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A pathogenesis study of Perry syndrome

Research Project

Project/Area Number 26860678
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionFukuoka University

Principal Investigator

Mishima Takayasu  福岡大学, 医学部, 助教 (00600602)

Project Period (FY) 2014-04-01 – 2018-03-31
Project Status Completed (Fiscal Year 2017)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2016: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2015: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
KeywordsPerry症候群 / DCTN1 / TDP-43 / モデルマウス / iPS細胞 / 診断基準 / トランスジェニックマウス / ダイナクチン / 家族性パーキンソン病 / パーキンソン病
Outline of Final Research Achievements

【Clinical study】We reported clinical features of patients with Perry syndrome. We established international diagnostic criteria for Perry syndrome.
【Basic study】We showed that Perry syndrome is a distinctive type of TDP-43 proteinopathy. We generated induced pluripotent stem cells (iPSCs) from a Perry syndrome patient and differentiated iPSCs into tyrosine hydroxylase (TH)-positive neurons. TH-positive neurons from a patient with Perry syndrome had dynactin aggregations in cytoplasm. Patient TH-positive neurons recapitulated an aspect of the disease phenotype of Perry syndrome. We generated DCTN1 G71A transgenic mice. These mice showed decreased exploratory activity and impaired motor coordination. These behavioral defects paralleled apathy-like symptoms and parkinsonism encountered in Perry syndrome.

Report

(5 results)
  • 2017 Annual Research Report   Final Research Report ( PDF )
  • 2016 Research-status Report
  • 2015 Research-status Report
  • 2014 Research-status Report
  • Research Products

    (18 results)

All 2018 2017 2016 2015 Other

All Int'l Joint Research (4 results) Journal Article (8 results) (of which Int'l Joint Research: 3 results,  Peer Reviewed: 7 results,  Open Access: 2 results,  Acknowledgement Compliant: 3 results) Presentation (6 results) (of which Int'l Joint Research: 4 results)

  • [Int'l Joint Research] Department of Neuroscience, Mayo Clinic/Department of Neurology, Mayo Clinic(米国)

    • Related Report
      2017 Annual Research Report
  • [Int'l Joint Research] University of British Columbia(カナダ)

    • Related Report
      2017 Annual Research Report
  • [Int'l Joint Research]

    • Related Report
      2017 Annual Research Report
  • [Int'l Joint Research] Mayo Clinic Jacksonville(米国)

    • Related Report
      2016 Research-status Report
  • [Journal Article] Establishing diagnostic criteria for Perry syndrome2018

    • Author(s)
      Mishima T, Fujioka S, Tomiyama H, Yabe I, Kurisaki R, Fujii N, Neshige R, Ross OA, Farrer MJ, Dickson DW, Wszolek ZK, Hattori N, Tsuboi Y
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 89 Issue: 5 Pages: 482-487

    • DOI

      10.1136/jnnp-2017-316864

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Behavioral defects in a DCTN1 G71A transgenic mouse model of Perry syndrome2018

    • Author(s)
      Mishima T, Deshimaru M, Watanabe T, Kubota K, Kinoshita-Kawada M, Yuasa-Kawada J, Takasaki K, Uehara Y, Jinno S, Iwasaki K, Tsuboi Y
    • Journal Title

      Neurosci Lett

      Volume: 666 Pages: 98-103

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Perry症候群2018

    • Author(s)
      三嶋崇靖,坪井義夫
    • Journal Title

      Frontiers in Parkinson Disease

      Volume: 11 Pages: 28-31

    • Related Report
      2017 Annual Research Report
  • [Journal Article] Dysphagia in Perry syndrome: pharyngeal pressure of two cases2017

    • Author(s)
      Umemoto G, Tsuboi Y, Furuya H, Mishima T, Fujioka S, Fujii N, Arahata H, Sugahara M, Sakai M
    • Journal Title

      Case report in Neurology

      Volume: 9 Issue: 2 Pages: 161-167

    • DOI

      10.1159/000457900

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Reduced orexin immunoreactivity in Perry syndrome and multiple system atrophy2017

    • Author(s)
      Mishima T, Kasanuki K, Koga S, Castanedes-Casey M, Wszolek ZK, Tsuboi Y, Dickson DW
    • Journal Title

      Parkinsonism Relat Disord

      Volume: 42 Pages: 85-89

    • DOI

      10.1016/j.parkreldis.2017.06.003

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Perry Syndrome: A Distinctive type of TDP-43 Proteinopathy2017

    • Author(s)
      Mishima T, Koga S, Lin WL, Kasanuki K, Castanedes-Casey M, Wszolek ZK, Oh SJ, Tsuboi Y, Dickson DW
    • Journal Title

      J Neuropathol Exp Neurol

      Volume: 76 Issue: 8 Pages: 676-682

    • DOI

      10.1093/jnen/nlx049

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Cytoplasmic aggregates of dynactin in iPSC-derived tyrosine hydroxylase-positive neurons from a patient with Perry syndrome2016

    • Author(s)
      Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
    • Journal Title

      Parkinsonism & Related Disorders

      Volume: 30 Pages: 67-72

    • DOI

      10.1016/j.parkreldis.2016.06.007

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Impulse control disorders and punding in Perry syndrome.2015

    • Author(s)
      Mishima T, Fujioka S, Kurisaki R, Yanamoto S, Higuchi MA, Tsugawa J, Fukae J, Neshige R, Tsuboi Y
    • Journal Title

      Parkinsonism & Related Disorders

      Volume: 21 Issue: 11 Pages: 1381-1382

    • DOI

      10.1016/j.parkreldis.2015.09.037

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Presentation] Perry syndrome is a distinctive type of TDP-43 proteinopathy2017

    • Author(s)
      Mishima T, Koga S, Lin WL, Kasanuki K, Castanedes-Casey M, Wszolek ZK, Oh SJ, Tsuboi Y, Dickson DW
    • Organizer
      XXIII World Congress of Neurology
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Human iPSC disease modeling of Perry syndrome2017

    • Author(s)
      Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
    • Organizer
      XXIII World Congress of Neurology
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Disease modeling for Perry syndrome using patient induced pluripotent stem cells2017

    • Author(s)
      Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
    • Organizer
      21th International Congress of Parkinson's Disease and Movement Disorders
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Perry症候群におけるマイネルト基底核のオレキシン染色性の低下2017

    • Author(s)
      三嶋 崇靖, 笠貫 浩史, 古賀 俊輔, Monica Castanedes-Casey, Zbigniew K. Wszolek, 坪井 義夫, Dennis W. Dickson
    • Organizer
      第11回パーキンソン病運動障害疾患コングレス
    • Related Report
      2017 Annual Research Report
  • [Presentation] A cellular model for Perry syndrome using patient iPSCs2016

    • Author(s)
      Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
    • Organizer
      第57回日本神経学会学術大会
    • Place of Presentation
      神戸コンベンションセンター(兵庫・神戸市)
    • Year and Date
      2016-05-18
    • Related Report
      2016 Research-status Report
  • [Presentation] Impulse control disorders and punding in Perry syndrome2015

    • Author(s)
      Mishima T, Fujioka S, Kurisaki R, Yanamoto S, Higuchi MA, Tsugawa J, Fukae J, Neshige R, Tsuboi Y
    • Organizer
      XXI Word Congress on Parkinson’s disease and Related Disorders
    • Place of Presentation
      イタリア
    • Year and Date
      2015-12-06
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research

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Published: 2014-04-04   Modified: 2022-06-07  

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