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Participation in ALS pathology of ALS linked mutant SOD1 proteins accumulated in MAM

Research Project

Project/Area Number 24700391
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Neurochemistry/Neuropharmacology
Research InstitutionDoshisha University

Principal Investigator

WATANABE Shoji  同志社大学, 高等研究教育機構, 助教 (80462745)

Project Period (FY) 2012 – 2013
Project Status Completed (Fiscal Year 2013)
Budget Amount *help
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2013: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Fiscal Year 2012: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
KeywordsSOD1 / ALS / MAM / Kv2.1 / 変異SOD1 / Sigma-1レセプター
Research Abstract

SOD1 is a major causative protein of familial amyotrophic lateral screosis (fALS). I found that fALS linked mutant SOD1 proteins are specifically accumulated in mitochondria-ER associated membrane (MAM) fraction. The toxic mechanisms by this event, however, are still unknown. I hypothesized that Sigma-1 receptor (SigR1), which is a major MAM protein was inhibited by the accumulation of mutant SOD1 proteins in MAM. In this study, I found two novel finding (1) SigR1 was directly interacted with Kv2.1 and Kv4.2 (2) SigR1 was related with the localization of Kv2.1. Although I need to investigate these results more, these findings might be contributed to elucidation of the pathomechanism of fALS.

Report

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

    (4 results)

All 2014 Other

All Journal Article (2 results) Remarks (2 results)

  • [Journal Article] Specific sorting and post-Golgi trafficking of dendric potassium channels in living neuron.2014

    • Author(s)
      Jensen C. S., Watanabe, S., Rasmussen H. B., Schmitt, N., Olsen S. P., Frost N. A., Blanpied T.A., Misonou, H.
    • Journal Title

      Journal of Biological chemistry

      Volume: vol.289(15) Pages: 10566-10581

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Journal Article] Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosis2014

    • Author(s)
      Nomura, T., Watanabe, S., Kaneko, K., Yamanaka, K., Nukina, N., Furukawa, Y.
    • Journal Title

      Journal of Biological chemistry

      Volume: vol.289(2) Pages: 1192-1202

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Remarks] 同志社大学研究者データベース

    • URL

      https://kenkyudb.doshisha.ac.jp/rd/html/japanese/researchersHtml/112206/112206_Researcher.html

    • Related Report
      2012 Research-status Report
  • [Remarks] 同志社大学脳科学研究科チャネル病態生理部門

    • URL

      http://brainscience.doshisha.ac.jp/introduction/pat/cp.html

    • Related Report
      2012 Research-status Report

URL: 

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

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