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Evaluation and application of the diversity of activity patterns in the population of fragile X syndrome neurons

Research Project

Project/Area Number 19K20683
Research Category

Grant-in-Aid for Early-Career Scientists

Allocation TypeMulti-year Fund
Review Section Basic Section 90110:Biomedical engineering-related
Research InstitutionHiroshima University (2021)
Institute of Physical and Chemical Research (2019-2020)

Principal Investigator

Yuichiro Yada  広島大学, 統合生命科学研究科(理), 研究員 (80805797)

Project Period (FY) 2019-04-01 – 2022-03-31
Project Status Completed (Fiscal Year 2021)
Budget Amount *help
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2021: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2020: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2019: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Keywords脆弱X症候群 / 疾患特異的iPS細胞 / 多点電極アレイ / 活動多様性 / 神経細胞 / パターン解析 / 神経同期活動
Outline of Research at the Start

脆弱X症候群(FXS)は遺伝性疾患で、学習障害やワーキングメモリ機能の低下などの認知障害を生じる。しかし、そのメカニズムは未だ詳しくは分かっていない。本研究は、FXSの神経細胞集団では活動パターンに異常が生じているという仮説を立て、FXS患者iPS細胞由来の神経細胞の活動を計測し、検証する。また、活動パターンを指標として病態改善に有効な薬剤を探索できる可能性を検討する。

Outline of Final Research Achievements

Fragile X syndrome (FXS) is an inherited disorder that causes learning disabilities and cognitive impairment. The electrophysiological basis that causes the disorder is not fully understood. In this study, we aimed to clarify the electrophysiological abnormalities of FXS using neurons derived from disease-specific iPS cells, multi electrode arrays, and pattern analysis techniques. First, we confirmed that the prepared cell lines has FXS-specific genotypes and phenotypes. Then, neurons derived from FXS patients showed higher firing rates than those from healthy controls. The increased firing rates reported in previous studies were also observed in the cell lines used in this study. In addition, there was variation in the initiation timing of neuronal synchronized bursts between FXS patient-derived and healthy control-derived neurons.

Academic Significance and Societal Importance of the Research Achievements

脆弱X症候群患者ではFMRPタンパクの発現が消失し、シナプス・イオンチャネルで様々なタンパク質が過剰発現する。FMRP消失が認知障害を引き起こす生理的なメカニズムは複雑であり、詳細は明らかでない。本研究においてFXS患者由来iPS細胞およびiPS細胞から分化誘導した神経細胞に対する電気生理計測系・取得した計測データ解析系の構築・基礎的な評価を実施し、使用した細胞でFXSでの電気生理的な異常を同定する手がかりとなるような示唆を得た。

Report

(4 results)
  • 2021 Annual Research Report   Final Research Report ( PDF )
  • 2020 Research-status Report
  • 2019 Research-status Report
  • Research Products

    (3 results)

All 2022 2021

All Journal Article (2 results) (of which Peer Reviewed: 2 results,  Open Access: 2 results) Presentation (1 results)

  • [Journal Article] Prediction Model of Amyotrophic Lateral Sclerosis by Deep Learning with Patient Induced Pluripotent Stem Cells2021

    • Author(s)
      Imamura Keiko、Yada Yuichiro、Izumi Yuishin、Morita Mitsuya、Kawata Akihiro、Arisato Takayo、Nagahashi Ayako、Enami Takako、Tsukita Kayoko、Kawakami Hideshi、Nakagawa Masanori、Takahashi Ryosuke、Inoue Haruhisa
    • Journal Title

      Annals of Neurology

      Volume: in press Issue: 6 Pages: 26047-26047

    • DOI

      10.1002/ana.26047

    • NAID

      120006979780

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Human induced pluripotent stem cells generated from a patient with idiopathic basal ganglia calcification2021

    • Author(s)
      Yada Yuichiro、Kondo Takayuki、Suga Mika、Tsukita Kayoko、Enami Takako、Shibukawa Ran、Sagara Yukako、Okanishi Yasue、Imamura Keiko、Kihara Takeshi、Inoue Haruhisa
    • Journal Title

      Stem Cell Research

      Volume: 53 Pages: 102274-102274

    • DOI

      10.1016/j.scr.2021.102274

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] 2次元神経細胞集団の活動多様性と応用2022

    • Author(s)
      矢田祐一郎
    • Organizer
      第6回理論免疫学ワークショップ
    • Related Report
      2021 Annual Research Report

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Published: 2019-04-18   Modified: 2023-01-30  

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