Cancer Institute A national cancer institute
designated cancer center

Joseph Shrager

Publication Details

  • DYSTROPHIN PROTECTS THE SARCOLEMMA FROM STRESSES DEVELOPED DURING MUSCLE-CONTRACTION PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA Petrof, B. J., Shrager, J. B., Stedman, H. H., Kelly, A. M., Sweeney, H. L. 1993; 90 (8): 3710-3714

    Abstract:

    The protein dystrophin, normally found on the cytoplasmic surface of skeletal muscle cell membranes, is absent in patients with Duchenne muscular dystrophy as well as mdx (X-linked muscular dystrophy) mice. Although its primary structure has been determined, the precise functional role of dystrophin remains the subject of speculation. In the present study, we demonstrate that dystrophin-deficient muscle fibers of the mdx mouse exhibit an increased susceptibility to contraction-induced sarcolemmal rupture. The level of sarcolemmal damage is directly correlated with the magnitude of mechanical stress placed upon the membrane during contraction rather than the number of activations of the muscle. These findings strongly support the proposition that the primary function of dystrophin is to provide mechanical reinforcement to the sarcolemma and thereby protect it from the membrane stresses developed during muscle contraction. Furthermore, the methodology used in this study should prove useful in assessing the efficacy of dystrophin gene therapy in the mdx mouse.

    View details for Web of Science ID A1993KX81600123

    View details for PubMedID 8475120

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