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Molecular mechanism of novel phospholipid transport proteins and its relationship with mitochondrial diseases

Research Project

Project/Area Number 17K08642
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field General medical chemistry
Research InstitutionDokkyo Medical University

Principal Investigator

HORIBATA YASUHIRO  獨協医科大学, 医学部, 准教授 (80392116)

Project Period (FY) 2017-04-01 – 2021-03-31
Project Status Completed (Fiscal Year 2020)
Budget Amount *help
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2019: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2018: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2017: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Keywordsリン脂質 / ミトコンドリア / 筋分化 / 骨格筋 / ホスファチジルコリン / 細胞内輸送 / 脂質
Outline of Final Research Achievements

Mitochondrial membranes are composed of two phospholipid bilayers. More than half of the phospholipids in mitochondrial membranes are phosphatidylcholine (PC). Mitochondria lack the enzymes required for PC synthesis. The lipid is therefore likely supplied from organelles such as the endoplasmic reticulum which contains the biosynthesis systems for PC. This study clarified the molecular basis for transporting PC to mitochondria by StarD7. This study also found that deficiency of StarD7 in myoblast caused not only impairement of mitochondrial functions but also inhibition of myogenic differentiation.

Academic Significance and Societal Importance of the Research Achievements

ERなどのオルガネラからミトコンドリアへのPC輸送機構は細胞生物学や脂質生化学において重要な研究課題の一つであるが、長い間解明されていなかった。今回、研究代表者が先行研究で見出したタンパク質StarD7が上記輸送機構の一端にかかわることを分子のレベルで明らかにした。ところで、ミトコンドリア病を引き起こす原因遺伝子の変異は未だ多くが不明である。本研究ではStarD7によるPC輸送機構が破綻すると、ミトコンドリアの機能低下が生じ、筋細胞の分化能が著しく阻害されミオパチーの原因になり得ることを示唆した。

Report

(5 results)
  • 2020 Annual Research Report   Final Research Report ( PDF )
  • 2019 Research-status Report
  • 2018 Research-status Report
  • 2017 Research-status Report
  • Research Products

    (15 results)

All 2020 2019 2018 2017 Other

All Int'l Joint Research (1 results) Journal Article (5 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 5 results,  Open Access: 4 results) Presentation (9 results) (of which Int'l Joint Research: 1 results)

  • [Int'l Joint Research] Rappaport School of Medicine/Hadassah-Hebrew University/Galilee Medical Center(イスラエル)

    • Related Report
      2018 Research-status Report
  • [Journal Article] Locations and contributions of the phosphotransferases EPT1 and CEPT1 to the biosynthesis of ethanolamine phospholipids.2020

    • Author(s)
      Horibata Y, Ando H, Sugimoto H.
    • Journal Title

      Journal of Lipid Research

      Volume: 61 Issue: 8 Pages: 1221-1231

    • DOI

      10.1194/jlr.ra120000898

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] The phosphatidylcholine transfer protein StarD7 is important for myogenic differentiation in mouse myoblast C2C12 cells and human primary skeletal myoblasts.2020

    • Author(s)
      Horibata Y, Mitsuhashi S, Shimizu H, Maejima S, Sakamoto H, Aoyama C, Ando H, Sugimoto H
    • Journal Title

      Scientific Reports

      Volume: 10 Issue: 1 Pages: 2845-2845

    • DOI

      10.1038/s41598-020-59444-y

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Ethanolamine phosphotransferase 1 (selenoprotein I) is critical for the neural development and maintenance of plasmalogen in human.2018

    • Author(s)
      Horibata Y, Elpeleg O, Eran A, Hirabayashi Y, Savitzki D, Tal G, Mandel H, Sugimoto H
    • Journal Title

      Journal of Lipid Research

      Volume: 59 Issue: 6 Pages: 1-12

    • DOI

      10.1194/jlr.p081620

    • Related Report
      2018 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Identification of the N-terminal transmembrane domain of StarD7 and its importance for mitochondrial outer membrane localization and phosphatidylcholine transfer.2017

    • Author(s)
      Horibata Y, Ando H, Satou M, Shimizu H, Mitsuhashi S, Shimizu Y, Itoh M, Sugimoto H
    • Journal Title

