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Genetic analysis and establishment of in vitro model for inclusion body myositis

Research Project

Project/Area Number 26670436
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionTohoku University

Principal Investigator

Aoki Masashi  東北大学, 医学(系)研究科(研究院), 教授 (70302148)

Co-Investigator(Kenkyū-buntansha) Kato MASAAKI  東北大学, 病院, 助教 (50622479)
SUZUKI Naoki  東北大学, 病院, 助教 (70451599)
WARITA Hitoshi  東北大学, 大学院医学系研究科, 助教 (30400245)
Project Period (FY) 2014-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2014: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Keywords封入体筋炎 / 骨格筋 / プロテアソーム
Outline of Final Research Achievements

Sporadic inclusion body myositis (sIBM) is an intractable and progressive skeletal muscle disease of unknown etiology and without effective treatment. Muscle biopsy typically reveals endomysial inflammation, invasion of mononuclear cells into non-necrotic fibers and rimmed vacuoles, suggesting inflammation and degeneration co-exist as part of the pathomechanism. We made muscle specific proteasomal deficient mice and reported in JCS paper. We also found familial case of inclusion body myopathy with the mutation in hnRNPA1. We also established iPS cells from these patients.

Report

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

    (2 results)

All 2015 2014

All Journal Article (2 results) (of which Peer Reviewed: 2 results,  Open Access: 2 results)

  • [Journal Article] A mutation in hnRNPA1 causes isolated inclusion body myopathy in two families with multisystem proteinopathy2015

    • Author(s)
      Izumi R, Warita H, Niihori T, Takahashi T, Tateyama M, Suzuki N, Nishiyama A, Shirota M, Funayama R, Nakayama K, Mitsuhashi S, Nishino I, Aoki Y, Aoki M.
    • Journal Title

      Neurology Genetics

      Volume: NA

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Proteasome dysfunction induces muscle growth defects and protein aggregation2014

    • Author(s)
      Kitajima Y, Tashiro Y, Suzuki N, Warita H, Kato M, Tateyama M, Ando R, Izumi R, Yamazaki M, Abe M, Sakimura K, Ito H, Urushitani M, Nagatomi R, Takahashi R, and Aoki M
    • Journal Title

      J Cell Sci

      Volume: 127 Pages: 5204-5217

    • DOI

      10.1242/jcs.150961

    • Related Report
      2014 Research-status Report
    • Peer Reviewed / Open Access

URL: 

Published: 2014-04-04   Modified: 2017-05-10  

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