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2012 Fiscal Year Final Research Report

Molecular Pathogenesis of Familial Amyotrophic Lateral SclerosisType 6

Research Project

  • PDF
Project/Area Number 22500335
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurochemistry/Neuropharmacology
Research InstitutionHiroshima Bunkyo Women's University

Principal Investigator

FUJII Ritsuko  広島文教女子大学, 人間科学部, 教授 (90342716)

Project Period (FY) 2010 – 2012
Keywords精神・神経疾患の病態と治療
Research Abstract

Recently, specific point mutations in C-terminus region of TLS have been identified as a cause of familial amyotrophic lateral sclerosis (FALS) type6. TLS with ALS-related point mutations are prone to aggregate and TLS-deficiency is correlated with defective RNA splicing of specific sets of RNAs. Our iCLIP analysis of TLS-deficient mouse brain and mass spectrometry analysis of TLS-protein complex have indicated that TLS-deficiency may induce mitochondrial stress responses and aberrant RNA metabolism in neurons.

  • Research Products

    (7 results)

All 2012 2011 2010 Other

All Journal Article (4 results) Presentation (2 results) Book (1 results)

  • [Journal Article] Expression of NR1 and NR2 splice variants in TLS-deficient mice2012

    • Author(s)
      R.Fujii,N. Nishimura and C. Yamamoto:
    • Journal Title

      J. Neurochem

      Volume: 123(S1) Pages: 120

  • [Journal Article] RNA binding region of TLS interacts with nucleoporin.2012

    • Author(s)
      R.Fujii and T. Takumi
    • Journal Title

      J. Neurochem.

      Volume: 123 (S1) Pages: 60

  • [Journal Article] RNA binding protein TLS and neuronal disorders.2010

    • Author(s)
      R.Fujii
    • Journal Title

      Journal of the Society for Food Nutrition

      Volume: 27/28 Pages: 2-5

  • [Journal Article] Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain

    • Author(s)
      B. Rogelj, L.E. Easton, G.K. Bogu, L.W.Stanton, G. Rot, T. Curk, B. Zupan, Y. Sugimoto, M. Modic, N. Haberman, J. Tollervey, R.Fujii, T. Takumi, C E. Shaw and J. Ule
    • Journal Title

      Sci. Rep

      Volume: 2

    • DOI

      doi:10.1038/srep00603, 2012.

  • [Presentation] Expression of NR1 and NR2 splice variants in TLS-deficient mice.2012

    • Author(s)
      R.Fujii,N. Nishimura and C. Yamamoto:
    • Organizer
      日本神経化学会
    • Year and Date
      20120000
  • [Presentation] RNA bindingregion of TLS interacts with nucleoporin.2012

    • Author(s)
      R.Fujii and T. Takumi
    • Organizer
      日本神経化学会
    • Year and Date
      20120000
  • [Book] Chapter 9 Animal Models of ALS. Animal models for neurodegenerative disease2011

    • Author(s)
      R.Fujii and T. Takumi
    • Total Pages
      177-213
    • Publisher
      RSC Publishing, Cambridge, UK

URL: 

Published: 2014-08-29  

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