|
|
|
|
|
|
|
|
|||||
| Home Help Feedback Subscriptions Archive Search Table of Contents | |||||
Journal of Cell Science, Vol 103, Issue 4 1223-1233, Copyright © 1992 by Company of Biologists
JOURNAL ARTICLES |
G Dickson, A Azad, GE Morris, H Simon, M Noursadeghi and FS Walsh
Department of Experimental Pathology, UMDS, Guy's Hospital, London Bridge, UK.
In Duchenne muscular dystrophy (DMD), deficiency of the protein dystrophin results in necrosis of muscle myofibres, associated with lesions in the sarcolemma and surrounding basal lamina. Dystrophin has been proposed to be a major component of the sub-sarcolemmal cytoskeleton involved in maintaining the integrity of the myofibre plasma membrane, and is known to associate with a group of sarcolemmal glycoproteins, one of which exhibits high affinity binding to the basal lamina component laminin. However, a direct or indirect transmembrane association of dystrophin in muscle cells with the myofibre basal lamina has not been demonstrated. To address this question we have examined dystrophin immunostaining and immunoprecipitation patterns in cultured mouse and human myotubes in comparison with that of the basal lamina component, laminin. Dual-immunolabelling revealed virtually complete co-localization of dystrophin on the inside surface of the muscle cell sarcolemma with plaques and veined arrays of laminin accumulating on the extracellular face. This pattern of laminin and dystrophin distribution was distinct from that of other cell surface molecules expressed in myotubes such as the neural cell adhesion molecule, NCAM, and the beta 1 integrin receptor, and immunoprecipitation of dystrophin from solubilized myotube extracts resulted in co-purification of laminin B1 chain confirming an association between these two components. The results thus provide the first direct cellular evidence of a transmembrane linkage between dystrophin in the sarcolemmal cytoskeleton with laminin in the overlying basal lamina. While the immunocytochemical distribution of laminin was apparently normal in dystrophin-deficient muscle cells, elevated levels of soluble laminin were present in extracts of mdx compared with normal mouse skeletal muscle.(ABSTRACT TRUNCATED AT 250 WORDS)
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati 
Twitter What's this?
This article has been cited by other articles:
|
|
 |
|
  P. Lafuste, C. Sonnet, B. Chazaud, P. A. Dreyfus, R. K. Gherardi, U. M. Wewer, and F.-J. Authier ADAM12 and {alpha}9{beta}1 Integrin Are Instrumental in Human Myogenic Cell Differentiation Mol. Biol. Cell, February 1, 2005; 16(2): 861 - 870. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Nawrotzki, M. Willem, N. Miosge, H. Brinkmeier, and U. Mayer Defective integrin switch and matrix composition at alpha 7-deficient myotendinous junctions precede the onset of muscular dystrophy in mice Hum. Mol. Genet., March 1, 2003; 12(5): 483 - 495. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Jacobson, P. D. Cote, S. G. Rossi, R. L. Rotundo, and S. Carbonetto The Dystroglycan Complex Is Necessary for Stabilization of Acetylcholine Receptor Clusters at Neuromuscular Junctions and Formation of the Synaptic Basement Membrane J. Cell Biol., February 5, 2001; 152(3): 435 - 450. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Montanaro, M. Lindenbaum, and S. Carbonetto {alpha}-Dystroglycan Is a Laminin Receptor Involved in Extracellular Matrix Assembly on Myotubes and Muscle Cell Viability J. Cell Biol., June 14, 1999; 145(6): 1325 - 1340. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Xu and M. M. Salpeter Acetylcholine Receptors in Innervated Muscles of Dystrophic mdx Mice Degrade as after Denervation J. Neurosci., November 1, 1997; 17(21): 8194 - 8200. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. D. Root In situ molecular association of dystrophin with actin revealed by sensitized emission immuno-resonance energy transfer PNAS, May 27, 1997; 94(11): 5685 - 5690. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. E. Deconinck, A. C. Potter, J. M. Tinsley, S. J. Wood, R. Vater, C. Young, L. Metzinger, A. Vincent, C. R. Slater, and K. E. Davies Postsynaptic Abnormalities at the Neuromuscular Junctions of Utrophin-deficient Mice J. Cell Biol., February 24, 1997; 136(4): 883 - 894. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. R. Smalheiser and E. Kim Purification of Cranin, a Laminin Binding Membrane Protein J. Biol. Chem., June 23, 1995; 270(25): 15425 - 15433. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Khurana, L. Kunkel, A. Frederickson, S Carbonetto, and S. Watkins Interaction of chromosome-6-encoded dystrophin related protein with the extracellular matrix J. Cell Sci., January 1, 1995; 108(1): 173 - 185. [Abstract] [PDF]
|
|
|
|
|
|
M. Dunckley, K. Wells, T. Piper, D. Wells, and G Dickson Independent localization of dystrophin N- and C-terminal regions to the sarcolemma of mdx mouse myofibres in vivo J. Cell Sci., January 6, 1994; 107(6): 1469 - 1475. [Abstract] [PDF]
|
|
|
|
 |
|
 E. P. Hoffman and J. Wang Duchenne-Becker Muscular Dystrophy and the Nondystrophic Myotonias: Paradigms for Loss of Function and Change of Function of Gene Products Arch Neurol, November 1, 1993; 50(11): 1227 - 1237. [Abstract] [PDF]
|
|
