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Study of disease model fish with peroxisomal biogenesis disorder for identification of pathological agent and treatment.

Research Project

Project/Area Number 16K09963
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Pediatrics
Research InstitutionGifu University

Principal Investigator

Takashima Shigeo  岐阜大学, 研究推進・社会連携機構, 助教 (50537610)

Project Period (FY) 2016-04-01 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
Budget Amount *help
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2018: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2017: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2016: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Keywordsペルオキシソーム / ペルオキシソーム形成異常症 / 極長鎖脂肪酸 / プラズマローゲン / フィタン酸 / PEX遺伝子 / ゼブラフィッシュ / ペルオキシソーム病
Outline of Final Research Achievements

In this research, a disease model fish (pex2 mutant zebrafish) for human peroxisomal biogenesis disorder was established and analyzed. pex2 mutant fish was found to recapitulate human patients’ symptoms such as the neuromotor defect, liver steatosis, short life span. Accumulation of distinct fatty acid species such as very-long-chain fatty acids in each organ was revealed by means of mass spectrometry assay. In the model fish, the expression of many other genes, which were thought to be involved in the progress of the defects, were also affected.

Academic Significance and Societal Importance of the Research Achievements

ヒトペルオキシソーム形成異常症は患者における病態発症機構がわかっておらず、治療法開発のためにもその解明が待たれている。本研究により、これまで明らかでなかった器官ごとに異なる異常な脂肪酸の蓄積が確認された。これらの異常脂肪酸はヒト患者において器官ごとの病態発症の引き金になっている可能性があり、今後その検証を行うことで発症の原因を明らかにできると考えられる。多岐にわたる遺伝子の働きにも影響が及んでいることも明らかになったことから本研究成果により病態の発症原因と進行過程の一端を明らかにできたと考える。

Report

(4 results)
  • 2018 Annual Research Report   Final Research Report ( PDF )
  • 2017 Research-status Report
  • 2016 Research-status Report
  • Research Products

    (15 results)

All 2018 2017 Other

All Journal Article (5 results) (of which Peer Reviewed: 5 results) Presentation (8 results) (of which Int'l Joint Research: 2 results,  Invited: 1 results) Remarks (2 results)

  • [Journal Article] Analyses of the fatty acid separation principle using liquid chromatography-mass spectrometry2018

    • Author(s)
      Takashima S, Toyoshi K, Shimozawa N
    • Journal Title

      Medical Mass Spectrometry

      Volume: 2 Pages: 21-33

    • NAID

      130007897983

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Ataxic form of autosomal recessive PEX10-related peroxisome biogenesis disorders with a novel compound heterozygous gene mutation and characteristic clinical phenotype.2017

    • Author(s)
      Toru Yamashita, Jun Mitsui, Nobuyuki Shimozawa, Shigeo Takashima, Hiroshi Umemurad, Kota Sato, Mami Takemoto, Nozomi Hishikawa, Yasuyuki Ohta, Takashi Matsukawa, Hiroyuki Ishiura, Jun Yoshimura, Koichiro Doi, Shinichi Morishita, Shoji Tsuji, Koji Abe.
    • Journal Title

      J Neurological Sciences

      Volume: 375 Pages: 424-429

    • DOI

      10.1016/j.jns.2017.02.058

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] Effect of Lorenzo's Oil on Hepatic Gene Expression and the Serum Fatty Acid Level in abcd1-Deficient Mice.2017

    • Author(s)
      Morita M, Honda A, Kobayashi A, Watanabe Y, Watanabe S, Kawaguchi K, Takashima S, Shimozawa N, Imanaka T.
    • Journal Title

      JIMD Rep.

