266761452016021620220321
1522-12109612016JanPhysiological reviewsPhysiol RevAbsence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy.253305253-30510.1152/physrev.00007.2015Dystrophin is a long rod-shaped protein that connects the subsarcolemmal cytoskeleton to a complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further connections via laminin to other extracellular matrix proteins. Initially considered a structural complex that protected the sarcolemma from mechanical damage, the DPC is now known to serve as a scaffold for numerous signaling proteins. Absence or reduced expression of dystrophin or many of the DPC components cause the muscular dystrophies, a group of inherited diseases in which repeated bouts of muscle damage lead to atrophy and fibrosis, and eventually muscle degeneration. The normal function of dystrophin is poorly defined. In its absence a complex series of changes occur with multiple muscle proteins showing reduced or increased expression or being modified in various ways. In this review, we will consider the various proteins whose expression and function is changed in muscular dystrophies, focusing on Ca(2+)-permeable channels, nitric oxide synthase, NADPH oxidase, and caveolins. Excessive Ca(2+) entry, increased membrane permeability, disordered caveolar function, and increased levels of reactive oxygen species are early changes in the disease, and the hypotheses for these phenomena will be critically considered. The aim of the review is to define the early damage pathways in muscular dystrophy which might be appropriate targets for therapy designed to minimize the muscle degeneration and slow the progression of the disease.Copyright © 2016 the American Physiological Society.AllenDavid GDGSydney Medical School & Bosch Institute, University of Sydney, New South Wales, Australia; and Department of Physiology & Biophysics, University of Washington, Seattle, Washington.WhiteheadNicholas PNPSydney Medical School & Bosch Institute, University of Sydney, New South Wales, Australia; and Department of Physiology & Biophysics, University of Washington, Seattle, Washington.FroehnerStanley CSCSydney Medical School & Bosch Institute, University of Sydney, New South Wales, Australia; and Department of Physiology & Biophysics, University of Washington, Seattle, Washington.engR01 NS33145NSNINDS NIH HHSUnited StatesP01 NS04678NSNINDS NIH HHSUnited StatesR01 NS033145NSNINDS NIH HHSUnited StatesR21 NS088691NSNINDS NIH HHSUnited StatesR01AR056221ARNIAMS NIH HHSUnited StatesJournal ArticleResearch Support, N.I.H., ExtramuralReview
United StatesPhysiol Rev02317140031-93330Dystrophin0Reactive Oxygen Species31C4KY9ESHNitric OxideSY7Q814VUPCalciumIMAnimalsCalciummetabolismCalcium SignalingDystrophindeficiencygeneticsmetabolismGene Expression RegulationHumansMuscle DevelopmentMuscle, SkeletalmetabolismpathologyphysiopathologyMuscular DystrophiesgeneticsmetabolismpathologyphysiopathologyNitric OxidemetabolismReactive Oxygen SpeciesmetabolismRegenerationSignal Transduction20151218602015121860201621860201711ppublish26676145PMC469839510.1152/physrev.00007.201596/1/253Aartsma-Rus A, Muntoni F. 194th ENMC International Workshop. 3rd ENMC workshop on exon skipping: towards clinical application of antisense-mediated exon skipping for Duchenne muscular dystrophy 8–10 December 2012, Naarden, The Netherlands. Neuromuscul Disord 23: 934–944, 2013.23890933Abramovici H, Gee SH. Morphological changes and spatial regulation of diacylglycerol kinase-zeta, syntrophins, and Rac1 during myoblast fusion. Cell Motil Cytoskeleton 64: 549–567, 2007.17410543Abramovici H, Hogan AB, Obagi C, Topham MK, Gee SH. Diacylglycerol kinase-zeta localization in skeletal muscle is regulated by phosphorylation and interaction with syntrophins. Mol Biol Cell 14: 4499–4511, 2003.PMC26676814551255Adamo CM, Dai DF, Percival JM, Minami E, Willis MS, Patrucco E, Froehner SC, Beavo JA. Sildenafil reverses cardiac dysfunction in the mdx mouse model of Duchenne muscular dystrophy. Proc Natl Acad Sci USA 107: 19079–19083, 2010.PMC297389420956307Adams ME, Butler MH, Dwyer TM, Peters MF, Murnane AA, Froehner SC. Two forms of mouse syntrophin, a 58 kd dystrophin-associated protein, differ in primary structure and tissue distribution. Neuron 11: 531–540, 1993.7691103Adams ME, Mueller HA, Froehner SC. In vivo requirement of the alpha-syntrophin PDZ domain for the sarcolemmal localization of nNOS and aquaporin-4. J Cell Biol 155: 113–122, 2001.PMC215078311571312Adams ME, Tesch Y, Percival JM, Albrecht DE, Conhaim JI, Anderson K, Froehner SC. Differential targeting of nNOS and AQP4 to dystrophin-deficient sarcolemma by membrane-directed alpha-dystrobrevin. J Cell Sci 121: 48–54, 2008.18057022Ahn AH, Freener CA, Gussoni E, Yoshida M, Ozawa E, Kunkel LM. The three human syntrophin genes are expressed in diverse tissues, have distinct chromosomal locations, and each bind to dystrophin and its relatives. J Biol Chem 271: 2724–2730, 1996.8576247Ahn AH, Kunkel LM. Syntrophin binds to an alternatively spliced exon of dystrophin. J Cell Biol 128: 363–371, 1995.PMC21203437844150Albrecht DE, Sherman DL, Brophy PJ, Froehner SC. The ABCA1 cholesterol transporter associates with one of two distinct dystrophin-based scaffolds in Schwann cells. Glia 56: 611–618, 2008.PMC433517018286648Alderton JM, Steinhardt RA. Calcium influx through calcium leak channels is responsible for the elevated levels of calcium-dependent proteolysis in dystrophic myotubes. J Biol Chem 275: 9452–9460, 2000.10734092Alessi A, Bragg AD, Percival JM, Yoo J, Albrecht DE, Froehner SC, Adams ME. γ-Syntrophin scaffolding is spatially and functionally distinct from that of the alpha/beta syntrophins. Exp Cell Res 312: 3084–3095, 2006.16857187Allen DG, Lamb GD, Westerblad H. Skeletal muscle fatigue: Cellular mechanisms. Physiol Rev 88: 287–332, 2008.18195089Altamirano F, Lopez JR, Henriquez C, Molinski T, Allen PD, Jaimovich E. Increased resting intracellular calcium modulates NF-kappaB-dependent inducible nitric-oxide synthase gene expression in dystrophic mdx skeletal myotubes. J Biol Chem 287: 20876–20887, 2012.PMC337551122549782Amann KJ, Renley BA, Ervasti JM. A cluster of basic repeats in the dystrophin rod domain binds F-actin through an electrostatic interaction. J Biol Chem 273: 28419–28423, 1998.9774469Amenta AR, Yilmaz A, Bogdanovich S, McKechnie BA, Abedi M, Khurana TS, Fallon JR. Biglycan recruits utrophin