Journal article

Making Fast-Twitch Dystrophic Muscles Bigger Protects Them from Contraction Injury and Attenuates the Dystrophic Pathology

Stefan M Gehrig, Rene Koopman, Timur Naim, Clarissa Tjoakarfa, Gordon S Lynch

AMERICAN JOURNAL OF PATHOLOGY | ELSEVIER SCIENCE INC | Published : 2010

Abstract

The lack of functional dystrophin protein in Duchenne muscular dystrophy (DMD) renders muscle fibers highly fragile and susceptible to damage during contractions. Contraction-mediated injury is a major contributor to the progressive degeneration and etiology of muscle wasting in DMD. The prevailing understanding is that large fibers are highly susceptible to contraction damage and are affected preferentially, whereas smaller fibers are relatively spared in DMD. We tested the hypothesis that a pharmacological treatment that caused myofiber hypertrophy would increase the susceptibility of muscles from dystrophin-deficient mdx mice to contraction-induced injury, and thus aggravate the dystrophi..

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Grants

Funding Acknowledgements

Supported by the Association Francaise contre les Myopathies (AFM, France). S.M.G. was supported by a National Heart Foundation scholarship. R.K. is supported by a Rubicon Fellowship for the Netherlands Organisations for Scientific Research.