      Volume: 38 Pages: 67-74

    • DOI

      10.1007/8904_2017_32

    • ISBN
      9783662566091, 9783662566107
    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] bHLH proneural genes as cell fate determinants of entero-endocrine cells, an evolutionarily conserved lineage sharing a common root with sensory neurons2017

    • Author(s)
      Hartenstein Volker、Takashima Shigeo、Hartenstein Parvana、Asanad Samuel、Asanad Kian
    • Journal Title

      Developmental Biology

      Volume: 431 Issue: 1 Pages: 36-47

    • DOI

      10.1016/j.ydbio.2017.07.013

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] Detection of unusual very-long-chain fatty acid and ether lipid derivatives in the fibroblasts and plasma of patients with peroxisomal diseases using liquid chromatography-mass spectrometry.2017

    • Author(s)
      Shigeo Takashima, Kayoko Toyoshi, Takahiro Itoh, Naomi Kajiwara, Ayako Honda, Akiko Ohba, Shoko Takemoto, Satoshi Yoshida, Nobuyuki Shimozawa.
    • Journal Title

      Molecular Genetics and Metabolism

      Volume: 120 Issue: 3 Pages: 255-268

    • DOI

      10.1016/j.ymgme.2016.12.013

    • Related Report
      2016 Research-status Report
    • Peer Reviewed
  • [Presentation] ゲノム編集により作製したPEX3変異細胞を用いたペルオキシソーム形成異常症の病態解明と治療法に関する研究2018

    • Author(s)
      藤田遥, 高島茂雄, 下澤伸行, 平田洋子, 大橋憲太郎
    • Organizer
      日本生化学会中部支部第82回例会
    • Related Report
      2018 Annual Research Report
  • [Presentation] Pathological study of Zellweger syndrome using disease model fish2018

    • Author(s)
      Shigeo Takashima, Shoko Takemoto, Kayoko Toyoshi, Akiko Ohba, Nobuyuki Shimozawa
    • Organizer
      第60回日本先天代謝異常学会総会/第16回アジア先天代謝異常症シンポジウム
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Therapeutic effect by bezafibrate, using fibroblasts from patients with peroxisomal diseases2018

    • Author(s)
      Hiroki Kawai, Shigeo Takashima, Toshiyuki Fukao and Nobuyuki Shimozawa
    • Organizer
      第60回日本先天代謝異常学会総会/第16回アジア先天代謝異常症シンポジウム
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Pathological study of Zellweger syndrome using disease model fish2018

    • Author(s)
      Shigeo Takashima, Shoko Takemoto, Kayoko Toyoshi, Akiko Ohba, Nobuyuki Shimozawa
    • Organizer
      第41回日本分子生物学会年会
    • Related Report
      2018 Annual Research Report
  • [Presentation] PEX3変異細胞を用いたペルオキシソーム形成異常症発症因子の探索2018

    • Author(s)
      藤田遥, 大橋憲太郎, 平田洋子, 下澤伸行, 高島茂雄
    • Organizer
      第41回日本分子生物学会年会
    • Related Report
      2018 Annual Research Report
  • [Presentation] Study of the molecular pathology of the human peroxisome biogenesis disorder using zebrafish2017

    • Author(s)
      Shoko Takemoto, Kayoko Toyoshi, Akiko Ohba, Haruka Fujita, Nobuyuki Shimozawa, Shigeo Takashima
    • Organizer
      第50回 日本発生生物学会年会
    • Related Report
      2017 Research-status Report
  • [Presentation] Establishment and Phenotypic Analysis of the Disease Model Fish for Peroxisomal Biogenesis Disorder2017

    • Author(s)
      Shigeo Takashima, Shoko Takemoto, Kayoko Toyoshi, Akiko Ohba, Haruka Fujita, Kentaro Oh-hashi, Yoko Morita, Nobuyuki Shimozawa
    • Organizer
      第59回 日本先天代謝異常学会総会
    • Related Report
      2017 Research-status Report
  • [Presentation] 質量分析を応用したペルオキシソーム病代謝解析法の開発2017

    • Author(s)
      高島 茂雄
    • Organizer
      第42回 日本医用マススペクトル学会年会
    • Related Report
      2017 Research-status Report
    • Invited
  • [Remarks]

    • URL

      http://stakashimaj.blogspot.jp/p/blog-page.html

    • Related Report
      2017 Research-status Report
  • [Remarks] http://stakashimaj.blogspot.jp/p/blog-page.html

    • Related Report
      2016 Research-status Report

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Published: 2016-04-21   Modified: 2020-03-30  

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