to the sacrolemma and counters dystrophic pathology in mdx mice. Proc Natl Acad Sci USA 108: 762–767, 2011.PMC302106821187385Amiry-Moghaddam M, Otsuka T, Hurn PD, Traystman RJ, Haug FM, Froehner SC, Adams ME, Neely JD, Agre P, Ottersen OP, Bhardwaj A. An alpha-syntrophin-dependent pool of AQP4 in astroglial end-feet confers bidirectional water flow between blood and brain. Proc Natl Acad Sci USA 100: 2106–2111, 2003.PMC14996612578959Asai A, Sahani N, Kaneki M, Ouchi Y, Martyn JA, Yasuhara SE. Primary role of functional ischemia, quantitative evidence for the two-hit mechanism, and phosphodiesterase-5 inhibitor therapy in mouse muscular dystrophy. PLoS One 2: e806, 2007.PMC195008617726536Ascah A, Khairallah M, Daussin F, Bourcier-Lucas C, Godin R, Allen BG, Petrof BJ, Des RC, Burelle Y. Stress-induced opening of the permeability transition pore in the dystrophin-deficient heart is attenuated by acute treatment with sildenafil. Am J Physiol Heart Circ Physiol 300: H144–H153, 2011.20971771Backman E, Nylander E, Johansson I, Henriksson KG, Tagesson C. Selenium and vitamin E treatment of Duchenne muscular dystrophy: no effect on muscle function. Acta Neurol Scand 78: 429–435, 1988.3218450Badalamente MA, Stracher A. Delay of muscle degeneration and necrosis in mdx mice by calpain inhibition. Muscle Nerve 23: 106–111, 2000.10590413Balasubramanian S, Fung ET, Huganir RL. Characterization of the tyrosine phosphorylation and distribution of dystrobrevin isoforms. FEBS Lett 432: 133–140, 1998.9720911Baltgalvis KA, Jaeger MA, Fitzsimons DP, Thayer SA, Lowe DA, Ervasti JM. Transgenic overexpression of gamma-cytoplasmic actin protects against eccentric contraction-induced force loss in mdx mice. Skelet Muscle 1: 32, 2011.PMC321476621995957Banks GB, Chamberlain JS. The value of mammalian models for duchenne muscular dystrophy in developing therapeutic strategies. Curr Top Dev Biol 84: 431–453, 2008.19186250Banks GB, Judge LM, Allen JM, Chamberlain JS. The polyproline site in hinge 2 influences the functional capacity of truncated dystrophins. PLoS Genet 6: e1000958, 2010.PMC287392420502633Bansal D, Miyake K, Vogel SS, Groh S, Chen CC, Williamson R, McNeil PL, Campbell KP. Defective membrane repair in dysferlin-deficient muscular dystrophy. Nature 423: 168–172, 2003.12736685Barbieri E, Sestili P. Reactive oxygen species in skeletal muscle signaling. J Signal Transduct 2012: 982794, 2012.PMC323581122175016Becker PE. Two families of benign sex-linked recessive muscular dystrophy. Rev Can Biol 21: 551–566, 1962.13970129Bedard K, Krause KH. The NOX family of ROS-generating NADPH oxidases: physiology and pathophysiology. Physiol Rev 87: 245–313, 2007.17237347Bellinger AM, Reiken S, Carlson C, Mongillo M, Liu X, Rothman L, Matecki S, Lacampagne A, Marks AR. Hypernitrosylated ryanodine receptor calcium release channels are leaky in dystrophic muscle. Nat Med 15: 325–330, 2009.PMC291057919198614Bellinger AM, Reiken S, Dura M, Murphy PW, Deng SX, Landry DW, Nieman D, Lehnart SE, Samaru M, Lacampagne A, Marks AR. Remodeling of ryanodine receptor complex causes “leaky” channels: a molecular mechanism for decreased exercise capacity. Proc Natl Acad Sci USA 105: 2198–2202, 2008.PMC253889818268335Berger J, Berger S, Hall TE, Lieschke GJ, Currie PD. Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology. Neuromuscul Disord 20: 826–832, 2010.20850317Best A, Ahmed S, Kozma R, Lim L. The Ras-related GTPase Rac1 binds tubulin. J Biol Chem 271: 3756–3762, 1996.8631991Bhasin N, Law R, Liao G, Safer D, Ellmer J, Discher BM, Sweeney HL, Discher DE. Molecular extensibility of mini-dystrophins and a dystrophin rod construct. J Mol Biol 352: 795–806, 2005.16139300Bhat HF, Baba RA, Adams ME, Khanday FA. Role of SNTA1 in Rac1 activation, modulation of ROS generation, and migratory potential of human breast cancer cells. Br J Cancer 110: 706–714, 2014.PMC391511024434436Blake DJ, Nawrotzki R, Peters MF, Froehner SC, Davies KE. Isoform diversity of dystrobrevin, the murine 87-kDa postsynaptic protein. J Biol Chem 271: 7802–7810, 1996.8631824Blake DJ, Weir A, Newey SE, Davies KE. Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol Rev 82: 291–329, 2002.11917091Blau HM, Webster C, Pavlath GK. Defective myoblasts identified in Duchenne muscular dystrophy. Proc Natl Acad Sci USA 80: 4856–4860, 1983.PMC3841446576361Bodensteiner JB, Engel AG. Intracellular calcium accumulation in Duchenne dystrophy and other myopathies: a study of 567,000 muscle fibers in 114 biopsies. Neurology 28: 439–446, 1978.76996Bodor M, McDonald CM. Why short stature is beneficial in Duchenne muscular dystrophy. Muscle Nerve 48: 336–342, 2013.23893308Boittin FX, Petermann O, Hirn C, Mittaud P, Dorchies OM, Roulet E, Ruegg UT. Ca2+-independent phospholipase A2 enhances store-operated Ca2+ entry in dystrophic skeletal muscle fibers. J Cell Sci 119: 3733–3742, 2006.16926189Bonilla E, Fischbeck K, Schotland DL. Freeze-fracture studies of muscle caveolae in human muscular dystrophy. Am J Pathol 104: 167–173, 1981.PMC19037517258302Boppart MD, Burkin DJ, Kaufman SJ. Alpha7beta1-integrin regulates mechanotransduction and prevents skeletal muscle injury. Am J Physiol Cell Physiol 290: C1660–C1665, 2006.16421207Bornman L, Rossouw H, Gericke GS, Polla BS. Effects of iron deprivation on the pathology and stress protein expression in murine X-linked muscular dystrophy. Biochem Pharmacol 56: 751–757, 1998.9751080Bowe MA, Mendis DB, Fallon JR. The small leucine-rich repeat proteoglycan biglycan binds to alpha-dystroglycan and is upregulated in dystrophic muscle. J Cell Biol 148: 801–810, 2000.PMC216936110684260Bozzi M, Morlacchi S, Bigotti MG, Sciandra F, Brancaccio A. Functional diversity of dystroglycan. Matrix Biol 28: 179–187, 2009.19303439Bradley WG, O'Brien MD, Walder DN, Murchison D, Johnson M, Newell DJ. Failure to confirm a vascular cause of muscular dystrophy. Arch Neurol 32: 466–473, 1975.1137513Brancaccio P, Maffulli N, Limongelli FM. Creatine kinase monitoring in sport medicine. Br Med Bull 81–82: 209–230, 2007.17569697Brenman JE, Chao DS, Gee SH, McGee AW, Craven SE, Santillano DR, Wu Z, Huang F, Xia H, Peters MF, Froehner SC, Bredt DS. Interaction of nitric oxide synthase with the postsynaptic density protein PSD-95 and alpha1-syntrophin mediated by PDZ domains. Cell 84: 757–767, 1996.8625413Brenman JE, Xia H, Chao DS, Black SM, Bredt DS. Regulation of neuronal nitric oxide synthase through