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The mechanisms of pulmonary hypertension in Down syndrome by iPSC technology

Research Project

Project/Area Number 17K16261
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Pediatrics
Research InstitutionOsaka University

Principal Investigator

Suginobe Hidehiro  大阪大学, 医学部附属病院, 医員 (30791595)

Project Period (FY) 2017-04-01 – 2020-03-31
Project Status Completed (Fiscal Year 2019)
Budget Amount *help
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2019: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2018: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2017: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Keywordsダウン症候群 / 肺高血圧 / iPS細胞
Outline of Final Research Achievements

It is well known that Down syndrome patients with congenital heart defects tend to progress pulmonary hypertension, however the moleccular mechanisms are unclarified until now. In this study, we established iPSCs from Down syndrome patients and differentiated them into vascular endotheial cells and vascular smooth muscle cells. Then we analyzed the cellular physiology and comprehensive gene expression profiling of these cells. We found that vascular smooth muscle cells of Down syndrome showed higher proliferative ability. We also found that endhothelial dysfunction with dysregulation of mitochondrial function, and gene expression patterns were significantly different in Down syndrome endothelial cells as compared to wild type controls.

Academic Significance and Societal Importance of the Research Achievements

本研究によって、ダウン症候群患者において肺高血圧が多くなる要因として、血管細胞機能が変化していることが判明した。特に内皮細胞におけるミトコンドリア機能障害が大きく寄与している可能性がある。さらに、内皮細胞の発現プロファイル解析から、21染色体以外の遺伝子においても特徴的な発現パターンを示している遺伝子が見つかった。これらの遺伝子の機能を解析することにより、新たな治療法の開発につながると考えられる。

Report

(4 results)
  • 2019 Annual Research Report   Final Research Report ( PDF )
  • 2018 Research-status Report
  • 2017 Research-status Report

Research Products

(3 results)

All 2020 2019 2017

All Presentation (3 results)

  • [Presentation] iPS細胞を用いたダウン症患者における肺高血圧症メカニズムの解析2020

    • Author(s)
      杉辺 英世
    • Organizer
      第26回 日本小児肺循環研究会学術集会
    • Related Report
      2019 Annual Research Report
  • [Presentation] iPS細胞から分化させた血管内皮細胞および平滑筋細胞は21トリソミーにおける肺動脈性肺高血圧症の細胞、分子レベルでの病態を明らかにする2019

    • Author(s)
      杉辺 英世
    • Organizer
      第55回 日本小児循環器学会総会
    • Related Report
      2019 Annual Research Report
  • [Presentation] iPS細胞を用いたダウン症候群特異的肺高血圧症発症機序の解明2017

    • Author(s)
      石田秀和,小垣滋豊,那波伸敏,杉辺英世,桂木慎一,馬殿洋樹,石井 良,鳥越史子,成田淳,北畠康司,大薗恵一.
    • Organizer
      第53回日本小児循環器学会総会・学術集会
    • Related Report
      2017 Research-status Report

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Published: 2017-04-28   Modified: 2021-02-19  

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