|
|
|
|
|
||
|
|||||
| Home Help Feedback Subscriptions Archive Search Table of Contents | |||||
Journal of Cell Science, Vol 112, Issue 2 209-216, Copyright © 1999 by Company of Biologists
JOURNAL ARTICLES |
SC Brown, A Fassati, L Popplewell, AM Page, MD Henry, KP Campbell and G Dickson
Division of Biochemistry, School of Biological Sciences, Royal Holloway College, University of London, Egham, Surrey TW20 0EX, UK.
alpha-dystroglycan is a glycoprotein expressed on the surface of skeletal muscle fibres and other cell types. In muscle, alpha-dystroglycan provides a link between the myofibre cytoskeleton through its indirect binding to dystrophin, and the basal lamina through its binding to laminin-2, a protein of the extracellular matrix. The disruption of this linkage between the myofibre cytoskeleton and the extracellular matrix is a common feature of Duchenne and other muscular dystrophies, though the pathogenic mechanisms leading to muscle wasting remain unknown. By treating primary mouse muscle cultures with a monoclonal antibody which blocks alpha-dystroglycan binding to laminin, we show here the induction of a dystrophic phenotype in vitro. The phenotype is inducible in differentiated cultures only, is characterised by reduced myotube size, myofibril disorganisation, loss of contractile activity, reduced spontaneous clustering of acetylcholine receptors and is reversed by addition of excess exogenous laminin-2. Thus, alpha-dystroglycan may be part of a signalling pathway for the maturation and maintenance of skeletal myofibres. Detailed knowledge of this signalling pathway may provide insights into the molecular pathology of the various inherited muscular dystrophies, and identify valuable pharmacological targets and new therapeutic strategies.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati 
Twitter What's this?
This article has been cited by other articles:
|
|
 |
|
  A. K. Peter, G. Miller, and R. H. Crosbie Disrupted mechanical stability of the dystrophin-glycoprotein complex causes severe muscular dystrophy in sarcospan transgenic mice J. Cell Sci., March 15, 2007; 120(6): 996 - 1008. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Driss, L. Charrier, Y. Yan, V. Nduati, S. Sitaraman, and D. Merlin Dystroglycan receptor is involved in integrin activation in intestinal epithelia Am J Physiol Gastrointest Liver Physiol, June 1, 2006; 290(6): G1228 - G1242. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. K. Patnaik and P. Stanley Mouse Large Can Modify Complex N- and Mucin O-Glycans on {alpha}-Dystroglycan to Induce Laminin Binding J. Biol. Chem., May 27, 2005; 280(21): 20851 - 20859. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. C. Brown, S. Torelli, M. Brockington, Y. Yuva, C. Jimenez, L. Feng, L. Anderson, I. Ugo, S. Kroger, K. Bushby, et al. Abnormalities in {alpha}-Dystroglycan Expression in MDC1C and LGMD2I Muscular Dystrophies Am. J. Pathol., February 1, 2004; 164(2): 727 - 737. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. K. Grewal and J. E. Hewitt Glycosylation defects: a new mechanism for muscular dystrophy? Hum. Mol. Genet., October 15, 2003; 12(90002): R259 - 264. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. J. Parsons, I. Campos, E. M. A. Hirst, and D. L. Stemple Removal of dystroglycan causes severe muscular dystrophy in zebrafish embryos Development, March 9, 2003; 129(14): 3505 - 3512. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. S. Chockalingam, R. Cholera, S. A. Oak, Y. Zheng, H. W. Jarrett, and D. B. Thomason Dystrophin-glycoprotein complex and Ras and Rho GTPase signaling are altered in muscle atrophy Am J Physiol Cell Physiol, August 1, 2002; 283(2): C500 - C511. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. L. Roberts, D. J. Wells, I. R. Graham, S. A. Fabb, V. J. Hill, G. Duisit, K. Yuasa, S.'i. Takeda, F.-L. Cosset, and G. Dickson Stable micro-dystrophin gene transfer using an integrating adeno-retroviral hybrid vector ameliorates the dystrophic pathology in mdx mouse muscle Hum. Mol. Genet., July 15, 2002; 11(15): 1719 - 1730. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y.-W. Chen, P. Zhao, R. Borup, and E. P. Hoffman Expression Profiling in the Muscular Dystrophies: Identification of Novel Aspects of Molecular Pathophysiology J. Cell Biol., December 11, 2000; 151(6): 1321 - 1336. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
V. Allamand and K. P. Campbell Animal models for muscular dystrophy: valuable tools for the development of therapies Hum. Mol. Genet., October 1, 2000; 9(16): 2459 - 2467. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Meier and M. A. Ruegg The Role of Dystroglycan and Its Ligands in Physiology and Disease Physiology, October 1, 2000; 15(5): 255 - 259. [Abstract] [Full Text] [PDF]
|
|