alternative transcripts. Dev Neurosci 19: 224–231, 1997.9208206Brinkmeier H, Ohlendieck K. Chaperoning heat shock proteins: proteomic analysis and relevance for normal and dystrophin-deficient muscle. Proteomics Clin Appl 8: 875–895, 2014.24895218Brown SC, Torelli S, Ugo I, De BF, Howman EV, Poon E, Britton J, Davies KE, Muntoni F. Syncoilin upregulation in muscle of patients with neuromuscular disease. Muscle Nerve 32: 715–725, 2005.16124004Buechler C, Boettcher A, Bared SM, Probst MC, Schmitz G. The carboxy terminus of the ATP-binding cassette transporter A1 interacts with a beta2-syntrophin/utrophin complex. Biochem Biophys Res Commun 293: 759–765, 2002.12054535Bulfield G, Siller WG, Wight PA, Moore KJ. X chromosome-linked muscular dystrophy (mdx) in the mouse. Proc Natl Acad Sci USA 81: 1189–1192, 1984.PMC3447916583703Buraei Z, Yang J. The ss subunit of voltage-gated Ca2+ channels. Physiol Rev 90: 1461–1506, 2010.PMC435350020959621Buraei Z, Yang J. Structure and function of the beta subunit of voltage-gated Ca2+ channels. Biochim Biophys Acta 1828: 1530–1540, 2013.PMC358700922981275Burkin DJ, Wallace GQ, Milner DJ, Chaney EJ, Mulligan JA, Kaufman SJ. Transgenic expression of α7β1 integrin maintains muscle integrity, increases regenerative capacity, promotes hypertrophy, and reduces cardiomyopathy in dystrophic mice. Am J Pathol 166: 253–263, 2005.PMC160228715632017Bushby KM, Gardner-Medwin D. The clinical, genetic and dystrophin characteristics of Becker muscular dystrophy I. Natural history. J Neurol 240: 98–104, 1993.8437027Call JA, Voelker KA, Wolff AV, McMillan RP, Evans NP, Hulver MW, Talmadge RJ, Grange RW. Endurance capacity in maturing mdx mice is markedly enhanced by combined voluntary wheel running and green tea extract. J Appl Physiol 105: 923–932, 2008.PMC253682118583385Carsana A, Frisso G, Tremolaterra MR, Lanzillo R, Vitale DF, Santoro L, Salvatore F. Analysis of dystrophin gene deletions indicates that the hinge III region of the protein correlates with disease severity. Ann Hum Genet 69: 253–259, 2005.15845029Ceco E, McNally EM. Modifying muscular dystrophy through transforming growth factor-beta. FEBS J 280: 4198–4209, 2013.PMC373141223551962Chamberlain JS, Benian GM. Muscular dystrophy: the worm turns to genetic disease. Curr Biol 10: R795–R797, 2000.11084353Chan S, Head SI. The role of branched fibres in the pathogenesis of Duchenne muscular dystrophy. Exp Physiol 96: 564–571, 2011.21421700Chan S, Head SI, Morley JW. Branched fibers in dystrophic mdx muscle are associated with a loss of force following lengthening contractions. Am J Physiol Cell Physiol 293: C985–C992, 2007.17567750Chandrasekharan K, Yoon JH, Xu Y, deVries S, Camboni M, Janssen PM, Varki A, Martin PT. A human-specific deletion in mouse Cmah increases disease severity in the mdx model of Duchenne muscular dystrophy. Sci Transl Med 2: 42ra54, 2010.PMC295011020668298Chang WJ, Iannaccone ST, Lau KS, Masters BS, McCabe TJ, McMillan K, Padre RC, Spencer MJ, Tidball JG, Stull JT. Neuronal nitric oxide synthase and dystrophin-deficient muscular dystrophy. Proc Natl Acad Sci USA 93: 9142–9147, 1996.PMC386098799168Chao DS, Gorospe JR, Brenman JE, Rafael JA, Peters MF, Froehner SC, Hoffman EP, Chamberlain JS, Bredt DS. Selective loss of sarcolemmal nitric oxide synthase in Becker muscular dystrophy. J Exp Med 184: 609–618, 1996.PMC21927298760814Chargé SB, Rudnicki MA. Cellular and molecular regulation of muscle regeneration. Physiol Rev 84: 209–238, 2004.14715915Chelly J, Kaplan JC, Maire P, Gautron S, Kahn A. Transcription of the dystrophin gene in human muscle and non-muscle tissue. Nature 333: 858–860, 1988.3290682Chen Q, Li W, Quan Z, Sumpio BE. Modulation of vascular smooth muscle cell alignment by cyclic strain is dependent on reactive oxygen species and P38 mitogen-activated protein kinase. J Vasc Surg 37: 660–668, 2003.12618707Chen YW, Nagaraju K, Bakay M, McIntyre O, Rawat R, Shi R, Hoffman EP. Early onset of inflammation and later involvement of TGFbeta in Duchenne muscular dystrophy. Neurology 65: 826–834, 2005.16093456Childers MK, Bogan JR, Bogan DJ, Greiner H, Holder M, Grange RW, Kornegay JN. Chronic administration of a leupeptin-derived calpain inhibitor fails to ameliorate severe muscle pathology in a canine model of duchenne muscular dystrophy. Front Pharmacol 2: 89, 2011.PMC325358322291646Chin ER, Allen DG. The role of elevations in intracellular Ca2+ concentration in the development of low frequency fatigue in mouse single muscle fibres. J Physiol 491: 813–824, 1996.PMC11588208815213Christensen AP, Corey DP. TRP channels in mechanosensation: direct or indirect activation? Nat Rev Neurosci 8: 510–521, 2007.17585304Ciciliot S, Schiaffino S. Regeneration of mammalian skeletal muscle. Basic mechanisms and clinical implications. Curr Pharm Des 16: 906–914, 2010.20041823Claflin DR, Brooks SV. Direct observation of failing fibers in muscles of dystrophic mice provides mechanistic insight into muscular dystrophy. Am J Physiol Cell Physiol 294: C651–C658, 2008.18171725Clarke MS, Khakee R, McNeil PL. Loss of cytoplasmic basic fibroblast growth factor from physiologically wounded myofibers of normal and dystrophic muscle. J Cell Sci 106: 121–133, 1993.8270618Cohen AW, Hnasko R, Schubert W, Lisanti MP. Role of caveolae and caveolins in health and disease. Physiol Rev 84: 1341–1379, 2004.15383654Compton AG, Albrecht DE, Seto JT, Cooper ST, Ilkovski B, Jones KJ, Challis D, Mowat D, Ranscht B, Bahlo M, Froehner SC, North KN. Mutations in contactin-1, a neural adhesion and neuromuscular junction protein, cause a familial form of lethal congenital myopathy. Am J Hum Genet 83: 714–724, 2008.PMC266806919026398Compton AG, Cooper ST, Hill PM, Yang N, Froehner SC, North KN. The syntrophin-dystrobrevin subcomplex in human neuromuscular disorders. J Neuropathol Exp Neurol 64: 350–361, 2005.15835271Connors NC, Adams ME, Froehner SC, Kofuji P. The potassium channel Kir4.1 associates with the dystrophin-glycoprotein complex via alpha-syntrophin in glia. J Biol Chem 279: 28387–28392, 2004.15102837Corona BT, Balog EM, Doyle JA, Rupp JC, Luke RC, Ingalls CP. Junctophilin damage contributes to early strength deficits and EC coupling failure after eccentric contractions. Am J Physiol Cell Physiol 298: C365–C376, 2010.19940065Costantini JL, Cheung SM, Hou S, Li H, Kung SK, Johnston JB, Wilkins JA, Gibson SB, Marshall AJ. TAPP2 links phosphoinositide 3-kinase signaling to B-cell adhesion through interaction with the cytoskeletal protein utrophin: expression of a novel cell adhesion-promoting complex in B-cell leukemia. Blood 114: 4703–4712, 2009.19786618Cox GA, Phelps SF, Chapman VM, Chamberlain JS. New mdx mutation disrupts expression of muscle and nonmuscle isoforms of dystrophin. Nat Genet 4: 87–93, 1993.8099842Crosbie RH, Barresi R, Campbell KP. Loss of sarcolemma nNOS in sarcoglycan-deficient muscle. FASEB J 16: 1786–1791, 2002.12409321Crosbie RH, Lebakken CS, Holt KH, Venzke DP, Straub V, Lee JC, Grady RM, Chamberlain JS, Sanes JR, Campbell KP. Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex. J Cell Biol 145: 153–165, 1999.PMC214822510189375Crosbie RH, Yamada H, Venzke DP, Lisanti MP, Campbell KP. Caveolin-3 is not an integral component of the dystrophin glycoprotein complex. FEBS Lett 427: 279–282, 1998.9607328Davies KE, Pearson PL, Harper PS, Murray JM, O'Brien T, Sarfarazi M, Williamson R. Linkage analysis of two cloned DNA sequences flanking the Duchenne muscular dystrophy locus on the short arm of the human X chromosome. Nucleic Acids Res 11: 2303–2312, 1983.PMC3258856304647Davis TA, Loos B, Engelbrecht AM. AHNAK: the giant jack of all trades. Cell Signal 26: 2683–2693, 2014.25172424De Palma C, Clementi E. Nitric oxide in myogenesis and therapeutic muscle repair. Mol Neurobiol 46: 682–692, 2012.22821188De Palma C, Morisi F, Cheli S, Pambianco S, Cappello V, Vezzoli M, Rovere-Querini P, Moggio M, Ripolone M, Francolini M, Sandri M, Clementi E. Autophagy as a new therapeutic target in Duchenne muscular dystrophy. Cell Death Dis 5: e1363, 2014.PMC445429825101676De Senzi Moraes PR, Ferretti R, Moraes LH, Neto HS, Marques MJ, Minatel E. N-acetylcysteine treatment reduces TNF-alpha levels and myonecrosis in diaphragm muscle of mdx mice. Clin Nutr 32: 472–475, 2013.22727548De PC, Morisi F, Cheli S, Pambianco S, Cappello V, Vezzoli M, Rovere-Querini P, Moggio M, Ripolone M, Francolini M, Sandri M, Clementi E. Autophagy as a new therapeutic target in Duchenne muscular dystrophy. Cell Death Dis 3: e418, 2012.PMC354259523152054Decary S, Hamida CB, Mouly V, Barbet JP, Hentati F, Butler-Browne GS. Shorter telomeres in dystrophic muscle consistent with extensive regeneration in young children. Neuromuscul Disord 10: 113–120, 2000.10714586Decary S, Mouly V, Hamida CB, Sautet A, Barbet JP, Butler-Browne GS. Replicative potential and telomere length in human skeletal muscle: implications for satellite cell-mediated gene therapy. Hum Gene Ther 8: 1429–1438, 1997.9287143Deconinck AE, Rafael JA, Skinner JA, Brown SC, Potter AC, Metzinger L, Watt DJ, Dickson JG, Tinsley JM, Davies KE. Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy. Cell 90: 717–727, 1997.9288751Desguerre I, Mayer M, Leturcq F, Barbet JP, Gherardi RK, Christov C. Endomysial fibrosis in Duchenne muscular dystrophy: a marker of poor outcome associated with macrophage alternative activation. J Neuropathol Exp Neurol 68: 762–773, 2009.19535995Disatnik MH, Dhawan J, Yu Y, Beal MF, Whirl MM, Franco AA, Rando TA. Evidence of oxidative stress in mdx mouse muscle: studies of the pre-necrotic state. J Neurol Sci 161: 77–84, 1998.9879685Divet A, Huchet-Cadiou C. Sarcoplasmic reticulum function in slow- and fast-twitch skeletal muscles from mdx mice. Pflügers Arch 444: 634–643, 2002.12194017Doran P, Dowling P, Lohan J, McDonnell K, Poetsch S, Ohlendieck K. Subproteomics analysis of Ca2+-binding proteins demonstrates decreased calsequestrin expression in dystrophic mouse skeletal muscle. Eur J Biochem 271: 3943–3952, 2004.15373840Dorchies OM, Wagner S, Vuadens O, Waldhauser K, Buetler TM, Kucera P, Ruegg UT. Green tea extract and its major polyphenol (−)-epigallocatechin gallate improve muscle function in a mouse model for Duchenne muscular dystrophy. Am J Physiol Cell Physiol 290: C616–C625, 2006.16403950Doyle DD, Goings G, Upshaw-Earley J, Ambler SK, Mondul A, Palfrey HC, Page E. Dystrophin associates with caveolae of rat cardiac myocytes: relationship to dystroglycan. Circ Res 87: 480–488, 2000.10988240Duchenne GBA. Recherches sur la paralysie musculaire pseudohypertrophique ou paralysie myo-sclerosique. Arch Gen Med 11: multiple articles, 1868.Ducret T, Vandebrouck C, Cao ML, Lebacq J, Gailly P. Functional role of store-operated and stretch-activated channels in murine adult skeletal muscle fibres. J Physiol 575: 913–924, 2006.PMC199567616825296Dudley RW, Danialou G, Govindaraju K, Lands L, Eidelman DE, Petrof BJ. Sarcolemmal damage in dystrophin deficiency is modulated by synergistic interactions between mechanical and oxidative/nitrosative stresses. Am J Pathol 168: 1276–1287, 2006.PMC160657416565501Duggan DJ, Hoffman EP. Autosomal recessive muscular dystrophy and mutations of the sarcoglycan complex. Neuromuscul Disord 6: 475–482, 1996.9027858Duguez S, Duddy W, Johnston H, Laine J, Le Bihan MC, Brown KJ, Bigot A, Hathout Y, Butler-Browne G, Partridge T. Dystrophin deficiency leads to disturbance of LAMP1-vesicle-associated protein secretion. Cell Mol Life Sci 70: 2159–2174, 2013.PMC1111377923344255Dutka TL, Mollica JP, Posterino GS, Lamb GD. Modulation of contractile apparatus Ca2+ sensitivity and disruption of excitation-contraction coupling by S-nitrosoglutathione in rat muscle fibres. J Physiol 589: 2181–2196, 2011.PMC309869721115647Dyachenko V, Husse B, Rueckschloss U, Isenberg G. Mechanical deformation of ventricular myocytes modulates both TRPC6 and Kir2.3 channels. Cell Calcium 45: 38–54, 2009.18635261Eagle M, Baudouin SV, Chandler C, Giddings DR, Bullock R, Bushby K. