843)
内海 健  九州大学, 医学研究院, 教授 (80253798)
Project Period (FY) 2018-04-01 – 2022-03-31
Project Status Completed (Fiscal Year 2021)
Budget Amount *help
¥17,290,000 (Direct Cost: ¥13,300,000、Indirect Cost: ¥3,990,000)
Fiscal Year 2021: ¥3,380,000 (Direct Cost: ¥2,600,000、Indirect Cost: ¥780,000)
Fiscal Year 2020: ¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2019: ¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2018: ¥5,590,000 (Direct Cost: ¥4,300,000、Indirect Cost: ¥1,290,000)
Keywordsミトコンドリア / タンパク質凝集
Outline of Final Research Achievements

It has been suggested that mitochondrial dysfunction triggers the onset of aging and lifestyle-related diseases. However, a simple and reproducible method to evaluate mitochondrial dysfunction such as decreased ATP synthesis has not been found. In this study, we focused on LONP1, a protease localized in the mitochondrial matrix, and found that LONP1 functions in a manner dependent on ATP hydrolysis and that its dysfunction causes the accumulation of protein aggregates in the mitochondrial matrix. This indicates that the accumulation of protein aggregates may be an indicator of abnormal mitochondrial function accompanied by a decrease in the amount of ATP in the mitochondrial matrix.

Academic Significance and Societal Importance of the Research Achievements

本研究はミトコンドリア機能異常をタンパク質凝集体の蓄積を介して評価するというこれまでとは全く異なるミトコンドリア機能解析法の可能性を示したものである。また。LONP1の機能低下に伴うミトコンドリアマトリクスでのタンパク質凝集体の蓄積はLONP1の変異が引き起こす「脳・眼・歯・耳介・骨格症候群」でも観察され現象であり、本研究成果は「脳・眼・歯・耳介・骨格症候群」の病態解明にも寄与する。

Report

(5 results)
  • 2021 Annual Research Report   Final Research Report ( PDF )
  • 2020 Annual Research Report
  • 2019 Annual Research Report
  • 2018 Annual Research Report
  • Research Products

    (7 results)

All 2022 2021 2020 2019

All Journal Article (3 results) (of which Peer Reviewed: 1 results,  Open Access: 1 results) Presentation (4 results) (of which Int'l Joint Research: 1 results)

  • [Journal Article] ミトコンドリアプロテアーゼLONP1の機能低下に伴うタンパク質凝集体の生成2022

    • Author(s)
      松島雄一
    • Journal Title

      バイオサイエンスとインダストリー

      Volume: 80(2) Pages: 126-127

    • Related Report
      2021 Annual Research Report
  • [Journal Article] Mitochondrial Lon protease is a gatekeeper for proteins newly imported into the matrix2021

    • Author(s)
      Matsushima Yuichi、Takahashi Kazuya、Yue Song、Fujiyoshi Yuki、Yoshioka Hideaki、Aihara Masamune、Setoyama Daiki、Uchiumi Takeshi、Fukuchi Satoshi、Kang Dongchon
    • Journal Title

      Communications Biology

      Volume: 4 Issue: 1 Pages: 974-974

    • DOI

      10.1038/s42003-021-02498-z

    • Related Report
      2021 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Selective Suppression of Endogenous Gene Expression Using RNAi in Schneider S2 Cells2021

    • Author(s)
      Matsushima Yuichi
    • Journal Title

      Methods Mol Biol .

      Volume: 2281 Pages: 303-312

    • DOI

      10.1007/978-1-0716-1290-3_19

    • ISBN
      9781071612897, 9781071612903
    • Related Report
      2021 Annual Research Report
  • [Presentation] CODAS症候群原因遺伝子LONP1の機能低下により生じるタンパク質凝集体形成機構の解析2021

    • Author(s)
      松島雄一、吉岡秀明、瀬戸山大樹、康東天
    • Organizer
      日本臨床化学会 第61回年次学術集会
    • Related Report
      2021 Annual Research Report
  • [Presentation] ミトコンドリアマトリクスに局在するプロテアーゼLonによるタンパク質の可溶性制御2020

    • Author(s)
      松島雄一
    • Organizer
      日本農芸化学会
    • Related Report
      2019 Annual Research Report
  • [Presentation] ミトコンドリアマトリクスに局在するプロテアーゼLonによるタンパク質の可溶性制御2019

    • Author(s)
      松島雄一
    • Organizer
      日本分子生物学会年会
    • Related Report
      2019 Annual Research Report
  • [Presentation] Mitochondrial Lon protease is a gatekeeper for proteins newly imported into the matrix.2019

    • Author(s)
      Yuichi Matsushima
    • Organizer
      Conference of Asian Society for Mitochondrial Research and Medicine and The 19th Conference of Japanese Society of Mitochondrial Research and Medicine
    • Related Report
      2019 Annual Research Report
    • Int'l Joint Research

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Published: 2018-04-23   Modified: 2023-12-25  

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