| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
1 Department of Pediatrics, Magee-Women's Research Institute, Duchenne Muscular Dystrophy Research Center, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213; and 2 Research Center for Genetic Medicine, Children's National Medical Center, Duchenne Muscular Dystrophy Research Center, Washington, DC 20010
Muscular dystrophies are a
clinically and genetically heterogeneous group of disorders that
show myofiber degeneration and regeneration. Identification of animal
models of muscular dystrophy has been instrumental in research on the
pathogenesis, pathophysiology, and treatment of these disorders. We
review our understanding of the functional status of dystrophic
skeletal muscle from selected animal models with a focus on
1) the mdx mouse model of Duchenne muscular
dystrophy, 2) the Bio 14.6 
-sarcoglycan-deficient hamster model of limb-girdle muscular dystrophy, and 3) transgenic
null mutant murine lines of sarcoglycan (
, 
, , and 
)
deficiencies. Although biochemical data from these models suggest that
the dystrophin-sarcoglycan-dystroglycan-laminin network is critical for
structural integrity of the myofiber plasma membrane, emerging studies
of muscle physiology suggest a more complex picture, with specific
functional deficits varying considerably from muscle to muscle and
model to model. It is likely that changes in muscle structure and
function, downstream of the specific, primary biochemical deficiency,
may alter muscle contractile properties.
dystrophin; mdx mouse; sarcoglycanopathies
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]
|
|
|
|
 |
|
 M. DiFranco, C. E. Woods, J. Capote, and J. L. Vergara Dystrophic skeletal muscle fibers display alterations at the level of calcium microdomains PNAS, September 23, 2008; 105(38): 14698 - 14703. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Friedrich, C. Weber, F. v. Wegner, J. S. Chamberlain, and R. H. A. Fink Unloaded Speed of Shortening in Voltage-Clamped Intact Skeletal Muscle Fibers from wt, mdx, and Transgenic Minidystrophin Mice Using a Novel High-Speed Acquisition System Biophys. J., June 15, 2008; 94(12): 4751 - 4765. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. S. Lynch and J. G. Ryall Role of {beta}-Adrenoceptor Signaling in Skeletal Muscle: Implications for Muscle Wasting and Disease Physiol Rev, April 1, 2008; 88(2): 729 - 767. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. D'Antona, L. Brocca, O. Pansarasa, C. Rinaldi, R. Tupler, and R. Bottinelli Structural and functional alterations of muscle fibres in the novel mouse model of facioscapulohumeral muscular dystrophy J. Physiol., November 1, 2007; 584(3): 997 - 1009. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. De Mori, S. Straino, A. Di Carlo, A. Mangoni, G. Pompilio, R. Palumbo, M. E. Bianchi, M. C. Capogrossi, and A. Germani Multiple Effects of High Mobility Group Box Protein 1 in Skeletal Muscle Regeneration Arterioscler. Thromb. Vasc. Biol., November 1, 2007; 27(11): 2377 - 2383. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Hibbitt, K. Coward, H. Kubota, N. Prathalingham, W. Holt, K. Kohri, and J. Parrington In Vivo Gene Transfer by Electroporation Allows Expression of a Fluorescent Transgene in Hamster Testis and Epididymal Sperm and Has No Adverse Effects upon Testicular Integrity or Sperm Quality Biol Reprod, January 1, 2006; 74(1): 95 - 101. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. E Woods, D. Novo, M. DiFranco, J. Capote, and J. L Vergara Propagation in the transverse tubular system and voltage dependence of calcium release in normal and mdx mouse muscle fibres J. Physiol., November 1, 2005; 568(3): 867 - 880. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Danieli-Betto, A. Esposito, E. Germinario, D. Sandona, T. Martinello, A. Jakubiec-Puka, D. Biral, and R. Betto Deficiency of {alpha}-sarcoglycan differently affects fast- and slow-twitch skeletal muscles Am J Physiol Regulatory Integrative Comp Physiol, November 1, 2005; 289(5): R1328 - R1337. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. A. Timmons, O. Larsson, E. Jansson, H. Fischer, T. Gustafsson, P. L. Greenhaff, J. Ridden, J. Rachman, M. Peyrard-Janvid, C. Wahlestedt, et al. Human muscle gene expression responses to endurance training provide a novel perspective on Duchenne muscular dystrophy FASEB J, May 1, 2005; 19(7): 750 - 760. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D.D.W. Cornelison, S. A. Wilcox-Adelman, P. F. Goetinck, H. Rauvala, A. C. Rapraeger, and B. B. Olwin Essential and separable roles for Syndecan-3 and Syndecan-4 in skeletal muscle development and regeneration Genes & Dev., September 15, 2004; 18(18): 2231 - 2236. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. E. Woods, D. Novo, M. DiFranco, and J. L. Vergara The action potential-evoked sarcoplasmic reticulum calcium release is impaired in mdx mouse muscle fibres J. Physiol., May 15, 2004; 557(1): 59 - 75. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. B. P. CHARGE and M. A. RUDNICKI Cellular and Molecular Regulation of Muscle Regeneration Physiol Rev, January 1, 2004; 84(1): 209 - 238. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. Moghadaszadeh, R. Albrechtsen, L. T. Guo, M. Zaik, N. Kawaguchi, R. H. Borup, P. Kronqvist, H. D. Schroder, K. E. Davies, T. Voit, et al. Compensation for dystrophin-deficiency: ADAM12 overexpression in skeletal muscle results in increased {alpha}7 integrin, utrophin and associated glycoproteins Hum. Mol. Genet., October 1, 2003; 12(19): 2467 - 2479. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Coirault, B. Pignol, R. N. Cooper, G. Butler-Browne, P.-E. Chabrier, and Y. Lecarpentier Severe muscle dysfunction precedes collagen tissue proliferation in mdx mouse diaphragm J Appl Physiol, May 1, 2003; 94(5): 1744 - 1750. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. R. Guyon, A. N. Mosley, Y. Zhou, K. F. O'Brien, X. Sheng, K. Chiang, A. J. Davidson, J. M. Volinski, L. I. Zon, and L. M. Kunkel The dystrophin associated protein complex in zebrafish Hum. Mol. Genet., March 15, 2003; 12(6): 601 - 615. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| Visit Other APS Journals Online |
