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Developing new therapeutic drugs that inhibit oligomer formation of polyglutamine disease proteins based on the hypothesis of oligomer toxicity in polyglutamine diseases

Research Project

Project/Area Number 25461272
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Neurology
Research InstitutionNiigata University

Principal Investigator

Tada Masayoshi  新潟大学, 脳研究所, 助教 (10467079)

Co-Investigator(Kenkyū-buntansha) Onodera Osamu  新潟大学, 脳研究所, 教授 (20303167)
Project Period (FY) 2013-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2015: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2013: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Keywords脊髄小脳変性症 / ポリグルタミン病 / 治療 / 重合体 / 臨床評価法 / 小脳性運動失調 / 新規治療法 / 評価法 / 重合体形成 / SCD / ataxia / polyglutamine disease / therapy / oligomer
Outline of Final Research Achievements

We developed new therapeutic drugs that inhibit oligomer formation of polyglutamine disease proteins based on the hypothesis of oligomer toxicity in polyglutamine diseases. Using a disease nematode model, we found a small compound that inhibit oligomer formation of disease proteins and ameliorate motor function of the nematode model. We also developed a novel system using iPad for the quantitative assessment of cerebellar ataxia. In addition, we started a clinical trial to investigate the safety and efficiency of varenicline (Champix), a prescription medication used to treat nicotine addiction, in the treatment of spinocerebellar degenerations.

Report

(4 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • 2013 Research-status Report
  • Research Products

    (16 results)

All 2016 2015 2014 2013

All Journal Article (9 results) (of which Peer Reviewed: 4 results,  Acknowledgement Compliant: 2 results,  Open Access: 1 results) Presentation (6 results) (of which Invited: 1 results) Book (1 results)

  • [Journal Article] 小脳症状をどう捉えるか:小脳機能に基づく小脳症状の再構築2016

    • Author(s)
      他田正義, 西澤正豊, 小野寺理
    • Journal Title

      MDSJ Letters

      Volume: 9(1) Pages: 4-5

    • Related Report
      2015 Annual Research Report
  • [Journal Article] 高齢者における小脳失調症に対する診断アプローチ2016

    • Author(s)
      高橋茉那, 他田正義, 下畑享良, 西澤正豊
    • Journal Title

      Geriat Med

      Volume: 54 Pages: 217-223

    • Related Report
      2015 Annual Research Report
  • [Journal Article] Roles of inositol 1,4,5-trisphosphate receptors in spinocerebellar ataxias.2016

    • Author(s)
      Tada M, Nishizawa M, Onodera O.
    • Journal Title

      Neurochem Int.

      Volume: 94 Pages: 1-8

    • DOI

      10.1016/j.neuint.2016.01.007

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Redefining cerebellar ataxia in degenerative ataxias: lessons from recent research on cerebellar systems.2015

    • Author(s)
      Tada M, Nishizawa M, Onodera O
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: - Issue: 8 Pages: 922-928

    • DOI

      10.1136/jnnp-2013-307225

    • Related Report
      2015 Annual Research Report 2014 Research-status Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] A mutation of COX6A1 causes a recessive axonal or mixed form of Charcot-Marie-Tooth disease.2014

    • Author(s)
      Tamiya G, Makino S, Hayashi M, Abe A, Numakura C, Ueki M, Tanaka A, Ito C, Toshimori K, Ogawa N, Terashima T, Maegawa H, Yanagisawa D, Tooyama I, Tada M, Onodera O, Hayasaka K.
    • Journal Title

      Am J Hum Genet

      Volume: 95(3) Pages: 294-300

    • Related Report
      2014 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Hereditary Diffuse Leukoencephalopathy with Spheroids (HDLS): A Review of the Literature on its Clinical Characteristics and Mutations in the Colony-Stimulating Factor-1 Receptor Gene.2014

    • Author(s)
      Konno T, Tada M, Tada M, Nishizawa M, Ikeuchi T.
    • Journal Title

