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Analysis of association between GEM body and motor neuron death

Research Project

Project/Area Number 24790875
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionNiigata University

Principal Investigator

YOKOSEKI Akio  新潟大学, 医歯(薬)学総合研究科, 特任准教授 (90515719)

Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2014: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2012: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
KeywordsALS / TDP-43 / GEM / SMN / spinal motor neuron / spinal cord
Outline of Final Research Achievements

TAR DNA binding protein of 43 kDa (TDP-43) is a nuclear protein and plays an important role for onset of amyotrophic lateral sclerosis (ALS). TDP-43 has been reported to associate with some nuclear bodies: Cajal body, GEM body, and PML. Therefore,I studied association between TDP-43 and GEM body in ALS pathology. The number of GEM bodies decreased in spinal motor neurons with ALS than those with normal; the volume of GEM bodies was not different between spinal motor neurons with ALS and normals. Moreover, the number of GEM bodies was similar between spinal motor neurons with and without nuclear TDP-43. These results suggest that decreased number of GEM bodies may be caused by dysfunction of TDP-43 in ALS.

Report

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

    (11 results)

All 2014 2013 2012 Other

All Journal Article (7 results) (of which Peer Reviewed: 7 results,  Acknowledgement Compliant: 1 results,  Open Access: 1 results) Presentation (1 results) Book (2 results) Remarks (1 results)

  • [Journal Article] The circulating level of leptin and blood pressure in patients with multiple system atrophy.2014

    • Author(s)
      Ozawa T, Tokunaga J, Arakawa M, Ishikawa A, Takeuchi R, Yokoseki A, Sone H, Nishizawa M.
    • Journal Title

      Journal of the Neurological Sciences

      Volume: 347 Issue: 1-2 Pages: 349-51

    • DOI

      10.1016/j.jns.2014.09.045

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Multifocal hits for propagation of prion protein in sporadic Creutzfeldt-Jakob disease.2014

    • Author(s)
      Kasuga K, Takeuchi R, Takahashi T, Matsubara N, Koike R, Yokoseki A, Nishizawa M.
    • Journal Title

      Neurol Neuroimmunol Neuroinflamm.

      Volume: 2 Issue: 1

    • DOI

      10.1212/nxi.0000000000000053

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Sporadic ALS with compound heterozygous mutation in the SQSTM1 gene.2013

    • Author(s)
      Shimizu H, Toyoshima Y, Shiga A, Yokoseki A, Arakawa K, Sekine Y, Shimohata T, Ikeuchi T, Nishizawa M, Kakita A, Onodera O, Takahashi H
    • Journal Title

      Acta Neuropathol

      Volume: 126 Issue: 3 Pages: 453-459

    • DOI

      10.1007/s00401-013-1150-5

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Decreased number of Gemini of coiled bodies and U12 snRNA level in amyotrophic lateral sclerosis.2013

    • Author(s)
      Ishihara T, Ariizumi Y, Shiga A, et al ;
    • Journal Title

      Human Molecular Genetics

      Volume: 22 Issue: 20 Pages: 4136-4147

    • DOI

      10.1093/hmg/ddt262

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Abnormal ghrelin secretion contributes to gastrointestinal symptoms in multiple system atrophy patients.2013

    • Author(s)
      Ozawa T, Tokunaga J, Arakawa M, Ishikawa A, Takeuchi R, Mezaki N, Miura T, Sakai N, Hokari M, Takeshima A, Utsumi K, Kondo T, Yokoseki A, Nishizawa M
    • Journal Title

      Journal of Neurology

      Volume: 260 Issue: 8 Pages: 2073-2077

    • DOI

      10.1007/s00415-013-6944-9

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72.2013

    • Author(s)
      Konno T
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 84(4) Issue: 4 Pages: 398-401

    • DOI

      10.1136/jnnp-2012-302272

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Alteration of P0LDIP3 Splicing Associated with Loss of Function of TDP-43 in Tissues Affected with ALS2012

    • Author(s)
      Shiga A, et al ;
    • Journal Title

      PLoS ONE

      Volume: 7(8) Issue: 8 Pages: e43120-e43120

    • DOI

      10.1371/journal.pone.0043120

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Presentation] 筋萎縮性側索硬化症の脊髄前角細胞におけるCajal小体数の減少2013

    • Author(s)
      横関明男、石原智彦、志賀篤、小山哲秀、西澤正豊、譚春鳳、 高橋均、佐藤達哉、小野寺理
    • Organizer
      第54回日本神経学会学術大会
    • Place of Presentation
      東京国際フォーラム
    • Related Report
      2013 Research-status Report
  • [Book] 別冊日本臨床 神経症候群第2版II 眼球運動失行と低アルブミン血症を伴う早発型失調症2014

    • Author(s)
      横関明男,西澤正豊,小野寺理
    • Total Pages
      903
    • Publisher
      日本臨床社
    • Related Report
      2013 Research-status Report
  • [Book] アクチュアル脳・神経疾患の臨床 すべてがわかるALS・運動ニューロン疾患 第IV章ALSの病態関連遺伝子と遺伝子変異 TDP-432013

    • Author(s)
      横関明男,西澤正豊,小野寺理
    • Total Pages
      370
    • Publisher
      中山書店
    • Related Report
      2013 Research-status Report
  • [Remarks] 新潟大学脳研究所 神経内科 研究紹介

    • URL

      http://www.bri.niigata-u.ac.jp/~neuroweb/laboratory/research_basic_002.html

    • Related Report
      2012 Research-status Report

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

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