Deficiency of a Dystrophin-Associated Glycoprotein (Adhalin) in a Patient with Muscular Dystrophy and Cardiomyopathy
Ricardo Fadic, M.D., Yoshihida Sunada, Ph.D., Andrew J. Waclawik, M.D., Scott Buck, M.D., Paul J. Lewandoski, Kevin P. Campbell, Ph.D., and Barend P. Lotz, M.D.
Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.
Cardiac muscle is commonly affected in muscular dystrophies.1,2,3,4X-linked Duchenne's muscular dystrophy and Becker's musculardystrophy are caused by mutations in the gene encoding dystrophin,5,6a membrane cytoskeletal protein.7 In skeletal and cardiac muscle,dystrophin is associated with a large oligomeric complex ofsarcolemmal glycoproteins.8,9 This dystrophinglycoproteincomplex is composed of five glycoproteins (35, 43 doublet, 50,and 156 kd) and two proteins (25 and 59 kd). It spans the sarcolemmato provide linkage between the subsarcolemmal cytoskeleton andlaminin, a major component of the extracellular matrix.9,10
In Duchenne's and Becker's muscular dystrophies, the primarydeficiency in dystrophin is associated . . . [Full Text of this Article]
Case Report
Methods
Muscle-Biopsy Specimens
Antibodies
Results
Discussion
Source Information
From the Departments of Neurology (R.F., A.J.W., P.J.L., B.P.L.) and Pediatrics (S.B.), University of Wisconsin, Madison, and the Howard Hughes Medical Institute and the Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City (Y.S., K.P.C.).
Address reprint requests to Dr. Lotz at the Department of Neurology, University of Wisconsin Hospital and Clinics, 600 Highland Ave., Madison, WI 53792.
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