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Research for investigating Alexander disease using astrocytes differentiated from iPS cells

Research Project

Project/Area Number 24659433
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionKyoto Prefectural University of Medicine

Principal Investigator

NAKAGAWA Masanori  京都府立医科大学, 医学(系)研究科(研究院), 教授 (50198040)

Co-Investigator(Kenkyū-buntansha) YOSHIDA Tomokatsu  京都府立医科大学, 大学院医学研究科・神経内科, 講師 (90457987)
Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2013: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2012: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Keywordsアレキサンダー病 / GFAP / iPS細胞 / 末梢血リンパ球 / リンパ球 / アストロサイト / 遺伝子 / 脳神経疾患 / 神経科学 / iPS / GFAP
Outline of Final Research Achievements

Alexander disease is a neurodegenerative disorder, in which the genetic mutation of glial fibrillary acidic protein (GFAP), which is specifically expressed in astrocytes, is observed. Initial purpose was to establish iPS cells derived from skin fibroblast obtained by patients with Alexander disease and to induce to differentiate astrocytes. However, with recent technology advancement, we established iPS cells derived from peripheral lymphocyte obtained by a non-Alexander disease patient as preliminary experiment. We intend to differentiate astrocytes using the iPS cells.
To evaluate appropriate method to detect GFAP aggregates, which may be crucial for investigation using cell model, we compared some kinds of immunostaining methods. As a result, conventional GFAP immunostaining was found to be most useful.

Report

(4 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • 2012 Research-status Report
  • Research Products

    (13 results)

All 2015 2014 2013 2012 2011 Other

All Journal Article (5 results) (of which Peer Reviewed: 5 results,  Acknowledgement Compliant: 1 results) Presentation (6 results) Book (2 results)

  • [Journal Article] Characteristic abnormal signals in medulla oblongata-“eye spot” sign: four cases of elderly-onset Alexander disease.2015

    • Author(s)
      Yoshida T, Mizuta I, Saito K, Kimura Y, Park K, Ito Y, Haji S, Nakagawa M, Mizuno T.
    • Journal Title

      Neurology clinical practice

      Volume: - Issue: 3 Pages: 259-262

    • DOI

      10.1212/cpj.0000000000000124

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Effects of a polymorphism in the GFAP promoter on age of onset and ambulatory disability in late-onset Alexander disease.2013

    • Author(s)
      Yoshida T, Mizuta I, Saito K, et al.
    • Journal Title

      Journal of Human Genetics

      Volume: 58 Issue: 9 Pages: 635-638

    • DOI

      10.1038/jhg.2013.83

    • NAID

      10031195302

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 小児期発症が疑われ、長期間進行停止後に憎悪したと考えられたAlexander病の1例2013

    • Author(s)
      永石彰子,中根俊成,松尾秀徳,吉田誠克
    • Journal Title

      臨床神経

      Volume: 53 Pages: 474-477

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Late-onset Alexander disease with a V87L mutation in glial fibrillary acidic protein (GFAP) and calcifying lesions in the sub-cortex and cortex.2012

    • Author(s)
      Suzuki H, Yoshida T, Kitada M, Ichihashi J, Sasayama H, Nishikawa Y, Mistui Y, Nakagawa M, Kusunoki S.
    • Journal Title

      J Neurol

      Volume: 259(3) Issue: 3 Pages: 457-461

    • DOI

      10.1007/s00415-011-6201-z

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Clinical aspects and pathology of Alexander disease, and morphological and functional alteration of astrocyte induced by GFAP mutation2011

    • Author(s)
      Yoshida T, Nakagawa M
    • Journal Title

      Neuropathology

      Volume: 32 Issue: 4 Pages: 440-446

    • DOI

      10.1111/j.1440-1789.2011.01268.x

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Presentation] 変異GFAP凝集体を同定評価するための各染色法の検討.2014

    • Author(s)
      斉藤光象,吉田誠克,建部陽嗣,水田依久子,中川正法,水野敏樹.
    • Organizer
      第55回日本神経学会学術大会.
    • Place of Presentation
      福岡国際会議場
    • Year and Date
      2014-05-23
    • Related Report
      2014 Annual Research Report
  • [Presentation] 延髄前部のMRI異常信号は成人期発症アレキサンダー病の特徴的な所見である.2014

    • Author(s)
      吉田誠克,水田依久子,斉藤光象,中川正法, 木村康義,朴貴瑛,伊藤康男,椎裕章,高橋義秋,井手俊宏,土師正太郎,水野敏樹.
    • Organizer
      第55回日本神経学会総会学術大会
    • Place of Presentation
      福岡国際会議場
    • Year and Date
      2014-05-22
    • Related Report
      2014 Annual Research Report
  • [Presentation] 変異GFAP遺伝子導入SW13細胞質異常凝集体の同定はアレキサンダー病の診断に有用である

    • Author(s)
      齋藤光象、吉田誠克、中川正法、他
    • Organizer
      第54回日本神経学会総会.
    • Place of Presentation
      東京国際ファーラム
    • Related Report
      2013 Research-status Report
  • [Presentation] 変異GFAP凝集体を同定評価するための各染色法の検討

    • Author(s)
      齋藤光象、吉田誠克、中川正法、他
    • Organizer
      第55回日本神経学会総会.
    • Place of Presentation
      福岡
    • Related Report
      2013 Research-status Report
  • [Presentation] アレキサンダー病の臨床診断指針n対する有用性の検討・

    • Author(s)
      吉田誠克、水田依久子、齋藤光象、中川正法
    • Organizer
      第54回日本神経学会学術大会.
    • Place of Presentation
      東京
    • Related Report
      2013 Research-status Report
  • [Presentation] アレキサンダー病におけるGFAPプロモーター遺伝子多型解析.

    • Author(s)
      吉田 誠克、水田依久子、栗崎博司、大成圭子、陸雄一、林祐一、鈴木秀和、椎裕章、藤原康弘、大原亮、米津禎宏、末原雅人、中川正法.
    • Organizer
      第53回日本神経学会学術集会.
    • Place of Presentation
      東京
    • Related Report
      2012 Research-status Report
  • [Book] 別冊 日本臨床 新領域別症候群シリーズ No.26 神経症候群(第2版) III2014

    • Author(s)
      吉田誠克.
    • Total Pages
      4
    • Publisher
      日本臨牀社
    • Related Report
      2014 Annual Research Report
  • [Book] 別冊 日本臨床 新領域別症候群シリーズ No.26 神経症候群(第2版) IV2014

    • Author(s)
      吉田誠克.
    • Total Pages
      5
    • Publisher
      日本臨牀社
    • Related Report
      2014 Annual Research Report

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Published: 2013-05-31   Modified: 2019-07-29  

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