Journal article
The role of the dystrophin-glycoprotein complex in the molecular pathogenesis of muscular dystrophies
Neuromuscular disorders : NMD, Vol.3(5), pp.533-535
1993
DOI: 10.1016/0960-8966(93)90110-6
PMID: 8186706
Abstract
The dystrophin-glycoprotein complex is considered to be a major trans-sarcolemmal structure which provides a linkage between the subsarcolemmal actin cytoskeleton and the extracellular matrix component laminin. Recently, deficiency of the dystrophin-associated proteins has been shown to play an important role in the molecular pathogenesis of several forms of muscular dystrophy. These include Duchenne muscular dystrophy (DMD), symptomatic DMD carriers, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy with DMD-like phenotype prevalent in North Africa. In Fukuyama-type congenital muscular dystrophy (FCMD), the finding of abnormal expression of the dystrophin-associated proteins may provide a clue to its molecular pathogenesis. These recent findings indicate that the linkage between the subsarcolemmal cytoskeleton and extracellular matrix via the dystrophin-glycoprotein complex is critical for maintaining the integrity of muscle cell function.
Details
- Title: Subtitle
- The role of the dystrophin-glycoprotein complex in the molecular pathogenesis of muscular dystrophies
- Creators
- K Matsumura - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, U.S.AKay Ohlendieck - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, U.S.AVictor V Ionasescu - Department of Pediatrics, University of Iowa College of Medicine, Iowa City, IA 52242, U.S.AFernando M.S Tomé - INSERM U.153, Paris 75005, FranceIkuya Nonaka - NCNP, Tokyo, JapanArthur H.M Burghes - Department of Neurology, Ohio State University, Columbus, Ohio, U.S.AMarina Mora - Instituto Nazionale Neurologico ‘Carlo Besta’, Milan, ItalyJean-Claude Kaplan - INSERM U.129, Institut Cochin de Génétique Moléculaire, Paris, FranceMichel Fardeau - INSERM U.153, Paris 75005, FranceKevin P Campbell - Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, U.S.A
- Resource Type
- Journal article
- Publication Details
- Neuromuscular disorders : NMD, Vol.3(5), pp.533-535
- Publisher
- Elsevier B.V
- DOI
- 10.1016/0960-8966(93)90110-6
- PMID
- 8186706
- ISSN
- 0960-8966
- eISSN
- 1873-2364
- Language
- English
- Date published
- 1993
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984068268802771
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