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Establishment of induced pluripotent stem cells from glycogen storage disease type Ib patient and making disease models

Research Project

Project/Area Number 26670038
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Pharmacology in pharmacy
Research InstitutionNagoya City University

Principal Investigator

Matsunaga Tamihide  名古屋市立大学, 薬学研究科(研究院), 教授 (40209581)

Co-Investigator(Kenkyū-buntansha) ITO Tetsuya  藤田保健衛生大学, 医学部, 教授 (80336677)
前田 徹  金城学院大学, 薬学部, 准教授 (90381855)
Co-Investigator(Renkei-kenkyūsha) MAEDA Tohru  金城学院大学, 薬学部, 准教授 (90381855)
Project Period (FY) 2014-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000)
Fiscal Year 2015: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2014: ¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
Keywordsヒト骨髄性白血病細胞 / 好中球 / 糖原病Ib / 疾患iPS細胞 / 糖原病Ib型 / 肝細胞 / HL-60細胞 / iPS細胞 / 酸化的ストレス / NADPH oxidase
Outline of Final Research Achievements

Glycogen storage disease type Ib (GSD-Ib) is caused by mutations in the glucose-6-phosphate transporter (G6PT) gene, which is involved in glycogen metabolism. Patients with GSD-Ib are known to develop neutropenia as a specific symptom, but the causes remain unclear. We examined the mechanism of reactive oxygen species production after differentiation from HL-60 cells, and the collapse of glycogen metabolism because of G6PT deficiency. A high intracellular glucose level leads to an increase in ROS production by PKC induction.
We established induced pluripotent stem cells (iPS cells) from patients with GSD-Ib. iPS cells-derived hepatocytes generated from patients with GSDIb metabolic abnormalities recapitulated key pathological features of the diseases affecting the patients from whom they were derived, such as glycogen, lactate, pyruvate and lipid accumulation. Cells that were differentiated into neutrophils also showed the GSDIb pathology.

Report

(3 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • Research Products

    (6 results)

All 2016 2015 2014

All Journal Article (3 results) (of which Peer Reviewed: 1 results) Presentation (3 results) (of which Invited: 1 results)

  • [Journal Article] ヒトiPS細胞の分化誘導の現状と創薬研究への応用2016

    • Author(s)
      岩尾岳洋、松永民秀
    • Journal Title

      HAB Newsletter

      Volume: 22 Pages: 6-7

    • Related Report
      2015 Annual Research Report
  • [Journal Article] 個別化(オーダーメイド)医療を志向した薬物動態研究及び毒性試験へのヒトiPS細胞の利用2015

    • Author(s)
      岩尾岳洋、松永民秀
    • Journal Title

      Organ Biology

      Volume: 22 Pages: 39-48

    • Related Report
      2015 Annual Research Report
  • [Journal Article] G6PT Inhibition Model Using HL-60 Cells and Induction of ROS Production through PKC/NOX2 Activation: Clinical Condition for Elucidation of Glycogen Storage Disease Type Ib2014

    • Author(s)
      Satoh D et al.
    • Journal Title

      Biological and Pharmaceutical Bulletin

      Volume: 37 Issue: 4 Pages: 534-540

    • DOI

      10.1248/bpb.b13-00708

    • NAID

      130003382133

    • ISSN
      0918-6158, 1347-5215
    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Presentation] ヒトiPS細胞から肝細胞への低分子化合物を用いた分化誘導2016

    • Author(s)
      松永民秀、栗木駿輔、坂本 栄、岩尾岳洋
    • Organizer
      細胞アッセイ研究会シンポジウム
    • Place of Presentation
      東京
    • Year and Date
      2016-01-19
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] 新規三次元培養培地を用いた浮遊培養法でのヒトiPS細胞から肝細胞への分化誘導2015

    • Author(s)
      阿武志保、岩尾岳洋、金木達朗、松永民秀
    • Organizer
      第38回日本分子生物学会・第88回日本生化学会大会合同大会
    • Place of Presentation
      神戸
    • Year and Date
      2015-12-01
    • Related Report
      2015 Annual Research Report
  • [Presentation] 糖原病Ib型患者iPS細胞を用いた好中球病態モデルの作成2014

    • Author(s)
      宮野百合香、大手万理子、佐藤大介、前田 徹、中村克徳、松永民秀
    • Organizer
      医療薬学フォーラム2014/第22回クリニカルファーマシーシンポジウム
    • Place of Presentation
      東京
    • Year and Date
      2014-06-28 – 2014-06-29
    • Related Report
      2014 Research-status Report

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Published: 2014-04-04   Modified: 2017-05-10  

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