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Journal of Muscle Research and Cell Motility

, Volume 34, Issue 1, pp 1–13 | Cite as

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

  • Jong-Hee KimEmail author
  • Hyo-Bum Kwak
  • LaDora V. Thompson
  • John M. Lawler
Review

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.

Keywords

Muscular dystrophies Oxidative stress Antioxidants nNOS NF-κB Cell signaling 

Notes

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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Copyright information

© Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  • Jong-Hee Kim
    • 1
    • 2
    Email author
  • Hyo-Bum Kwak
    • 3
  • LaDora V. Thompson
    • 1
  • John M. Lawler
    • 2
  1. 1.Department of Physical Medicine and RehabilitationUniversity of Minnesota Medical SchoolMinneapolisUSA
  2. 2.Department of Health and Kinesiology, Department of Biomedical EngineeringTexas A&M UniversityCollege StationUSA
  3. 3.Department of KinesiologyInha UniversityIncheonKorea

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