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Investigation of pathogenesis of leukemia in congenital bone marrow failure syndrome using human iPS cells

Research Project

Project/Area Number 17K19600
Research Category

Grant-in-Aid for Challenging Research (Exploratory)

Allocation TypeMulti-year Fund
Research Field Tumor biology and related fields
Research InstitutionHiroshima University

Principal Investigator

Mochizuki Shinji  広島大学, 医系科学研究科(医), 助教 (90349473)

Co-Investigator(Kenkyū-buntansha) 小林 正夫  広島大学, 医系科学研究科(医), 名誉教授 (00162016)
Project Period (FY) 2017-06-30 – 2020-03-31
Project Status Completed (Fiscal Year 2019)
Budget Amount *help
¥6,500,000 (Direct Cost: ¥5,000,000、Indirect Cost: ¥1,500,000)
Fiscal Year 2018: ¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
Fiscal Year 2017: ¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Keywords小児血液学 / iPS細胞 / 白血病発症機序 / 先天性骨髄不全症候群 / 小児がん / 重症先天性好中球減少症 / 白血病 / 遺伝性腫瘍症候群 / cancer predisposition
Outline of Final Research Achievements

We established iPS cells from congenital bone marrow failure syndrome. Myeloid cells derived from severe congenital neutropenia (SCN)-iPS cells revealed the maturation arrest at a promyelocyte stage, reflecting the symptom of SCN patients. One from cyclic neutropenia (CyN) didn’t show that. We also knocked-in and -out some genes which are associated with leukemogenesis in SCN by genome-editing technologies and compared the process of the differentiation into blood cells. In addition, SCN-iPS cells were expected to be useful for the investigation into the mechanisms causing the occurrence of leukemia and the establishment of the methods to prevent it in SCN.

Academic Significance and Societal Importance of the Research Achievements

小児にみられる先天性骨髄不全症候群には重症先天性好中球減少症など高率に白血病化することが知られているものが多い。重症先天性好中球減少症と近接した遺伝子変異によるとされる周期性好中球減少症では白血病化は起こらず、また臨床経過も異なる。これら疾患の患者由来iPS細胞の比較・解析は白血病化のメカニズムの解明に有効であると考えられる。特に血液に分化していく過程で白血病化を時間経過ごとに追跡することが可能であるiPS細胞を用いることの意義は大きい。今回これらの解析系の確立、安定化により様々な疾患の解析が可能となった。

Report

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

    (20 results)

All 2020 2019 2018 2017 1994

All Journal Article (8 results) (of which Int'l Joint Research: 2 results,  Peer Reviewed: 6 results,  Open Access: 5 results) Presentation (12 results)

  • [Journal Article] Impact of graft-versus-host disease on the clinical outcome of allogeneic hematopoietic stem cell transplantation for non-malignant diseases2020

    • Author(s)
      Umeda K, Imai K, Yanagimachi M, Yabe H, Kobayashi M, Takahashi Y, Kajiwara M, Yoshida N, Cho Y, Inoue M, Hashii Y, Atsuta Y, Morio T
    • Journal Title

      Int J Hematol

      Volume: - Issue: 6 Pages: 869-876

    • DOI

      10.1007/s12185-020-02839-4

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Downregulation of endothelial nitric oxide synthase (eNOS) and endothelin-1 (ET-1) in a co-culture system with human stimulated X-linked CGD neutrophils2020

    • Author(s)
      Nakamura-Utsunomiya Akari、Tsumura Miyuki、Okada Satoshi、Kawaguchi Hiroshi、Kobayashi Masao
    • Journal Title

      PLOS ONE

      Volume: 15 Issue: 4 Pages: e0230665-e0230665

    • DOI

      10.1371/journal.pone.0230665

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Simple and Robust Differentiation of Human Pluripotent Stem Cells toward Chondrocytes by Two Small-Molecule Compounds2019

    • Author(s)
      Kawata Manabu、Mori Daisuke、Kanke Kosuke、Hojo Hironori、Ohba Shinsuke、Chung Ung-il、Yano Fumiko、Masaki Hideki、Otsu Makoto、Nakauchi Hiromitsu、Tanaka Sakae、Saito Taku
    • Journal Title

      Stem Cell Reports

      Volume: 13 Issue: 3 Pages: 530-544

    • DOI

      10.1016/j.stemcr.2019.07.012

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Generation of three induced pluripotent stem cell lines from postmortem tissue derived following sudden death of a young patient with STXBP1 mutation2019

    • Author(s)
      Yamamoto T, Otsu M, Okumura T, Horie Y, Ueno Y, Taniguchi H, Ohtaka M, Nakanishi M, Abe Y, Murase T, Umehara T, Ikematsu K
    • Journal Title

      Stem Cell Res

      Volume: 39 Pages: 101485-101485

    • DOI

      10.1016/j.scr.2019.101485

    • NAID

      120006987680

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Robust and highly efficient hiPSC generation from patient non-mobilized peripheral blood-derived CD34+ cells using the auto-erasable Sendai virus vector.2019

    • Author(s)
      Okumura T, Horie Y, et al.
    • Journal Title

      Stem Cell Res Ther.

