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Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy

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Abstract

Duchenne muscular dystrophy (DMD) is a degenerative skeletal muscle disease that makes walking and breathing difficult. DMD is caused by an X-linked (Xp21) mutation in the dystrophin gene. Dystrophin is a scaffolding protein located in the sarcolemmal cytoskeleton, important in maintaining structural integrity and regulating muscle cell (muscle fiber) growth and repair. Dystrophin deficiency in mouse models (e.g., mdx mouse) destabilizes the interface between muscle fibers and the extracellular matrix, resulting in profound damage, inflammation, and weakness in diaphragm and limb muscles. While the link between dystrophin deficiency with inflammation and pathology is multi-factorial, elevated oxidative stress has been proposed as a central mediator. Unfortunately, the use of non-specific antioxidant scavengers in mouse and human studies has led to inconsistent results, obscuring our understanding of the importance of redox signaling in pathology of muscular dystrophy. However, recent studies with more mechanistic approaches in mdx mice suggest that NAD(P)H oxidase and nuclear factor-kappaB are important in amplifying dystrophin-deficient muscle pathology. Therefore, more targeted antioxidant therapeutics may ameliorate damage and weakness in human population, thus promoting better muscle function and quality of life. This review will focus upon the pathobiology of dystrophin deficiency in diaphragm and limb muscle primarily in mouse models, with a rationale for development of targeted therapeutic antioxidants in DMD patients.

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Acknowledgments

This work is supported by the Sydney and JL Huffines research grant (JHK, JML) and NIH (AG017768, AR054084) (LVT, JML).

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Authors and Affiliations

  1. Department of Physical Medicine and Rehabilitation, University of Minnesota Medical School, Minneapolis, MN, 55455, USA

    Jong-Hee Kim & LaDora V. Thompson

  2. Department of Health and Kinesiology, Department of Biomedical Engineering, Texas A&M University, College Station, TX, USA

    Jong-Hee Kim & John M. Lawler

  3. Department of Kinesiology, Inha University, Incheon, Korea

    Hyo-Bum Kwak

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  1. Jong-Hee Kim
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  2. Hyo-Bum Kwak
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Correspondence to Jong-Hee Kim.

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Kim, JH., Kwak, HB., Thompson, L.V. et al. Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy. J Muscle Res Cell Motil 34, 1–13 (2013). https://doi.org/10.1007/s10974-012-9330-9

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