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Updated: Aug 10, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle
J M Ervasti1, K Ohlendieck, S D Kahl
1Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242.
Abstract:
Dystrophin, the protein encoded by the Duchenne muscular dystrophy (DMD) gene, exists in a large oligomeric complex. We show here that four glycoproteins are integral components of the dystrophin complex and that the concentration of one of these is greatly reduced in DMD patients. Thus, the absence of dystrophin may lead to the loss of a dystrophin-associated glycoprotein, and the reduction in this glycoprotein may be one of the first stages of the molecular pathogenesis of muscular dystrophy.
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