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Role of the Ubiquitin-Proteasome Pathway in Skeletal Muscle

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The Plasticity of Skeletal Muscle

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Abstract

Skeletal muscle is a plastic organ that is maintained by multiple pathways regulating cell and protein turnover. The ubiquitin-proteasome system (UPS) is now recognized as a major intracellular degradation system, and its proper function is critical to health and muscle homeostasis. Alterations in muscle proteasomes have been linked to several pathological phenotypes. Indeed, excessive or defective activity of UPS leads to detrimental effects on muscle homeostasis. Emerging evidence suggests that UPS can specifically target proteins that govern pathological signaling pathways for degradation, thus altering downstream effectors and disease outcomes. An increasing number of studies link abnormalities in the regulation of UPS to myofiber degeneration and muscle weakness. Therefore, an understanding of the pathogenic role of the proteostatic system in each inherited muscle disorder may provide novel therapeutic targets to counteract muscle wasting. In this section, we focus on the function of the proteasome system with respect to several diseases with altered proteostasis.

Study Funding

This study was supported by an Intramural Research Grant (26-7, 26-8) for Neurological and Psychiatric Disorders through the NCNP; a grant for Research on Rare and Intractable Diseases (H26-intractable disease 037 and 082, H26-nanchitou(nan)-ippan-079) from the Ministry of Health, Labour and Welfare of Japan; Health and Labour Sciences Research Grants for Comprehensive Research on Persons with Disabilities from Japan Agency for Medical Research and Development (15Adk0310043h0002); Grants-in-Aid for Research on Rare and Intractable Diseases; a Grant-in-Aid for Challenging Exploratory Research (26670436); a Grant-in-Aid for Young Scientists (B) (15K16486); a Grant-in-Aid for Young Scientists (A) (15H05667) from the Japanese Ministry of Education, Culture, Sports, Science and Technology; and a Grant-in-Aid for JSPS Fellows (16J00431).

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Acknowledgments

We thank Naoko Shimakura, Risa Ando, and Akiko Machii for their excellent technical assistance.

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Authors and Affiliations

  1. Division of Biomedical Engineering for Health and Welfare, Tohoku University Graduate School of Biomedical Engineering, 2-1 Seiryo-machi, Aoba-ku, Sendai, 980-8575, Japan

    Yasuo Kitajima

  2. Department of Stem Cell Biology, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, 852-8523, Japan

    Yasuo Kitajima

  3. Department of Neurology, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan

    Naoki Suzuki

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Correspondence to Yasuo Kitajima .

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  1. Institute for Liberal Arts, Environment and Society, Tokyo Institute of Technology, Tokyo, Japan

    Kunihiro Sakuma

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Kitajima, Y., Suzuki, N. (2017). Role of the Ubiquitin-Proteasome Pathway in Skeletal Muscle. In: Sakuma, K. (eds) The Plasticity of Skeletal Muscle. Springer, Singapore. https://doi.org/10.1007/978-981-10-3292-9_2

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