Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle.
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.
Ervasti
J M
JM
Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City 52242.
Ohlendieck
K
K
Kahl
S D
SD
Gaver
M G
MG
Campbell
K P
KP
D000818:Animals; D000911:Antibodies, Monoclonal; D002462:Cell Membrane / Q000032:analysis; D002845:Chromatography; D016189:Dystrophin; D005455:Fluorescent Antibody Technique; D006023:Glycoproteins / Q000032:analysis / Q000172:deficiency* / Q000378:metabolism; D006801:Humans; D007163:Immunosorbent Techniques; D046911:Macromolecular Substances; D051379:Mice; D008817:Mice, Mutant Strains; D008970:Molecular Weight; D009124:Muscle Proteins / Q000032:analysis / Q000378:metabolism*; D009132:Muscles / Q000032:analysis; D009136:Muscular Dystrophies / Q000378:metabolism*; D009137:Muscular Dystrophy, Animal / Q000378:metabolism; D011817:Rabbits; D012508:Sarcolemma / Q000032:analysis
D000911:Antibodies, Monoclonal; D016189:Dystrophin; D006023:Glycoproteins; D046911:Macromolecular Substances; D009124:Muscle Proteins