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Analysis of fibrodysplasia ossificans progressive by using iPSCs

Research Project

Project/Area Number 25293320
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypePartial Multi-year Fund
Section一般
Research Field Orthopaedic surgery
Research InstitutionKyoto University

Principal Investigator

Ikeya Makoto  京都大学, iPS細胞研究所, 准教授 (20442923)

Co-Investigator(Kenkyū-buntansha) TOGUCHIDA Junya  京都大学, 再生医科学研究所, 教授 (40273502)
Co-Investigator(Renkei-kenkyūsha) SAKURAI Hidetoshi  京都大学, ips細胞研究所, 准教授 (80528745)
Project Period (FY) 2013-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥16,120,000 (Direct Cost: ¥12,400,000、Indirect Cost: ¥3,720,000)
Fiscal Year 2015: ¥5,590,000 (Direct Cost: ¥4,300,000、Indirect Cost: ¥1,290,000)
Fiscal Year 2014: ¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2013: ¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Keywords異所性骨化 / 進行性骨化性線維異形成症
Outline of Final Research Achievements

Heterotypic ossification (HO) is an event that forms bone in mesenchymal tissues such as skeletal muscle, tendon, and ligament. Previously, it has been shown that growth factors such as bone morphogenetic protein (BMP), and artificial bone scaffold can induce HO, However, the molecular mechanisms and cell-of-origin of the HO is not fully elucidated. In this grant, we used patient-specific iPSCs generated from fibrodysplasia ossificans progressive (FOP-iPSCs) and mutation-corrected FOP-iPSCs (rescued FOP-iPSCs) as genetically-matched control cells, and analyze the molecular mechanisms underlying HO.

Report

(4 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Annual Research Report
  • 2013 Annual Research Report
  • Research Products

    (30 results)

All 2016 2015 2014 2013 Other

All Journal Article (12 results) (of which Peer Reviewed: 4 results,  Open Access: 3 results,  Acknowledgement Compliant: 2 results) Presentation (14 results) (of which Int'l Joint Research: 2 results,  Invited: 2 results) Remarks (3 results) Patent(Industrial Property Rights) (1 results) (of which Overseas: 1 results)

  • [Journal Article] Neofunction of ACVR1 in Fibrodysplasia Ossificance Progressiva2016

    • Author(s)
      Hino K, Ikeya M, Horigome K, Matsumoto Y, Ebise H, Nishio M, Sekiguchi K, Shibata M, Nagata S, Matsuda S, Toguchida J
    • Journal Title

      Proc Natl Acad Sci USA

      Volume: 112 Issue: 50 Pages: 15438-15443

    • DOI

      10.1073/pnas.1510540112

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] 慢性自己炎症性疾患における成長軟骨板の病態2015

    • Author(s)
      戸口田淳也、横山宏司、池谷真、平家俊男、西小森隆太
    • Journal Title

      臨床免疫・アレルギー科

      Volume: 64 Pages: 205-211

    • NAID

      40020563486

    • Related Report
      2015 Annual Research Report
  • [Journal Article] 疾患特異的iPS細胞を活用した筋骨格系難病研究2015

    • Author(s)
      戸口田淳也、横山宏司、池谷 真、平家俊男、西小森隆太
    • Journal Title

      日本臨牀

      Volume: 73 Pages: 416-422

    • Related Report
      2015 Annual Research Report
  • [Journal Article] 疾患特異的iPS細胞を活用した骨軟骨疾患研究2015

    • Author(s)
      戸口田淳也、池谷 真、西小森隆太
    • Journal Title

      病理と臨床

      Volume: 33 Pages: 599-606

    • Related Report
      2015 Annual Research Report
  • [Journal Article] New Protocol to Optimize iPS Cells for Genome Analysis of Fibrodysplasia Ossificans Progressiva.2015

    • Author(s)
      Matsumoto Y, Ikeya M, Hino K, Horigome K, Fukuta M, Watanabe M, Nagata S, Yamamoto T, Otsuka T, Toguchida J.
    • Journal Title

      Stem Cells.

      Volume: 印刷中 Issue: 6 Pages: 1730-1742

    • DOI

      10.1002/stem.1981

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] 患者由来iPS細胞を用いた疾患モデル作製研究:骨軟骨疾患2015

    • Author(s)
      戸口田淳也、池谷 真
    • Journal Title

      医学の歩み

      Volume: 252 Pages: 1245-1250

    • Related Report
      2014 Annual Research Report
  • [Journal Article] 進行性骨化性線維異形成症2015

    • Author(s)
      池谷 真、松本佳久、戸口田淳也
    • Journal Title

      遺伝子医学MOOK

      Volume: 27 Pages: 203-207

    • Related Report
      2014 Annual Research Report
  • [Journal Article] 疾患特異的iPS細胞を用いた難治性骨軟骨疾患の病態解析・創薬2015

    • Author(s)
      戸口田淳也、池谷 真、西小森隆太、松本佳久、横山宏司
    • Journal Title

      実験医学増刊 再生医療2015 幹細胞と疾患iPS細胞の研究最前線

      Volume: 33 Pages: 147-151

    • Related Report
      2014 Annual Research Report
  • [Journal Article] Derivation of mesenchymal stromal cells from pluripotent stem cells through a neural crest lineage using small molecule compounds with defined media.2014

