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Is endoplasmic reticulum stress essential for motor neuron degeneration?

Research Project

Project/Area Number 25860718
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionKumamoto University

Principal Investigator

Mori Akira  熊本大学, 医学部附属病院, 非常勤診療医師 (70608877)

Co-Investigator(Renkei-kenkyūsha) ERA Takumi  熊本大学, 発生医学研究所, 教授 (00273706)
Project Period (FY) 2013-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2015: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Keywords筋萎縮性側索硬化症 / 小胞体ストレス / 運動ニューロン / TDP-43 / FUS
Outline of Final Research Achievements

We investigated whether endoplasmic reticulum (ER) stress is essential for motor neuron degeneration in both TDP-43 and FUS/TLS-aggregation models. In motor neurons differentiated from iPS cells derived from ALS patients, some molecules on ER stress pathways were upregulated in mutant FUS/TLS expressing cells. However, only a few molecules were upregulated commonly in all different types of mutant FUS/TLS cells. In TDP-43-aggregation model, it was suggested that sarcoplasmic TDP-43 aggregation induced ER stress and then provided tissue toxicity. In our observations, both FUS/TLS and TDP-43 aggregation might induce ER stress. Therefore, regulation of ER stress pathways would be a therapeutic target for amyotrophic lateral sclerosis.

Report

(4 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • 2013 Research-status Report
  • Research Products

    (2 results)

All 2015 2013

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

  • [Journal Article] Clinicopathological features of the first Asian family having vocal cord and pharyngeal weakness with distal myopathy due to a MATR3 mutation.2015

    • Author(s)
      Yamashita S, Mori A, Nishida Y, Kurisaki R, Tawara N, Nishikami T, Misumi Y, Ueyama H, Imamura S, Higuchi Y, Hashiguchi A, Higuchi I, Morishita S, Yoshimura J, Uchino M, Takashima H, Tsuji S, Ando Y.
    • Journal Title

      Neuropathol Appl Neurobiol

      Volume: 41 Issue: 3 Pages: 391-398

    • DOI

      10.1111/nan.12179

    • Related Report
      2015 Annual Research Report 2014 Research-status Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Muscle biopsy findings predictive of malignancy in rare infiltrative dermatomyositis2013

    • Author(s)
      Uchino M, Yamashita S, Uchino K, Mori A, Hara A, Suga T, Hirahara T, Koide T, Kimura E, Yamashita T, Ueda A, Kurisaki R, Suzuki J, Honda S, Maeda Y, Hirano T, Ando Y
    • Journal Title

      Clin Neurol Neurosurg

      Volume: 115(5) Issue: 5 Pages: 603-6

    • DOI

      10.1016/j.clineuro.2012.07.019

    • Related Report
      2013 Research-status Report
    • Peer Reviewed

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Published: 2014-07-25   Modified: 2019-07-29  

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