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Physiol. Rev. 82: 291-329, 2002;
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Physiological Reviews, Vol. 82, No. 2, April 2002, pp. 291-329; 10.1152/physrev.00028.2001.
Copyright ©2002 by the American Physiological Society

Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle

Derek J. Blake, Andrew Weir, Sarah E. Newey, and Kay E. Davies

Medical Research Council, Functional Genetics Unit, Department of Human Anatomy and Genetics, University of Oxford, Oxford, United Kingdom

Blake, Derek J., Andrew Weir, Sarah E. Newey, and Kay E. Davies. Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle. Physiol. Rev. 82: 291-329, 2002.The X-linked muscle-wasting disease Duchenne muscular dystrophy is caused by mutations in the gene encoding dystrophin. There is currently no effective treatment for the disease; however, the complex molecular pathology of this disorder is now being unravelled. Dystrophin is located at the muscle sarcolemma in a membrane-spanning protein complex that connects the cytoskeleton to the basal lamina. Mutations in many components of the dystrophin protein complex cause other forms of autosomally inherited muscular dystrophy, indicating the importance of this complex in normal muscle function. Although the precise function of dystrophin is unknown, the lack of protein causes membrane destabilization and the activation of multiple pathophysiological processes, many of which converge on alterations in intracellular calcium handling. Dystrophin is also the prototype of a family of dystrophin-related proteins, many of which are found in muscle. This family includes utrophin and alpha -dystrobrevin, which are involved in the maintenance of the neuromuscular junction architecture and in muscle homeostasis. New insights into the pathophysiology of dystrophic muscle, the identification of compensating proteins, and the discovery of new binding partners are paving the way for novel therapeutic strategies to treat this fatal muscle disease. This review discusses the role of the dystrophin complex and protein family in muscle and describes the physiological processes that are affected in Duchenne muscular dystrophy.




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Y. Yue, Z. Li, S. Q. Harper, R. L. Davisson, J. S. Chamberlain, and D. Duan
Microdystrophin Gene Therapy of Cardiomyopathy Restores Dystrophin-Glycoprotein Complex and Improves Sarcolemma Integrity in the Mdx Mouse Heart
Circulation, September 30, 2003; 108(13): 1626 - 1632.
[Abstract] [Full Text] [PDF]

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 NEJMHome page
W. Burke
Genomics as a Probe for Disease Biology
N. Engl. J. Med., September 4, 2003; 349(10): 969 - 974.
[Full Text] [PDF]

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 Am. J. Physiol. Cell Physiol.Home page
D. R. Plant and G. S. Lynch
Depolarization-induced contraction and SR function in mechanically skinned muscle fibers from dystrophic mdx mice
Am J Physiol Cell Physiol, September 1, 2003; 285(3): C522 - C528.
[Abstract] [Full Text] [PDF]

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 Hum Mol GenetHome page
J. D. Porter, A. P. Merriam, P. Leahy, B. Gong, and S. Khanna
Dissection of temporal gene expression signatures of affected and spared muscle groups in dystrophin-deficient (mdx) mice
Hum. Mol. Genet., August 1, 2003; 12(15): 1813 - 1821.
[Abstract] [Full Text] [PDF]

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 GlycobiologyHome page
P. T. Martin
Dystroglycan glycosylation and its role in matrix binding in skeletal muscle
Glycobiology, August 1, 2003; 13(8): 55R - 66R.
[Abstract] [Full Text] [PDF]

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 J. Neurosci.Home page
R. V. Sillitoe, M. A. Benson, D. J. Blake, and R. Hawkes
Abnormal Dysbindin Expression in Cerebellar Mossy Fiber Synapses in the mdx Mouse Model of Duchenne Muscular Dystrophy
J. Neurosci., July 23, 2003; 23(16): 6576 - 6585.
[Abstract] [Full Text] [PDF]

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 Proc. Natl. Acad. Sci. USAHome page
J. V. Chakkalakal, M. A. Stocksley, M.-A. Harrison, L. M. Angus, J. Deschenes-Furry, S. St-Pierre, L. A. Megeney, E. R. Chin, R. N. Michel, and B. J. Jasmin
Expression of utrophin A mRNA correlates with the oxidative capacity of skeletal muscle fiber types and is regulated by calcineurin/NFAT signaling
PNAS, June 24, 2003; 100(13): 7791 - 7796.
[Abstract] [Full Text] [PDF]

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 Hum Mol GenetHome page
R. Gilbert, R. W. R. Dudley, A.-B. Liu, B. J. Petrof, J. Nalbantoglu, and G. Karpati
Prolonged dystrophin expression and functional correction of mdx mouse muscle following gene transfer with a helper-dependent (gutted) adenovirus-encoding murine dystrophin
Hum. Mol. Genet., June 1, 2003; 12(11): 1287 - 1299.
[Abstract] [Full Text] [PDF]

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 J. Cell Sci.Home page
S. W. Oh, R. K. Pope, K. P. Smith, J. L. Crowley, T. Nebl, J. B. Lawrence, and E. J. Luna
Archvillin, a muscle-specific isoform of supervillin, is an early expressed component of the costameric membrane skeleton
J. Cell Sci., June 1, 2003; 116(11): 2261 - 2275.
[Abstract] [Full Text] [PDF]

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 J. Appl. Physiol.Home page
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]

