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Search for the candidate gene of a novel hereditary cerebellar degeneration : an approach using proteomics.

Research Project

Project/Area Number 15500231
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionNiigata University

Principal Investigator

TOYOSHIMA Yasuko  Niigata University, Brain Research Institute, Assistant, 脳研究所, 助手 (20334675)

Co-Investigator(Kenkyū-buntansha) TAKAHASHI Hitoshi  Niigata University, Brain Research Institute, Professor, 脳研究所, 教授 (90206839)
Project Period (FY) 2003 – 2004
Project Status Completed (Fiscal Year 2004)
Budget Amount *help
¥3,800,000 (Direct Cost: ¥3,800,000)
Fiscal Year 2004: ¥1,600,000 (Direct Cost: ¥1,600,000)
Fiscal Year 2003: ¥2,200,000 (Direct Cost: ¥2,200,000)
KeywordsCerebellar degeneration / 1 C2 / polyglutamine disease / western blotting / two dimensional electrophoresis / アミノ酸配列 / ポリグルタミン / 脊髄小脳変性症
Research Abstract

We have found three novel polyglutamine (polyQ) disease families in last four years. In histological examination of the nervous system, each case has shown unique distribution of the polyQ-positive neuronal nuclei. We revealed one case was a homozygote of SCA 17,and reported the clinico-pathological findings.
After informed consent, we analyzed the protein extracted from autopsied brain. We found a case had an extra band in western blotting pattern using antibody to polyQ stretches (1 C2).
To profile the expression of proteins, we used 2D fluorescence difference gel electrophoresis (2D-DIGE) system (Amersham Bioscience). We chose a patient, who had been revealed as novel polyQ disease, and six controls. The protein samples were extracted from their cerebellum, and were labeled with CyDyes (patient : Cy5,control : Cy3,and internal standard : Cy2). The samples were separated over first and second dimensions according to their charge and size, respectively. Once the samples had been separated in the second dimension, gels were scanned for Cy2,Cy3 and Cy5 fluorescence using an appropriate scanner, Typhoon^<TM> 9400 imager (Amersham Bioscience). And image analysis was performed using DeCyder (Amersham Bioscience). As a result, we discovered a certain protein which was expressed massively in the patient's brain. Besides, from the result of 2D western blotting, the protein was the very thing that we have recognized as an extra band in the ID western blotting with 1 C2. We picked the protein spot, and sequenced the peptide fragments by MALDI-TOF mass spectrometry.

Report

(3 results)
  • 2004 Annual Research Report   Final Research Report Summary
  • 2003 Annual Research Report
  • Research Products

    (9 results)

All 2004 2003 Other

All Journal Article (8 results) Publications (1 results)

  • [Journal Article] Spinocerebellar ataxia type 17 repeat in patients with Huntington's disease-like and ataxia, reply2004

    • Author(s)
      Yasuko Toyoshima
    • Journal Title

      Annals of Neurology 56(1)

      Pages: 163-164

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Annual Research Report 2004 Final Research Report Summary
  • [Journal Article] SCA 17 homozygote showing Huntington's disease-like phenotype2004

    • Author(s)
      Yasuko Toyoshima
    • Journal Title

      Annals of Neurology 55(2)

      Pages: 281-286

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Annual Research Report 2004 Final Research Report Summary
  • [Journal Article] Spinocerebellar ataxia type 17 repeat in patients with Huntington's disease-like and ataxia, Reply.2004

    • Author(s)
      Toyoshima Y, Yamada M, Onodera O, Shimohata M, Inenaga C, Fujita N, Morita M, Tsuji S, Takahashi H.
    • Journal Title

      Ann Neurol 56

      Pages: 163-164

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] SCA 17 homozygote showing Huntington's disease-like phenotype.2004

    • Author(s)
      Toyoshima Y, Yamada M, Onodera O, Shimohata M, Inenaga C, Fujita N, Morita M, Tsuji S, Takahashi H.
    • Journal Title

      Ann Neurol 55

      Pages: 281-286

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Extracerebral ectopic mass with huge cysts in the anterior cranial cavity2003

    • Author(s)
      Yasuko Toyoshima
    • Journal Title

      Neuropathology 23

      Pages: 301-306

    • NAID

      10013404880

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Pathological involvement of the motor neuron system and hippocampal formation in motor neuron disease-inclusion dementia2003

    • Author(s)
      Yasuko Toyoshima
    • Journal Title

      Acta Neuropathol 106

      Pages: 50-56

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Extracerebral ectopic mass with huge cysts in the anterior cranial cavity.2003

    • Author(s)
      Toyoshima Y, Kakita A, Yamada M, Sato G, Mori H, Okamoto K, Tanaka R, Takahashi H.
    • Journal Title

      Neuropathology 23

      Pages: 301-306

    • NAID

      10013404880

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Pathological involvement of the motor neuron system and hippocampal formation in motor neuron disease-inclusion dementia.2003

    • Author(s)
      Toyoshima Y, Piao YS, Tan OF, Morita M, Tanaka M, Oyanagi K, Okamoto K, Takahashi H.
    • Journal Title

      Acta Neuropathol 106

      Pages: 50-56

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Publications] Yasuko Toyoshima: "SCA 17 homozygote showing Huntington's disease-like phenotype"Annals of Neurology. 55. 281-286 (2004)

    • Related Report
      2003 Annual Research Report

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Published: 2003-04-01   Modified: 2016-04-21  

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