    • Author(s)
      Fukuta M, Nakai Y, Kirino K, Nakagawa M, Sekiguchi K, Nagata S, Matsumoto Y, Yamamoto T, Umeda K, Heike T, Okumura N, Koizumi N, Sato T, Nakahata T, Saito M, Otsuka T, Kinoshita S, Ueno M, Ikeya M, Toguchida J.
    • Journal Title

      PLOS ONE

      Volume: 9 Issue: 12 Pages: e112291-e112291

    • DOI

      10.1371/journal.pone.0112291

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Special Review 間葉系幹細胞を巡る最近の進歩について2014

    • Author(s)
      戸口田淳也、福田誠、池谷真
    • Journal Title

      細胞工学

      Volume: 33 Pages: 1282-1287

    • Related Report
      2014 Annual Research Report
  • [Journal Article] 骨への分化誘導2014

    • Author(s)
      松本佳久、池谷真、戸口田淳也
    • Journal Title

      実験医学別冊 ES・iPS細胞実験スタンダード

      Volume: 39 Pages: 246-257

    • Related Report
      2013 Annual Research Report
  • [Journal Article] Induced pluripotent stem cells from patients with human fibrodysplasia ossificans progressiva show increased mineralization and cartilage formation.2013

    • Author(s)
      Matsumoto Y, Hayashi Y, Schlieve CR, Ikeya M, Kim H, Nguyen TD, Sami S, Baba S, Barruet E, Nasu A, Asaka I, Otsuka T, Yamanaka S, Conklin BR, Toguchida J, Hsiao EC.
    • Journal Title

      Orphanet J Rare Dis.

      Volume: 8 Issue: 1 Pages: 190-190

    • DOI

      10.1186/1750-1172-8-190

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Presentation] 患者由来iPS細胞を用いた進行性骨化性線維異形成症 (FOP)の病態解明2016

    • Author(s)
      日野 恭介,松本 佳久,池谷 真,堀込 一彦,永田 早苗,戸口田 淳也
    • Organizer
      第15回日本再生医療学会総会
    • Place of Presentation
      大阪国際会議場(大阪府大阪市)
    • Year and Date
      2016-03-17
    • Related Report
      2015 Annual Research Report
  • [Presentation] 患者由来iPS細胞を用いた進行性骨化性線維異形成症(FOP)の病態解明2016

    • Author(s)
      日野 恭介,松本 佳久,池谷 真,堀込 一彦,永田 早苗,戸口田 淳也
    • Organizer
      第29回日本軟骨代謝学会
    • Place of Presentation
      広仁会館(広島県広島市)
    • Year and Date
      2016-02-20
    • Related Report
      2015 Annual Research Report
  • [Presentation] New protocol to optimize iPS cells for genome analysis of fibrodysplasia ossificans progressive2015

    • Author(s)
      Yoshihisa Matsumoto, Makoto Ikeya, Makoto Fukuta, Takanobu Otsuka, Junya Toguchida
    • Organizer
      ASBMR2015 米国骨代謝学会
    • Place of Presentation
      シアトル(米国)
    • Year and Date
      2015-10-11
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 患者由来iPS細胞を用いた進行性骨化性線維異形成症 (FOP)への取り組み2015

    • Author(s)
      日野 恭介,池谷 真,堀込 一彦,松本 佳久,西尾 恵,松田 秀一,戸口田 淳也
    • Organizer
      第33回日本骨代謝学会学術集会
    • Place of Presentation
      京王プラザホテル(東京都新宿区)
    • Year and Date
      2015-07-23
    • Related Report
      2015 Annual Research Report
  • [Presentation] ESTABLISHMENT OF NEW DRUG SCREENING SYSTEM USING FIBRODYSPLASIA OSSIFICANS PROGRESSIVA IPS CELLS2015

    • Author(s)
      Yoshihisa Matsumoto, Makoto Ikeya, Makoto Fukuta, Takanobu Otsuka, Junya Toguchida.
    • Organizer
      第13回国際幹細胞生物学会
    • Place of Presentation
      ストックホルム(スウェーデン)
    • Year and Date
      2015-06-26
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 疾患特異的iPS細胞を用いた進行性骨化性線維異形成症の創薬に向けた薬剤 スクリーニング系の構築2015

    • Author(s)
      松本佳久、 池谷真、 福田誠、永田早苗、 浅香勲、 大塚隆信、 戸口田淳也
    • Organizer
      第14回日本再生医療学会総会
    • Place of Presentation
      パシフィコ横浜(神奈川県横浜市)
    • Year and Date
      2015-03-20
    • Related Report
      2014 Annual Research Report
  • [Presentation] Derivation of mesenchymal stromal cells from pluripotent stem cells through a neural crest lineage using small molecule compounds with defined media2015