      Brain Nerve

      Volume: 66 Pages: 581-590

    • NAID

      40020089061

    • Related Report
      2014 Research-status Report
  • [Journal Article] セナタキシン欠損症2014

    • Author(s)
      他田正義,西澤正豊
    • Journal Title

      神経症候群(第2版)

      Volume: 27 Pages: 389-393

    • Related Report
      2013 Research-status Report
  • [Journal Article] Progressive myoclonus epilepsy: extraneuronal brown pigment deposition and system neurodegeneration in the brains of Japanese patients with novel SCARB2 mutations2013

    • Author(s)
      Fu YJ, Aida I, Tada M, Tada M, Toyoshima Y, Takeda S, Nakajima T, Naito H, Nishizawa M, Onodera O, Kakita A, and Takahashi H
    • Journal Title

      Neuropathol Appl Neurobiol

      Volume: doi:10.1111/nan.12057. Pages: 12057-12057

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 【小脳の神経学】 治療研究に向けた小脳機能評価法の将来2013

    • Author(s)
      徳永純, 他田正義, 永井貴大, 西澤正豊, 小野寺理
    • Journal Title

      神経内科

      Volume: 78 Pages: 687-694

    • Related Report
      2013 Research-status Report
  • [Presentation] 小脳症状をどう捉えるか:小脳機能に基づく小脳症状の再構築2015

    • Author(s)
      他田正義, 小野寺理, 西澤正豊
    • Organizer
      第9回パーキンソン病・運動障害疾患コングレス
    • Place of Presentation
      品川プリンスホテル(東京都品川区)
    • Year and Date
      2015-10-15
    • Related Report
      2015 Annual Research Report
    • Invited
  • [Presentation] Kinect を用いた3次元歩行解析システムによる小脳性歩行障害の解析2015

    • Author(s)
      他田正義, 徳永純, 小野寺理, 西澤正豊
    • Organizer
      第56回日本神経学会学術大会
    • Place of Presentation
      朱鷺メッセ(新潟県新潟市)
    • Year and Date
      2015-05-20
    • Related Report
      2015 Annual Research Report
  • [Presentation] SCARB2変異を認めた進行性ミオクローヌスてんかん 2剖検例の臨床病理・生化学的解析.2014

    • Author(s)
      他田正義,付永娟,会田泉,他田真理,武田茂樹,豊島靖子,中島孝,内藤明彦,高橋均,小野寺理,西澤正豊.
    • 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
      2014-05-21
    • Related Report
      2014 Research-status Report
  • [Presentation] iPatax:小脳性運動失調の新たな定量評価法.2014

    • Author(s)
      徳永純,他田正義,永井貴大,小野寺理,西澤正豊.
    • 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
      2014-05-21
    • Related Report
      2014 Research-status Report
  • [Presentation] iPatax: A new method for quantitative assessment for motor coordination of ataxia.2014

    • Author(s)
      Tada M, Tokunaga J, Nagai T, Nishizawa M, Onodera O.
    • Organizer
      AAN Annual Meeting 2014
    • Place of Presentation
      Philadelphia, PA
    • Year and Date
      2014-05-01
    • Related Report
      2014 Research-status Report
  • [Presentation] 等速直線運動の速度変動に着手した小脳性運動失調の新たな定量評価法2013

    • Author(s)
      徳永純,他田正義,永井貴大,小野寺理,西澤正豊
    • 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
  • [Book] Pathologies of calcium channels; Chapter 28, IP3 Receptors in Neurodegenerative Disorders: Spinocerebellar Ataxias and Huntington’s and Alzheimer’s Diseases2014

    • Author(s)
      Tada M, Nishizawa M, and Onodera O
    • Publisher
      Springer
    • Related Report
      2013 Research-status Report

URL: 

Published: 2014-07-25   Modified: 2019-07-29  

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