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 12: 926–929, 2002.12467747Edwards JN, Friedrich O, Cully TR, von WF, Murphy RM, Launikonis BS. Upregulation of store-operated Ca2+ entry in dystrophic mdx mouse muscle. Am J Physiol Cell Physiol 299: C42–C50, 2010.20427714Edwards RHT, Hill DK, Jones DA, Merton PA. Fatigue of long duration in human skeletal muscle after exercise. J Physiol 272: 769–778, 1977.PMC1353654592214Elhanany-Tamir H, Yu YV, Shnayder M, Jain A, Welte M, Volk T. Organelle positioning in muscles requires cooperation between two KASH proteins and microtubules. J Cell Biol 198: 833–846, 2012.PMC343276422927463Emery AE, Muntoni F. Duchenne Muscular Dystrophy. New York: Oxford Univ. Press, 2003.England SB, Nicholson LV, Johnson MA, Forrest SM, Love DR, Zubrzycka-Gaarn EE, Bulman DE, Harris JB, Davies KE. Very mild muscular dystrophy associated with the deletion of 46% of dystrophin. Nature 343: 180–182, 1990.2404210Ermolova NV, Martinez L, Vetrone SA, Jordan MC, Roos KP, Sweeney HL, Spencer MJ. Long-term administration of the TNF blocking drug Remicade (cV1q) to mdx mice reduces skeletal and cardiac muscle fibrosis, but negatively impacts cardiac function. Neuromuscul Disord 24: 583–595, 2014.PMC412252024844454Ervasti JM. Costameres: the Achilles' heel of Herculean muscle. J Biol Chem 278: 13591–13594, 2003.12556452Ervasti JM, Campbell KP. A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin. J Cell Biol 122: 809–823, 1993.PMC21195878349731Fairclough RJ, Wood MJ, Davies KE. Therapy for Duchenne muscular dystrophy: renewed optimism from genetic approaches. Nat Rev Genet 14: 373–378, 2013.23609411Faulkner JA, Ng R, Davis CS, Li S, Chamberlain JS. Diaphragm muscle strip preparation for evaluation of gene therapies in mdx mice. Clin Exp Pharmacol Physiol 35: 725–729, 2008.18215182Feske S, Gwack Y, Prakriya M, Srikanth S, Puppel SH, Tanasa B, Hogan PG, Lewis RS, Daly M, Rao A. A mutation in Orai1 causes immune deficiency by abrogating CRAC channel function. Nature 441: 179–185, 2006.16582901Finanger Hedderick EL, Simmers JL, Soleimani A, Andres-Mateos E, Marx R, Files DC, King L, Crawford TO, Corse AM, Cohn RD. Loss of sarcolemmal nNOS is common in acquired and inherited neuromuscular disorders. Neurology 76: 960–967, 2011.PMC305913921403107Fink RH, Stephenson DG, Williams DA. Physiological properties of skinned fibres from normal and dystrophic (Duchenne) human muscle activated by Ca2+ and Sr2+. J Physiol 420: 337–353, 1990.PMC11900532324989Finkel RS, Flanigan KM, Wong B, Bonnemann C, Sampson J, Sweeney HL, Reha A, Northcutt VJ, Elfring G, Barth J, Peltz SW. Phase 2a study of ataluren-mediated dystrophin production in patients with nonsense mutation duchenne muscular dystrophy. PLoS One 8: e81302, 2013.PMC385949924349052Firestein BL, Bredt DS. Interaction of neuronal nitric-oxide synthase and phosphofructokinase-M. J Biol Chem 274: 10545–10550, 1999.10187848Flanigan KM. The muscular dystrophies. Semin Neurol 32: 255–263, 2012.23117950Franco A Jr, Lansman JB. Calcium entry through stretch-inactivated ion channels in mdx myotubes. Nature 344: 670–673, 1990.1691450Franco-Obregon A, Lansman JB. Changes in mechanosensitive channel gating following mechanical stimulation in skeletal muscle myotubes from the mdx mouse. J Physiol 539: 391–407, 2002.PMC229016711882673Friden J, Lieber RL. Eccentric exercise-induced injuries to contractile and cytoskeletal muscle fibre components. Acta Physiol Scand 171: 321–326, 2001.11412144Friden J, Lieber RL. Serum creatine kinase level is a poor predictor of muscle function after injury. Scand J Med Sci Sports 11: 126–127, 2001.11252462Friedrich O, Both M, Weber C, Schurmann S, Teichmann MD, von WF, Fink RH, Vogel M, Chamberlain JS, Garbe C. Microarchitecture is severely compromised but motor protein function is preserved in dystrophic mdx skeletal muscle. Biophys J 98: 606–616, 2010.PMC282064620159157Froehner SC, Reed SM, Anderson KN, Huang PL, Percival JM. Loss of nNOS inhibits compensatory muscle hypertrophy and exacerbates inflammation and eccentric contraction-induced damage in mdx mice. Hum Mol Genet 24: 492–505, 2015.PMC427507525214536Fuhrmann-Stroissnigg H, Noiges R, Descovich L, Fischer I, Albrecht DE, Nothias F, Froehner SC, Propst F. The light chains of microtubule-associated proteins MAP1A and MAP1B interact with alpha1-syntrophin in the central and peripheral nervous system. PLoS One 7: e49722, 2012.PMC349670723152929Gailly P, Boland B, Himpens B, Casteels R, Gillis JM. Critical evaluation of cytosolic calcium determination in resting muscle fibres from normal and dystrophic (mdx) mice. Cell Calcium 14: 473–483, 1993.8358771Gailly P, De Backer F, Van Schoor M, Gillis JM. In situ measurements of calpain activity in isolated muscle fibres from normal and dystrophin-lacking, mdx mice. Ca2+ dependence in various physiological conditions. J Physiol 582: 1261–1275, 2007.PMC207523617510188Galbiati F, Engelman JA, Volonte D, Zhang XL, Minetti C, Li M, Hou H Jr, Kneitz B, Edelmann W, Lisanti MP. Caveolin-3 null mice show a loss of caveolae, changes in the microdomain distribution of the dystrophin-glycoprotein complex, and t-tubule abnormalities. J Biol Chem 276: 21425–21433, 2001.11259414Galbiati F, Volonte D, Chu JB, Li M, Fine SW, Fu M, Bermudez J, Pedemonte M, Weidenheim KM, Pestell RG, Minetti C, Lisanti MP. Transgenic overexpression of caveolin-3 in skeletal muscle fibers induces a Duchenne-like muscular dystrophy phenotype. Proc Natl Acad Sci USA 97: 9689–9694, 2000.PMC1692610931944Gamstorp I, Gustavson KH, Hellstrom O, Nordgren B. A trial of selenium and vitamin E in boys with muscular dystrophy. J Child Neurol 1: 211–214, 1986.3298399Garcia-Pelagio KP, Bloch RJ, Ortega A, Gonzalez-Serratos H. Biomechanics of the sarcolemma and costameres in single skeletal muscle fibers from normal and dystrophin-null mice. J Muscle Res Cell Motil 31: 323–336, 2011.PMC432608221312057Gazzerro E, Bonetto A, Minetti C. Caveolinopathies: translational implications of caveolin-3 in skeletal and cardiac muscle disorders. Handb Clin Neurol 101: 135–142, 2011.21496630Gazzerro E, Sotgia F, Bruno C, Lisanti MP, Minetti C. Caveolinopathies: from the biology of caveolin-3 to human diseases. Eur J Hum Genet 18: 137–145, 2010.PMC298718319584897Gee SH, Madhavan R, Levinson SR, Caldwell JH, Sealock R, Froehner SC. Interaction of muscle and brain sodium channels with multiple members of the syntrophin family of dystrophin-associated proteins. J Neurosci 18: 128–137, 1998.PMC67933849412493Gehrig SM, van der Poel C, Sayer TA, Schertzer JD, Henstridge DC, Church JE, Lamon S, Russell AP, Davies KE, Febbraio MA, Lynch GS. Hsp72 preserves muscle function and slows progression of severe muscular dystrophy. Nature 484: 394–398, 2012.22495301Gentil C, Leturcq F, Ben YR, Kaplan JC, Laforet P, Penisson-Besnier I, Espil-Taris C, Voit T, Garcia L, Pietri-Rouxel F. Variable phenotype of del45-55 Becker patients correlated with nNOSmu mislocalization and RYR1 hypernitrosylation. Hum Mol Genet 21: 3449–3460, 2012.22589245Gervasio OL, Whitehead NP, Yeung EW, Phillips WD, Allen DG. TRPC1 binds to caveolin-3 and is regulated by Src kinase: role in Duchenne muscular dystrophy. J Cell Sci 121: 2246–2255, 2008.18544631Giacomotto J, Brouilly N, Walter L, Mariol MC, Berger