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Establish of gene repair methods and analysis of pathological conditions using iPS cells derived from patients with glycogen storage disease type Ia

Research Project

Project/Area Number 26870544
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Metabolomics
Pediatrics
Research InstitutionKumamoto University

Principal Investigator

Kido Jun  熊本大学, 医学部附属病院, 診療助手 (70721215)

Project Period (FY) 2014-04-01 – 2017-03-31
Project Status Completed (Fiscal Year 2016)
Budget Amount *help
¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000)
Fiscal Year 2016: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2015: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Keywords糖原病Ia型 / iPS細胞 / 肝臓 / 肝臓分化 / グリコーゲン / 糖原病 / 疾患iPS細胞 / 遺伝子修復
Outline of Final Research Achievements

Glycogen storege disease type Ia (GSD Ia) develop accumulation of glycogen in liver and hypoglycemia by impaired glyconeogenesis because of defect of Glucose-6-Phosphatase (G6Pase) in endoplasmic reticulum. In this study, we developed each iPS cell line with mutation of G6Pase gene from two patients with GSD Ia. Then, we induced iPS cells derived form normal subject and patients with GSD Ia into differentiated hepatocyte. The autophagy in hepatocyte from patients with GSD Ia more enhanced compared to that of hepatocyte from normal subject. Moreover, the autophagy in hepatocyte from patients with GSD Ia increasingly upregulated under low-glucose culture.

Report

(4 results)
  • 2016 Annual Research Report   Final Research Report ( PDF )
  • 2015 Research-status Report
  • 2014 Research-status Report
  • Research Products

    (7 results)

All 2017 2016

All Journal Article (5 results) (of which Int'l Joint Research: 5 results,  Peer Reviewed: 5 results,  Open Access: 5 results,  Acknowledgement Compliant: 4 results) Presentation (2 results) (of which Int'l Joint Research: 2 results)

  • [Journal Article] Hyperammonemia crisis following parturition in a female patient with ornithine transcarbamylasedeficiency.2017

    • Author(s)
      Kido J, Kawasaki T, Mitsubuchi H, Kamohara H, Ohba T, Matsumoto F, Endo F, Nakamura K.
    • Journal Title

      World J Hepatol.

      Volume: 9(6) Issue: 6 Pages: 343-348

    • DOI

      10.4254/wjh.v9.i6.343

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Pulmonary artery hypertension in methylmalonic acidemia.2017

    • Author(s)
      Kido J, Mitsubuchi H, Sakanashi M, Matsubara J, Matsumoto S, Sakamoto R, Endo F, Nakamura K.
    • Journal Title

      Hemodial Int.

      Volume: Apr;21(2) Issue: 2

    • DOI

      10.1111/hdi.12506

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] Plasma exchange and chelator therapy rescues acute liver failure in Wilson disease without liver transplantation.2017

    • Author(s)
      Kido J, Matsumoto S, Momosaki K, Sakamoto R, Mitsubuchi H, Inomata Y, Endo F, Nakamura K
    • Journal Title

      Hepatol Res.

      Volume: Mar;47(4) Issue: 4 Pages: 359-363

    • DOI

      10.1111/hepr.12711

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] Evaluation of the skin-prick test for predicting the outgrowth of cow's milk allergy.2016

    • Author(s)
      Kido J, Hirata M, Ueno H, Nishi N, Mochinaga M, Ueno Y, Yanai M, Johno M, Matsumoto T.
    • Journal Title

      Allergy Rhinol (Providence)

      Volume: Jan 1;7(3) Issue: 3 Pages: 139-143

    • DOI

      10.2500/ar.2016.7.0175

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] Advanced Endometrial Cancer in Phenylketonuria2016

    • Author(s)
      Jun Kido, Hiroshi Mitsubuchi, Fumiko Itoh, Takanobu Yoshida, Shirou Matsumoto, Rieko Sakamoto, Fumio Endo, Kimitoshi Nakamura
    • Journal Title

      Med Sci Case Rep

      Volume: 3 Pages: 108-111

    • DOI

      10.12659/mscr.901499

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Presentation] Plasma exchange and chelator therapy rescues acute liver failure in Wilson disease without liver transplantation2016

    • Author(s)
      Jun Kido, Shirou Matsumoto, Ken Momosaki, Rieko Sakamoto, Hiroshi Mitsubuchi,
    • Organizer
      第58回日本先天代謝異常学会
    • Place of Presentation
      東京
    • Year and Date
      2016-10-27
    • Related Report
      2016 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Improved neurodevelopmental outcomes in patients with urea cycle disorders after liver transplantation.2016

    • Author(s)
      Kido Jun
    • Organizer
      Society for the study of inborn errors of metabolism
    • Place of Presentation
      ローマ
    • Year and Date
      2016-09-06
    • Related Report
      2016 Annual Research Report
    • Int'l Joint Research

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Published: 2014-04-04   Modified: 2018-03-22  

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