    • Author(s)
      Makoto Fukuta, Yoshinori Nakai, Kosuke Kirino, Morio Ueno, Makoto Ikeya, Takanobu Otsuka, Junya Toguchida
    • Organizer
      第18回武田科学振興財団生命科学シンポジウム
    • Place of Presentation
      武田薬品研修所(大阪府吹田市)
    • Year and Date
      2015-01-15
    • Related Report
      2014 Annual Research Report
  • [Presentation] 疾患特異的iPS細胞を用いた進行性骨化性線維異形成症の病態解析2014

    • Author(s)
      松本佳久、池谷真、福田誠、永田早苗、浅香勲、大塚隆信、戸口田淳也
    • Organizer
      第29回日本整形外科学会基礎学術集会
    • Place of Presentation
      城山観光ホテル(鹿児島県鹿児島市)
    • Year and Date
      2014-10-09
    • Related Report
      2014 Annual Research Report
  • [Presentation] Application of iPS cells for rare and intractable bone and cartilage diseases2014

    • Author(s)
      池谷真
    • Organizer
      第29回日本整形外科学会基礎学術集会
    • Place of Presentation
      城山観光ホテル(鹿児島県鹿児島市)
    • Year and Date
      2014-10-09
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Identification of gene sets dysregulated by mutant ACVR1 gene causing a rare intractable disease, fibrodysplasia ossificance progressive2014

    • Author(s)
      Yoshihisa Matsumoto, Makoto Ikeya, Makoto Fukuta, Edward Hsiao, Yohei Hayashi, Isao Asaka, Takanobu Otsuka, Bruce R. Conklin, Junya Toguchida
    • Organizer
      米国骨代謝学会
    • Place of Presentation
      Houston(USA)
    • Year and Date
      2014-09-14
    • Related Report
      2014 Annual Research Report
  • [Presentation] Identification of gene sets dysregulated by mutant ACVR1 gene causing a rare intractable disease, fibrodysplasia ossificance progressive2014

    • Author(s)
      Yoshihisa Matsumoto, Makoto Ikeya, Makoto Fukuta, Edward Hsiao, Yohei Hayashi, Isao Asaka, Takanobu Otsuka, Bruce R. Conklin, Junya Toguchida
    • Organizer
      第12回国際幹細胞生物学会
    • Place of Presentation
      Vancouver(Canada)
    • Year and Date
      2014-06-18
    • Related Report
      2014 Annual Research Report
  • [Presentation] Application of iPS cells to the research of Fibrodysplasia Ossificans Progressiva

    • Author(s)
      Yoshihisa Matsumoto, Makoto Ikeya, Edward Hsiao, Christopher R. Schlieve, Isao Asaka, Takanobu Otsuka, Bruce R. Conklin, Junya Toguchida
    • Organizer
      ISSCR 11th annual meeting
    • Place of Presentation
      Boston Convention and Exhibition Center(Boston, MA, USA)
    • Related Report
      2013 Annual Research Report
  • [Presentation] Application of iPS cells for a rare and intractable disease involving mesenchymal tissues

    • Author(s)
      Yoshihisa Matsumoto, Makoto Ikeya, Edward Hsiao, Christopher R. Schlieve, Akira Nasu, Isao Asaka, Takanobu Otsuka, Bruce R. Conklin, Junya Toguchida
    • Organizer
      The 8th Combined Meeting of Orthopaedic Research Societies (CORS)
    • Place of Presentation
      Congress Center of San Servolo(San Servolo, Venice, Italy)
    • Related Report
      2013 Annual Research Report
  • [Presentation] Application of iPS cells for rare and intractable bone and cartilage diseases

    • Author(s)
      Makoto Ikeya
    • Organizer
      The 7th Takeda Science Foundation Symposium on PharmaScience
    • Place of Presentation
      Center for Learning and Innovation Takeda Pharmaceutical Campany Ltd(Osaka, Japan)
    • Related Report
      2013 Annual Research Report
    • Invited
  • [Remarks] FOP患者さん由来iPS細胞を用いて、 病態再現と創薬に向けた評価系の構築に成功

    • URL

      http://www.cira.kyoto-u.ac.jp/j/pressrelease/news/150313-163653.html

    • Related Report
      2014 Annual Research Report
  • [Remarks] ヒトiPS/ES細胞より高効率に神経堤細胞および間葉系間質細胞の作製に成功 PLOS ONEに掲載

    • URL

      http://www.cira.kyoto-u.ac.jp/j/pressrelease/news/141203-075150.html

    • Related Report
      2014 Annual Research Report
  • [Remarks] FOP患者さん由来のiPS細胞で病態を再現することに成功

    • URL

      https://www.cira.kyoto-u.ac.jp/j/pressrelease/news/131225-105830.html

    • Related Report
      2013 Annual Research Report
  • [Patent(Industrial Property Rights)] 異所性骨化の予防・治療剤及びそのスクリーニング方法2014

    • Inventor(s)
      戸口田淳也/池谷真/松本佳久
    • Industrial Property Rights Holder
      戸口田淳也/池谷真/松本佳久
    • Industrial Property Rights Type
      特許
    • Filing Date
      2014-12-24
    • Related Report
      2014 Annual Research Report
    • Overseas

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Published: 2013-05-21   Modified: 2019-07-29  

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