J, Segalat L, Becker TS, Currie PD, Gieseler K. Chemical genetics unveils a key role of mitochondrial dynamics, cytochrome c release and IP3R activity in muscular dystrophy. Hum Mol Genet 22: 4562–4578, 2013.23804750Gillis JM. Understanding dystrophinopathies: an inventory of the structural and functional consequences of the absence of dystrophin in muscles of the mdx mouse. J Muscle Res Cell Motil 20: 605–625, 1999.10672510Goll DE, Thompson VF, Li H, Wei W, Cong J. The calpain system. Physiol Rev 83: 731–801, 2003.12843408Goonasekera SA, Lam CK, Millay DP, Sargent MA, Hajjar RJ, Kranias EG, Molkentin JD. Mitigation of muscular dystrophy in mice by SERCA overexpression in skeletal muscle. J Clin Invest 121: 1044–1052, 2011.PMC304936721285509Gottlieb P, Folgering J, Maroto R, Raso A, Wood TG, Kurosky A, Bowman C, Bichet D, Patel A, Sachs F, Martinac B, Hamill OP, Honore E. Revisiting TRPC1 and TRPC6 mechanosensitivity. Pflügers Arch 455: 1097–1103, 2008.17957383Gowers WR. Pseudo-Hypertrophic Muscular Paralysis–A Clinical Lecture. London: Churchill, 1879.Grady RM, Akaaboune M, Cohen AL, Maimone MM, Lichtman JW, Sanes JR. Tyrosine-phosphorylated and nonphosphorylated isoforms of alpha-dystrobrevin: roles in skeletal muscle and its neuromuscular and myotendinous junctions. J Cell Biol 160: 741–752, 2003.PMC217335212604589Grady RM, Grange RW, Lau KS, Maimone MM, Nichol MC, Stull JT, Sanes JR. Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies. Nat Cell Biol 1: 215–220, 1999.10559919Grady RM, Teng HB, Nichol MC, Cunningham JC, Wilkinson RS, Sanes JR. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Cell 90: 729–738, 1997.9288752Grosso S, Perrone S, Longini M, Bruno C, Minetti C, Gazzolo D, Balestri P, Buonocore G. Isoprostanes in dystrophinopathy: evidence of increased oxidative stress. Brain Dev 30: 391–395, 2008.18180123Grote K, Flach I, Luchtefeld M, Akin E, Holland SM, Drexler H, Schieffer B. Mechanical stretch enhances mRNA expression and proenzyme release of matrix metalloproteinase-2 (MMP-2) via NAD(P)H oxidase-derived reactive oxygen species. Circ Res 92: e80-e86, 2003.12750313Grounds MD, Torrisi J. Anti-TNFalpha (Remicade) therapy protects dystrophic skeletal muscle from necrosis. FASEB J 18: 676–682, 2004.15054089Grupe M, Myers G, Penner R, Fleig A. Activation of store-operated I(CRAC) by hydrogen peroxide. Cell Calcium 48: 1–9, 2010.PMC292931620646759Guharay F, Sachs F. Stretch-activated single ion channel currents in tissue-cultured embryonic chick skeletal muscle. J Physiol 352: 685–701, 1984.PMC11932376086918Gyurko R, Leupen S, Huang PL. Deletion of exon 6 of the neuronal nitric oxide synthase gene in mice results in hypogonadism and infertility. Endocrinology 143: 2767–2774, 2002.12072412Haenggi T, Fritschy JM. Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue. Cell Mol Life Sci 63: 1614–1631, 2006.PMC1113631316710609Hamer PW, McGeachie JM, Davies MJ, Grounds MD. Evans Blue Dye as an in vivo marker of myofibre damage: optimising parameters for detecting initial myofibre membrane permeability. J Anat 200: 69–79, 2002.PMC157088311837252Hamill OP. Twenty odd years of stretch-sensitive channels. Pflügers Arch 453: 333–351, 2006.17021800Hammadi M, Oulidi A, Gackiere F, Katsogiannou M, Slomianny C, Roudbaraki M, Dewailly E, Delcourt P, Lepage G, Lotteau S, Ducreux S, Prevarskaya N, Van CF. Modulation of ER stress and apoptosis by endoplasmic reticulum calcium leak via translocon during unfolded protein response: involvement of GRP78. FASEB J 27: 1600–1609, 2013.23322163Han W, Li H, Villar VA, Pascua AM, Dajani MI, Wang X, Natarajan A, Quinn MT, Felder RA, Jose PA, Yu P. Lipid rafts keep NADPH oxidase in the inactive state in human renal proximal tubule cells. Hypertension 51: 481–487, 2008.18195159Hansford RG, Hogue BA, Mildaziene V. Dependence of H2O2 formation by rat heart mitochondria on substrate availability and donor age. J Bioenerg Biomembr 29: 89–95, 1997.9067806Hara H, Nolan PM, Scott MO, Bucan M, Wakayama Y, Fischbeck KH. Running endurance abnormality in mdx mice. Muscle Nerve 25: 207–211, 2002.11870688Harper SQ, Hauser MA, DelloRusso C, Duan D, Crawford RW, Phelps SF, Harper HA, Robinson AS, Engelhardt JF, Brooks SV, Chamberlain JS. Modular flexibility of dystrophin: implications for gene therapy of Duchenne muscular dystrophy. Nat Med 8: 253–261, 2002.11875496Hasegawa M, Cuenda A, Spillantini MG, Thomas GM, Buee-Scherrer V, Cohen P, Goedert M. Stress-activated protein kinase-3 interacts with the PDZ domain of alpha1-syntrophin. A mechanism for specific substrate recognition. J Biol Chem 274: 12626–12631, 1999.10212242Hauser E, Hoger H, Bittner R, Widhalm K, Herkner K, Lubec G. Oxyradical damage and mitochondrial enzyme activities in the mdx mouse. Neuropediatrics 26: 260–262, 1995.8552217Haycock JW, MacNeil S, Jones P, Harris JB, Mantle D. Oxidative damage to muscle protein in Duchenne muscular dystrophy. Neuroreport 8: 357–361, 1996.9051810Hays AP, Hallett M, Delfs J, Morris J, Sotrel A, Shevchuk MM, DiMauro S. Muscle phosphofructokinase deficiency: abnormal polysaccharide in a case of late-onset myopathy. Neurology 31: 1077–1086, 1981.6943439Head BP, Patel HH, Roth DM, Murray F, Swaney JS, Niesman IR, Farquhar MG, Insel PA. Microtubules and actin microfilaments regulate lipid raft/caveolae localization of adenylyl cyclase signaling components. J Biol Chem 281: 26391–26399, 2006.16818493Head SI. Branched fibres in old dystrophic mdx muscle are associated with mechanical weakening of the sarcolemma, abnormal Ca2+ transients and a breakdown of Ca2+ homeostasis during fatigue. Exp Physiol 95: 641–656, 2010.20139167Heller KN, Montgomery CL, Janssen PM, Clark KR, Mendell JR, Rodino-Klapac LR. AAV-mediated overexpression of human alpha7 integrin leads to histological and functional improvement in dystrophic mice. Mol Ther 21: 520–525, 2013.PMC358916723319059Henricson EK, Abresch RT, Cnaan A, Hu F, Duong T, Arrieta A, Han J, Escolar DM, Florence JM, Clemens PR, Hoffman EP, McDonald CM. The cooperative international neuromuscular research group Duchenne natural history study: glucocorticoid treatment preserves clinically meaningful functional milestones and reduces rate of disease progression as measured by manual muscle testing and other commonly used clinical trial outcome measures. Muscle Nerve 48: 55–67, 2013.PMC410317023649481Heydemann A, McNally EM. Consequences of disrupting the dystrophin-sarcoglycan