      Scientific Reports

      Volume: 7(1) Issue: 1 Pages: 8793-8793

    • DOI

      10.1038/s41598-017-09205-1

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Changes of plasmalogen phospholipid levels during differentiation of induced pluripotent stem cells 409B2 to endothelial phenotype cells2017

    • Author(s)
      Yusuke Nakamura, Yasuo Shimizu, Yasuhiro Horibata, Rinna Tei, Ryosuke Koike1, Meitetsu Masawa, Taiji Watanabe, Taichi Shiobara, Ryo Arai, Kazuyuki Chibana, Akihiro Takemasa, Hiroyuki Sugimoto, Yoshiki Ishii
    • Journal Title

      Scientific Reports

      Volume: 7 Issue: 1 Pages: 1-9

    • DOI

      10.1038/s41598-017-09980-x

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] リン脂質輸送タンパク質StarD7がミトコンドリアにおいて果たす役割2020

    • Author(s)
      堀端康博, 杉本博之
    • Organizer
      第62回日本脂質生化学会プレシンポジウム
    • Related Report
      2020 Annual Research Report
  • [Presentation] Kennedy経路における最終酵素EPT1とCEPT1の細胞内局在およびこれらの酵素が合成するエタノールアミンリン脂質分子種の相違2020

    • Author(s)
      堀端康博, 杉本博之
    • Organizer
      第62回日本脂質生化学会
    • Related Report
      2020 Annual Research Report
  • [Presentation] ミトコンドリアのリン脂質恒常性に関わる輸送タンパク質STARD7は骨格筋分化に必須である2019

    • Author(s)
      堀端 康博、三橋 里美、清水 裕晶、青山 智英子、杉本 博之
    • Organizer
      第92回日本生化学会大会
    • Related Report
      2019 Research-status Report
  • [Presentation] 筋分化におけるホスファチジルコリン輸送タンパク質STARD7の役割の解析2019

    • Author(s)
      堀端 康博、三橋 里美、青山 智英子、清水 裕晶、杉本 博之
    • Organizer
      第61回日本脂質生化学会
    • Related Report
      2019 Research-status Report
  • [Presentation] Identification, characterization, and functional analysis of a novel phospholipid carrier protein to mitochondria in mammalian cells.2019

    • Author(s)
      Horibata Yasuhiro and Sugimoto Hiroyuki
    • Organizer
      27th FAOBMB & 44th MSBMB Conference IUBMB Special Symposia
    • Related Report
      2019 Research-status Report
    • Int'l Joint Research
  • [Presentation] EPT1はエタノールアミンプラズマローゲンの産生と維持に主要な役割を担い、脳神経系の正常な発達を司る2018

    • Author(s)
      堀端康博, Orly Elpeleg, 平林義雄, Hanna Mandel, 杉本博之
    • Organizer
      第60回日本脂質生化学会
    • Related Report
      2018 Research-status Report
  • [Presentation] プラズマローゲンの保持におけるEPT1の役割とその変異による脳変性疾患2018

    • Author(s)
      堀端康博, Orly Elpeleg, 平林義雄, Hanna Mandel, 杉本博之
    • Organizer
      第91回日本生化学会大会
    • Related Report
      2018 Research-status Report
  • [Presentation] StarD7が有する膜貫通領域の同定とミトコンドリア局在およびPC輸送における役割2017

    • Author(s)
      堀端康博, 安戸博美, 青山智英子, 佐藤元康, 清水泰生, 伊藤雅彦, 杉本博之
    • Organizer
      第59回日本脂質生化学会
    • Related Report
      2017 Research-status Report
  • [Presentation] ホスファチジルコリン輸送タンパク質StarD7は、ミトコンドリア外膜の外葉に局在する2017

    • Author(s)
      堀端康博, 安戸博美, 佐藤元康, 清水裕晶, 三橋里美, 清水泰生, 伊藤雅彦, 杉本博之
    • Organizer
      第90回日本生化学会大会
    • Related Report
      2017 Research-status Report

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Published: 2017-04-28   Modified: 2022-01-27  

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