Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle
JM Ervasti, K Ohlendieck, SD Kahl, MG Gaver… - Nature, 1990 - nature.com
Nature, 1990•nature.com
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.
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.
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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