Journal article
Dystroglycan: from biosynthesis to pathogenesis of human disease
Journal of cell science, Vol.119(Pt 2), pp.199-207
01/15/2006
DOI: 10.1242/jcs.02814
PMID: 16410545
Abstract
Alpha- and beta-dystroglycan constitute a membrane-spanning complex that connects the extracellular matrix to the cytoskeleton. Although a structural role for dystroglycan had been identified, biochemical and genetic discoveries have recently highlighted the significance of posttranslational processing for dystroglycan function. Glycosylation is the crucial modification that modulates the function of dystroglycan as a receptor for extracellular binding partners. It has become clear that perturbation of dystroglycan glycosylation is the central event in the pathogenesis of several complex disorders, and recent advances suggest that glycosylation could be modulated to ameliorate the pathological features. Our increased understanding of the mechanisms of interaction of dystroglycan with its ligands has become an essential tool in deciphering the biological processes related to the human diseases in which the proteins are implicated.
Details
- Title: Subtitle
- Dystroglycan: from biosynthesis to pathogenesis of human disease
- Creators
- Rita Barresi - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USAKevin P Campbell
- Resource Type
- Journal article
- Publication Details
- Journal of cell science, Vol.119(Pt 2), pp.199-207
- DOI
- 10.1242/jcs.02814
- PMID
- 16410545
- NLM abbreviation
- J Cell Sci
- ISSN
- 0021-9533
- eISSN
- 1477-9137
- Publisher
- England
- Language
- English
- Date published
- 01/15/2006
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984068383902771
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