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 IOVSHome page
S. Khanna, C. R. Richmonds, H. J. Kaminski, and J. D. Porter
Molecular Organization of the Extraocular Muscle Neuromuscular Junction: Partial Conservation of and Divergence from the Skeletal Muscle Prototype
Invest. Ophthalmol. Vis. Sci., May 1, 2003; 44(5): 1918 - 1926.
[Abstract] [Full Text] [PDF]

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 J. Biol. Chem.Home page
J. M. Ervasti
Costameres: the Achilles' Heel of Herculean Muscle
J. Biol. Chem., April 11, 2003; 278(16): 13591 - 13594.
[Full Text] [PDF]

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 J. Biol. Chem.Home page
K. P. Campbell and J. T. Stull
Skeletal Muscle Basement Membrane-Sarcolemma-Cytoskeleton Interaction Minireview Series
J. Biol. Chem., April 4, 2003; 278(15): 12599 - 12600.
[Full Text] [PDF]

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 J. Biol. Chem.Home page
J. R. Sanes
The Basement Membrane/Basal Lamina of Skeletal Muscle
J. Biol. Chem., April 4, 2003; 278(15): 12601 - 12604.
[Full Text] [PDF]

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 JCBHome page
R. M. Grady, M. Akaaboune, A. L. Cohen, M. M. Maimone, J. W. Lichtman, and J. R. Sanes
Tyrosine-phosphorylated and nonphosphorylated isoforms of {alpha}-dystrobrevin: roles in skeletal muscle and its neuromuscular and myotendinous junctions
J. Cell Biol., March 3, 2003; 160(5): 741 - 752.
[Abstract] [Full Text] [PDF]

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 Hum Mol GenetHome page
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]

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 JCBHome page
P. Bagnato, V. Barone, E. Giacomello, D. Rossi, and V. Sorrentino
Binding of an ankyrin-1 isoform to obscurin suggests a molecular link between the sarcoplasmic reticulum and myofibrils in striated muscles
J. Cell Biol., January 21, 2003; 160(2): 245 - 253.
[Abstract] [Full Text] [PDF]

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 J. Neurosci.Home page
J. Kahl and J. T. Campanelli
A Role for the Juxtamembrane Domain of beta -Dystroglycan in Agrin-Induced Acetylcholine Receptor Clustering
J. Neurosci., January 15, 2003; 23(2): 392 - 402.
[Abstract] [Full Text] [PDF]

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 J. Biol. Chem.Home page
Y. Ou, P. Strege, S. M. Miller, J. Makielski, M. Ackerman, S. J. Gibbons, and G. Farrugia
Syntrophin gamma 2 Regulates SCN5A Gating by a PDZ Domain-mediated Interaction
J. Biol. Chem., January 10, 2003; 278(3): 1915 - 1923.
[Abstract] [Full Text] [PDF]

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 J. Pharmacol. Exp. Ther.Home page
A. De Luca, S. Pierno, A. Liantonio, M. Cetrone, C. Camerino, B. Fraysse, M. Mirabella, S. Servidei, U. T. Ruegg, and D. Conte Camerino
Enhanced Dystrophic Progression in mdx Mice by Exercise and Beneficial Effects of Taurine and Insulin-Like Growth Factor-1
J. Pharmacol. Exp. Ther., January 1, 2003; 304(1): 453 - 463.
[Abstract] [Full Text] [PDF]

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 Hum Mol GenetHome page
C. T. Esapa, M. A. Benson, J. E. Schroder, E. Martin-Rendon, M. Brockington, S. C. Brown, F. Muntoni, S. Kroger, and D. J. Blake
Functional requirements for fukutin-related protein in the Golgi apparatus
Hum. Mol. Genet., December 15, 2002; 11(26): 3319 - 3331.
[Abstract] [Full Text] [PDF]

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 Hum Mol GenetHome page
S. Squire, J.M. Raymackers, C. Vandebrouck, A. Potter, J. Tinsley, R. Fisher, J.M. Gillis, and K.E. Davies
Prevention of pathology in mdx mice by expression of utrophin: analysis using an inducible transgenic expression system
Hum. Mol. Genet., December 15, 2002; 11(26): 3333 - 3344.
[Abstract] [Full Text] [PDF]

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 J. Biol. Chem.Home page
R. C. Austin, J. E. B. Fox, G. H. Werstuck, A. R. Stafford, D. E. Bulman, G. Y. Dally, C. A. Ackerley, J. I. Weitz, and P. N. Ray
Identification of Dp71 Isoforms in the Platelet Membrane Cytoskeleton. POTENTIAL ROLE IN THROMBIN-MEDIATED PLATELET ADHESION
J. Biol. Chem., November 27, 2002; 277(49): 47106 - 47113.
[Abstract] [Full Text] [PDF]

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 J. Biol. Chem.Home page
A. P. Weir, E. A. Burton, G. Harrod, and K. E. Davies
A- and B-utrophin Have Different Expression Patterns and Are Differentially Up-regulated in mdx Muscle
J. Biol. Chem., November 15, 2002; 277(47): 45285 - 45290.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. S. Chamberlain
Gene therapy of muscular dystrophy
Hum. Mol. Genet., October 1, 2002; 11(20): 2355 - 2362.
[Abstract] [Full Text] [PDF]


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