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Human dystroglycan: skeletal muscle cDNA, genomic structure, origin of tissue specific isoforms and chromosomal localization.
Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City 52242.
Dystroglycan is a novel laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. Here we report the cDNA and genomic structure of human dystroglycan. The human dystroglycan is encoded by a single gene (DAG1) mapped to chromosome 3 band p21. The coding sequence is organized into two exons, separated by a large intron. The predicted amino acid sequence of human and rabbit dystroglycan are 93% identical with predicted glycosylation sites being conserved. Human dystroglycan is expressed in a variety of fetal and adult tissues. Our data suggest that muscle and non-muscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence. Therefore, we hypothesize that variable glycosylation of the conserved protein core might modulate laminin binding. The relationship of dystroglycan to human diseases is discussed.
PMID: 8268918 [PubMed - indexed for MEDLINE]
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Cited by 19 PubMed Central articles
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Muscle dystroglycan organizes the postsynapse and regulates presynaptic neurotransmitter release at the Drosophila neuromuscular junction.
Bogdanik L, Framery B, Frölich A, Franco B, Mornet D, Bockaert J, Sigrist SJ, Grau Y, Parmentier ML.
PLoS One. 2008 Apr 30; 3(4):e2084. Epub 2008 Apr 30.
[PLoS One. 2008]
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Duplication of the dystroglycan gene in most branches of teleost fish.
Pavoni E, Cacchiarelli D, Tittarelli R, Orsini M, Galtieri A, Giardina B, Brancaccio A.
BMC Mol Biol. 2007 May 17; 8:34. Epub 2007 May 17.
[BMC Mol Biol. 2007]
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O Mannosylation of alpha-dystroglycan is essential for lymphocytic choriomeningitis virus receptor function.
Imperiali M, Thoma C, Pavoni E, Brancaccio A, Callewaert N, Oxenius A.
J Virol. 2005 Nov; 79(22):14297-308.
[J Virol. 2005]
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