|
|
|
|
|
||
|
|||||
| Home Help Feedback Subscriptions Archive Search Table of Contents | |||||
Journal of Cell Science, Vol 107, Issue 6 1477-1483, Copyright © 1994 by Company of Biologists
JOURNAL ARTICLES |
DJ Law, DL Allen and JG Tidball
Department of Physiological Science, University of California, Los Angeles 90024-1527.
Duchenne muscular dystrophy (DMD) and the myopathy seen in the mdx mouse both result from absence of the protein dystrophin. Structural similarities between dystrophin and other cytoskeletal proteins, its enrichment at myotendinous junctions, and its indirect association with laminin mediated by a transmembrane glycoprotein complex suggest that one of dystrophin's functions in normal muscle is to form one of the links between the actin cytoskeleton and the extracellular matrix. Unlike Duchenne muscular dystrophy patients, mdx mice suffer only transient muscle necrosis, and are able to regenerate damaged muscle tissue. The present study tests the hypothesis that mdx mice partially compensate for dystrophin's absence by upregulating one or more dystrophin-independent mechanisms of cytoskeleton-membrane association. Quantitative analysis of immunoblots of adult mdx muscle samples showed an increase of approximately 200% for vinculin and talin, cytoskeletal proteins that mediate thin filament-membrane interactions at myotendinous junctions. Blots also showed an increase (143%) in the dystrophin-related protein called utrophin, another myotendinous junction constituent, which may be able to substitute for dystrophin directly. Muscle samples from 2-week-old animals, a period immediately preceding the onset of muscle necrosis, showed no significant differences in protein concentration between mdx and controls. Quantitative analyses of confocal images of myotendinous junctions from mdx and control muscles show significantly higher concentrations of talin and vinculin at the myotendinous junctions of mdx muscle. These findings indicate that mdx mice may compensate in part for the absence of dystrophin by increased expression of other molecules that subsume dystrophin's mechanical function.
This article has been cited by other articles:
|
|
 |
|
  D. G. Cerri, L. C. Rodrigues, S. R. Stowell, D. D. Araujo, M. C. Coelho, S. R. Oliveira, J. C. S. Bizario, R. D. Cummings, M. Dias-Baruffi, and M. C. R. Costa Degeneration of dystrophic or injured skeletal muscles induces high expression of Galectin-1 Glycobiology, November 1, 2008; 18(11): 842 - 850. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Hijikata, A. Nakamura, K. Isokawa, M. Imamura, K. Yuasa, R. Ishikawa, K. Kohama, S. Takeda, and H. Yorifuji Plectin 1 links intermediate filaments to costameric sarcolemma through {beta}-synemin, {alpha}-dystrobrevin and actin J. Cell Sci., June 15, 2008; 121(12): 2062 - 2074. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Liu, D. J. Burkin, and S. J. Kaufman Increasing {alpha}7{beta}1-integrin promotes muscle cell proliferation, adhesion, and resistance to apoptosis without changing gene expression Am J Physiol Cell Physiol, February 1, 2008; 294(2): C627 - C640. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. E. Rooney, J. V. Welser, M. A. Dechert, N. L. Flintoff-Dye, S. J. Kaufman, and D. J. Burkin Severe muscular dystrophy in mice that lack dystrophin and {alpha}7 integrin J. Cell Sci., June 1, 2006; 119(11): 2185 - 2195. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. M. Hanft, I. N. Rybakova, J. R. Patel, J. A. Rafael-Fortney, and J. M. Ervasti Cytoplasmic {gamma}-actin contributes to a compensatory remodeling response in dystrophin-deficient muscle PNAS, April 4, 2006; 103(14): 5385 - 5390. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. KUMAR, N. KHANDELWAL, R. MALYA, M. B. REID, and A. M. BORIEK Loss of dystrophin causes aberrant mechanotransduction in skeletal muscle fibers FASEB J, January 1, 2004; 18(1): 102 - 113. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. I. Bassett and P. D. Currie The zebrafish as a model for muscular dystrophy and congenital myopathy Hum. Mol. Genet., October 15, 2003; 12(90002): R265 - 270. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. Galvagni, M. Cantini, and S. Oliviero The Utrophin Gene Is Transcriptionally Up-regulated in Regenerating Muscle J. Biol. Chem., May 17, 2002; 277(21): 19106 - 19113. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. A. Rice, O. W. Nadeau, Q. Yang, and G. M. Carlson The Calmodulin-binding Domain of the Catalytic gamma Subunit of Phosphorylase Kinase Interacts with Its Inhibitory alpha Subunit. EVIDENCE FOR A Ca2+-SENSITIVE NETWORK OF QUATERNARY INTERACTIONS J. Biol. Chem., April 19, 2002; 277(17): 14681 - 14687. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. J. Koh and S. V. Brooks Lengthening contractions are not required to induce protection from contraction-induced muscle injury Am J Physiol Regulatory Integrative Comp Physiol, July 1, 2001; 281(1): R155 - R161. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. J. Burkin, G. Q. Wallace, K. J. Nicol, D. J. Kaufman, and S. J. Kaufman Enhanced Expression of the {alpha}7{beta}1 Integrin Reduces Muscular Dystrophy and Restores Viability in Dystrophic Mice J. Cell Biol., March 19, 2001; 152(6): 1207 - 1218. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 V. Rider, S. R. Jones, R. T. Foster, and K. Imakawa Changes in the Temporal and Spatial Expression of H{beta}58 During Formation and Maturation of the Chorioallantoic Placenta in the Rat Biol Reprod, December 1, 2000; 63(6): 1735 - 1746. [Abstract] [Full Text]
|
|
|
|
|
|
J. Wilson, W. Putt, C. Jimenez, and Y. H. Edwards Up71 and Up140, two novel transcripts of utrophin that are homologues of short forms of dystrophin Hum. Mol. Genet., July 1, 1999; 8(7): 1271 - 1278. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Porter, J. Rafael, R. Ragusa, J. Brueckner, J. Trickett, and K. Davies The sparing of extraocular muscle in dystrophinopathy is lost in mice lacking utrophin and dystrophin J. Cell Sci., January 7, 1998; 111(13): 1801 - 1811. [Abstract] [PDF]
|
|
|
|
|
|
B. Hodges, Y. Hayashi, I Nonaka, W Wang, K Arahata, and S. Kaufman Altered expression of the alpha7beta1 integrin in human and murine muscular dystrophies J. Cell Sci., January 11, 1997; 110(22): 2873 - 2881. [Abstract] [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]
|
|