      Volume: 10 Issue: 1 Pages: 185-185

    • DOI

      10.1186/s13287-019-1273-2

    • Related Report
      2019 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] 好中球減少症2017

    • Author(s)
      望月慎史
    • Journal Title

      小児科診療

      Volume: 80 Pages: 1203-1209

    • Related Report
      2017 Research-status Report
  • [Journal Article] オンコロジック・エマージェンシー 白血球増多症2017

    • Author(s)
      望月慎史
    • Journal Title

      小児内科

      Volume: 49 Pages: 1757-1760

    • Related Report
      2017 Research-status Report
  • [Journal Article] 日本小児科学会雑誌 = The journal of the Japan Pediatric Society 98(9)1994

    • Author(s)
      野間康輔、望月慎史ら
    • Volume
      122
    • Pages
      -
    • DOI

      10.11501/3401280

    • ISSN
      0001-6543
    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Presentation] 補体C1q欠損症に対する同種造血幹細胞移植2019

    • Author(s)
      松村梨紗、望月慎史ほか
    • Organizer
      日本造血細胞移植学会
    • Related Report
      2019 Annual Research Report
  • [Presentation] 白血病転化から特異な経過を呈した重症先天性好中球減少症(SCN)の1例2019

    • Author(s)
      早川博子、望月慎史ほか
    • Organizer
      日本免疫不全・自己炎症学会
    • Related Report
      2019 Annual Research Report
  • [Presentation] 白血病転化から特異な経過を呈した重症先天性好中球減少症の1例2019

    • Author(s)
      森下祐介、望月慎史ほか
    • Organizer
      日本小児血液・がん学会
    • Related Report
      2019 Annual Research Report
  • [Presentation] 維持療法中にヒトパルボウイルスB19感染による汎血球減少を呈した混合形質性急性白血病(MPAL)の一例2019

    • Author(s)
      芦原康介、望月慎史ほか
    • Organizer
      日本小児血液・がん学会
    • Related Report
      2019 Annual Research Report
  • [Presentation] 補体C1q欠損症に対する同種造血幹細胞移植2019

    • Author(s)
      松村梨紗、望月慎史ら
    • Organizer
      第40回日本造血細胞移植学会
    • Related Report
      2018 Research-status Report
  • [Presentation] 同種造血幹細胞移植後、骨髄再発に先行して骨単独再発を認めた治療抵抗性BCP-ALLの1例2018

    • Author(s)
      松村梨紗、望月慎史ら
    • Organizer
      第60回日本小児血液・がん学会学術集会
    • Related Report
      2018 Research-status Report
  • [Presentation] 両側多発性腎腫瘍の一例2018

    • Author(s)
      森下祐介、望月慎史ら
    • Organizer
      第60回日本小児血液・がん学会学術集会
    • Related Report
      2018 Research-status Report
  • [Presentation] 初診時に意識障害、呼吸障害、腎障害など多彩な全身症状を呈したBCP-ALLの一例2018

    • Author(s)
      江口勇太、望月慎史ら
    • Organizer
      第121回日本小児科学会学術集会
    • Related Report
      2018 Research-status Report
  • [Presentation] Efficacy of clofarabine based chemotherapy followed by haplo-HSCT for refractory childhood ALL2018

    • Author(s)
      望月慎史ら
    • Organizer
      第80回日本血液学会学術集会
    • Related Report
      2018 Research-status Report
  • [Presentation] 初診時に意識障害、呼吸障害、腎障害など多彩な全身症状を呈したBCP-ALLの一例2018

    • Author(s)
      江口勇太、望月慎史、小林正夫、他
    • Organizer
      第121回日本小児科学会学術集会
    • Related Report
      2017 Research-status Report
  • [Presentation] 両側多発性腎腫瘍の一例 A case of bilateral multifocal renal tumors.2017

    • Author(s)
      野間康輔、望月慎史、小林正夫、他
    • Organizer
      第59回日本小児血液がん学会学術集会
    • Related Report
      2017 Research-status Report
  • [Presentation] Mixed-phenotype acute leukemia with B, T, and myeloid lineages - a pediatric case -2017

    • Author(s)
      野間康輔、望月慎史、小林正夫、他
    • Organizer
      第79回日本血液学会学術集会
    • Related Report
      2017 Research-status Report

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Published: 2017-07-21   Modified: 2021-02-19  

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