complex in cardiac and skeletal myopathy. Trends Cardiovasc Med 17: 55–59, 2007.17292047Hidalgo C, Sanchez G, Barrientos G, Aracena-Parks P. A transverse tubule NADPH oxidase activity stimulates calcium release from isolated triads via ryanodine receptor type 1 S-glutathionylation. J Biol Chem 281: 26473–26482, 2006.16762927Hill MM, Bastiani M, Luetterforst R, Kirkham M, Kirkham A, Nixon SJ, Walser P, Abankwa D, Oorschot VM, Martin S, Hancock JF, Parton RG. PTRF-Cavin, a conserved cytoplasmic protein required for caveola formation and function. Cell 132: 113–124, 2008.PMC226525718191225Hillier BJ, Christopherson KS, Prehoda KE, Bredt DS, Lim WA. Unexpected modes of PDZ domain scaffolding revealed by structure of nNOS-syntrophin complex. Science 284: 812–815, 1999.10221915Hirokawa N. Kinesin and dynein superfamily proteins and the mechanism of organelle transport. Science 279: 519–526, 1998.9438838Hnia K, Hugon G, Rivier F, Masmoudi A, Mercier J, Mornet D. Modulation of p38 mitogen-activated protein kinase cascade and metalloproteinase activity in diaphragm muscle in response to free radical scavenger administration in dystrophin-deficient Mdx mice. Am J Pathol 170: 633–643, 2007.PMC185188117255331Hodgetts S, Radley H, Davies M, Grounds MD. Reduced necrosis of dystrophic muscle by depletion of host neutrophils, or blocking TNFalpha function with Etanercept in mdx mice. Neuromuscul Disord 16: 591–602, 2006.16935507Hoffman EP, Brown RH Jr, Kunkel LM. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell 51: 919–928, 1987.3319190Hoffman EP, Connor EM. Orphan drug development in muscular dystrophy: update on two large clinical trials of dystrophin rescue therapies. Discov Med 16: 233–239, 2013.24229740Hoffman EP, McNally EM. Exon-skipping therapy: a roadblock, detour, or bump in the road? Sci Transl Med 6: 230fs14, 2014.PMC446478524695683Hollingworth S, Zeiger U, Baylor SM. Comparison of the myoplasmic calcium transient elicited by an action potential in intact fibres of mdx and normal mice. J Physiol 586: 5063–5075, 2008.PMC265214218772198Hoogerwaard EM, Bakker E, Ippel PF, Oosterwijk JC, Majoor-Krakauer DF, Leschot NJ, van Essen AJ, Brunner HG, van der Wouw PA, Wilde AA, de VM. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in The Netherlands: a cohort study. Lancet 353: 2116–2119, 1999.10382696Hopf FW, Turner PR, Denetclaw WF Jr, Reddy P, Steinhardt RA. A critical evaluation of resting intracellular free calcium regulation in dystrophic mdx muscle. Am J Physiol Cell Physiol 271: C1325–C1339, 1996.8897840Huang H, Bae C, Sachs F, Suchyna TM. Caveolae regulation of mechanosensitive channel function in myotubes. PLoS One 8: e72894, 2013.PMC375835124023653Huang PL, Dawson TM, Bredt DS, Snyder SH, Fishman MC. Targeted disruption of the neuronal nitric oxide synthase gene. Cell 75: 1273–1286, 1993.7505721Huebner KD, Jassal DS, Halevy O, Pines M, Anderson JE. Functional resolution of fibrosis in mdx mouse dystrophic heart and skeletal muscle by halofuginone. Am J Physiol Heart Circ Physiol 294: H1550–H1561, 2008.18263710Hurt KJ, Sezen SF, Champion HC, Crone JK, Palese MA, Huang PL, Sawa A, Luo X, Musicki B, Snyder SH, Burnett AL. Alternatively spliced neuronal nitric oxide synthase mediates penile erection. Proc Natl Acad Sci USA 103: 3440–3443, 2006.PMC141393616488973Hutter OF. The membrane hypothesis of Duchenne muscular dystrophy: quest for functional evidence. J Inherit Metab Dis 15: 565–577, 1992.1528017Hutter OF, Burton FL, Bovell DL. Mechanical properties of normal and mdx mouse sarcolemma: bearing on function of dystrophin. J Muscle Res Cell Motil 12: 585–589, 1991.1791198Ilsley JL, Sudol M, Winder SJ. The interaction of dystrophin with beta-dystroglycan is regulated by tyrosine phosphorylation. Cell Signal 13: 625–632, 2001.11495720Ilsley JL, Sudol M, Winder SJ. The WW domain: linking cell signalling to the membrane cytoskeleton. Cell Signal 14: 183–189, 2002.11812645Iwata Y, Katanosaka Y, Arai Y, Shigekawa M, Wakabayashi S. Dominant-negative inhibition of Ca2+ influx via TRPV2 ameliorates muscular dystrophy in animal models. Hum Mol Genet 18: 824–834, 2009.19050039Jackson MJ. Redox regulation of adaptive responses in skeletal muscle to contractile activity. Free Radic Biol Med 47: 1267–1275, 2009.19748570Jaffrey SR, Snyder SH. PIN: an associated protein inhibitor of neuronal nitric oxide synthase. Science 274: 774–777, 1996.8864115Javesghani D, Magder SA, Barreiro E, Quinn MT, Hussain SN. Molecular characterization of a superoxide-generating NAD(P)H oxidase in the ventilatory muscles. Am J Respir Crit Care Med 165: 412–418, 2002.11818330Joe MK, Kee C, Tomarev SI. Myocilin interacts with syntrophins and is member of dystrophin-associated protein complex. J Biol Chem 287: 13216–13227, 2012.PMC333994122371502Johnson BD, Scheuer T, Catterall WA. Convergent regulation of skeletal muscle Ca2+ channels by dystrophin, the actin cytoskeleton, and cAMP-dependent protein kinase. Proc Natl Acad Sci USA 102: 4191–4196, 2005.PMC55481715753322Johnson EK, Li B, Yoon JH, Flanigan KM, Martin PT, Ervasti J, Montanaro F. Identification of new dystroglycan complexes in skeletal muscle. PLoS One 8: e73224, 2013.PMC373856423951345Johnson EK, Zhang L, Adams ME, Phillips A, Freitas MA, Froehner SC, Green-Church KB, Montanaro F. Proteomic analysis reveals new cardiac-specific dystrophin-associated proteins. PLoS One 7: e43515, 2012.PMC342737222937058Jones DP. Redefining oxidative stress. Antioxid Redox Signal 8: 1865–1879, 2006.16987039Kargacin ME, Kargacin GJ. The sarcoplasmic reticulum calcium pump is functionally altered in dystrophic muscle. Biochim Biophys Acta 1290: 4–8, 1996.8645705Kawahara G, Gasperini MJ, Myers JA, Widrick JJ, Eran A, Serafini PR, Alexander MS, Pletcher MT, Morris CA, Kunkel LM. Dystrophic muscle improvement in zebrafish via increased heme oxygenase signaling. Hum Mol Genet 23: 1869–1878, 2014.PMC394352324234649Kawahara G, Kunkel LM. Zebrafish based small molecule screens for novel DMD drugs. Drug Discov Today Technol 10: e91-e96, 2013.PMC363949723646060Kawamura S, Miyamoto S, Brown JH. Initiation and transduction of stretch-induced RhoA and Rac1 activation through caveolae: cytoskeletal regulation of ERK translocation. J Biol Chem 278: 31111–31117, 2003.12777392Kawasaki BT, Liao Y, Birnbaumer L. Role of Src in C3 transient receptor potential channel function and evidence for a heterogeneous makeup of receptor- and store-operated Ca2+ entry channels. Proc Natl Acad Sci USA 103: 335–340, 2006.PMC132616716407161Khairallah M, Khairallah RJ, Young ME, Allen BG, Gillis MA, Danialou G, Deschepper CF, Petrof BJ, Des RC. Sildenafil and cardiomyocyte-specific cGMP signaling prevent cardiomyopathic changes associated with dystrophin deficiency. Proc Natl Acad Sci USA 105: 7028–7033, 2008.PMC238397718474859Khairallah RJ, Shi G, Sbrana F, Prosser BL, Borroto C, Mazaitis MJ, Hoffman EP, Mahurkar A, Sachs F, Sun Y, Chen YW, Raiteri R, Lederer WJ, Dorsey SG, Ward CW. Microtubules underlie dysfunction in duchenne muscular dystrophy. Sci Signal 5: ra56, 2012.PMC383566022871609Kim JH, Kwak HB, Thompson LV, Lawler JM. Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy. J Muscle Res Cell Motil 34: 1–13, 2013.23104273Kim MJ, Hwang SH, Lim JA, Froehner SC, Adams ME, Kim HS. Alpha-syntrophin modulates myogenin expression in differentiating myoblasts. PLoS One 5: e15355, 2010.PMC300368521179410Klinge L, Dekomien G, Aboumousa A, Charlton R, Epplen JT, Barresi R, Bushby K, Straub V. Sarcoglycanopathies: can muscle immunoanalysis predict the genotype? Neuromuscul Disord 18: 934–941, 2008.18996010Kobayashi YM, Rader EP, Crawford RW, Iyengar NK, Thedens DR, Faulkner JA, Parikh SV, Weiss RM, Chamberlain JS, Moore SA, Campbell KP. Sarcolemma-localized nNOS is required to maintain activity after mild exercise. Nature 456: 511–515, 2008.PMC258864318953332Koenig M, Kunkel LM. Detailed analysis of the repeat domain of dystrophin reveals four potential hinge segments that may confer flexibility. J Biol Chem 265: 4560–4566, 1990.2407739Kornegay JN, Bogan JR, Bogan DJ, Childers MK, Li J, Nghiem P, Detwiler DA, Larsen CA, Grange RW, Bhavaraju-Sanka RK, Tou S, Keene BP, Howard JF Jr, Wang J, Fan Z, Schatzberg SJ, Styner MA, Flanigan KM, Xiao X, Hoffman EP. Canine models of Duchenne muscular dystrophy and their use in therapeutic strategies. Mamm Genome 23: 85–108, 2012.PMC391188422218699Kottlors M, Kirschner J. Elevated satellite cell number in Duchenne muscular dystrophy. Cell Tissue Res 340: 541–548, 2010.20467789Kramerova I, Kudryashova E, Venkatraman G, Spencer MJ. Calpain 3 participates in sarcomere remodeling by acting upstream of the ubiquitin-proteasome pathway. Hum Mol Genet 14: 2125–2134, 2005.15961411Kroemer G, Levine B. Autophagic cell death: the story of a misnomer. Nat Rev Mol Cell Biol 9: 1004–1010, 2008.PMC272735818971948Kumar A, Boriek AM. Mechanical stress activates the nuclear factor-kappaB pathway in skeletal muscle fibers: a possible role in Duchenne muscular dystrophy. FASEB J 17: 386–396, 2003.12631578Kung C. A possible unifying principle for mechanosensation. Nature 436: 647–654, 2005.16079835Kunkel LM. 2004 William Allan Award address. Cloning of the DMD gene. Am J Hum Genet 76: 205–214, 2005.PMC119636315714686Kunkel LM, Hejtmancik JF, Caskey CT, Speer A, Monaco AP, Middlesworth W, Colletti CA, Bertelson C, Muller U, Bresnan M, Shapiro F, Tantravahi U, Speer J, Latt SA, Bartlett R, Pericak-Vance MA, Roses AD, Thompson MW, Ray PN, Worton RG, Fischbeck KH, Gallano P, Coulon M, Duros C, Boue J, Junien C, Chelly J, Hamard G, Jeanpierre M, Lambert M, Kaplan JC, Emery A, Dorkins H, McGlade S, Davies KE, Boehm C, Arveiler B, Lemaire C, Morgan GJ, Denton MJ, Amos J, Bobrow M, Benham F, Boswinkel E, Cole C, Dubowitz V, Hart K, Hodgson S, Johnson L, Walker A, Roncuzzi L, Ferlini A, Nobile C, Romeo G, Wilcox DE, Affara NA, Ferguson-Smith MA, Lindolf M, Kaariainen H, de la Chapelle A, Ionasescu V, Searby C, Ionasescu R, Bakker E, van Ommen GJ, Pearson PL, Greenberg CR, Hamerton JL, Wrogemann K, Doherty RA, Polakowska R, Hyser C, Quirk S, Thomas N, Harper JF, Darras BT, Francke U. Analysis of deletions in DNA from patients with Becker and Duchenne muscular dystrophy. Nature 322: 73–77, 1986.3014348Kunkel LM, Monaco AP, Middlesworth W, Ochs HD, Latt SA. Specific cloning of DNA fragments absent from the DNA of a male patient with an X-chromosome deletion. Proc Natl Acad Sci USA 82: 4778–4782, 1985.PMC3909882991893Kurebayashi N, Ogawa Y. Depletion of Ca2+ in the sarcoplasmic reticulum stimulates Ca2+ entry into mouse skeletal muscle fibres. J Physiol 533: 185–199, 2001.PMC227859111351027Lai Y, Thomas GD, Yue Y, Yang HT, Li D, Long C, Judge L, Bostick B, Chamberlain JS, Terjung RL, Duan D. Dystrophins carrying spectrin-like repeats 16 and 17 anchor nNOS to the sarcolemma and enhance exercise performance in a mouse model of muscular dystrophy. J Clin Invest 119: 624–635, 2009.PMC264869219229108Lai Y, Zhao J, Yue Y, Duan D. alpha2 and alpha3 helices of dystrophin R16 and R17 frame a microdomain in the alpha1 helix of dystrophin R17 for neuronal NOS binding. Proc Natl Acad Sci USA 110: 525–530, 2013.PMC354579123185009Lamb CA, Yoshimori T, Tooze SA. The autophagosome: origins unknown, biogenesis complex. Nat Rev Mol Cell Biol 14: 759–774, 2013.24201109Lamb GD, Junankar PR, Stephenson DG. Raised intracellular [Ca2+] abolishes excitation-contraction coupling in skeletal muscle fibres of rat and toad. J Physiol 489: 349–362, 1995.PMC11567638847631Lamb GD, Westerblad H. Acute effects of reactive oxygen and nitrogen species on the contractile function of skeletal muscle. J Physiol 589: 2119–2127, 2011.PMC309869121041533Lambeth JD. Nox enzymes, ROS, and chronic disease: an example of antagonistic pleiotropy. Free Radic Biol Med 43: 332–347, 2007.PMC201373717602948Launikonis BS, Barnes M, Stephenson DG. Identification of the coupling between skeletal muscle store-operated Ca2+ entry and the inositol trisphosphate receptor. Proc Natl Acad Sci USA 100: 2941–2944, 2003.PMC15144512601149Launikonis BS, Murphy RM, Edwards JN. Toward the roles of store-operated Ca2+ entry in skeletal muscle. Pflügers Arch 460: 813–823, 2010.20577885Le Borgne F, Guyot S, Logerot M, Beney L, Gervais P, Demarquoy J. Exploration of lipid metabolism in relation with plasma membrane properties of Duchenne muscular dystrophy cells: influence of l-carnitine. PLoS One 7: e49346, 2012.PMC350783023209572Le Rumeur E, Hubert JF, Winder SJ. A new twist to coiled coil. FEBS Lett 586: 2717–2722, 2012.22584055Le Rumeur E, Winder SJ, Hubert JF. Dystrophin: more than just the sum of its parts. Biochim Biophys Acta 1804: 1713–1722, 2010.20472103Leask A, Abraham DJ. TGF-beta signaling and the fibrotic response. FASEB J 18: 816–827, 2004.15117886Legardinier S, Raguenes-Nicol C, Tascon C, Rocher C, Hardy S, Hubert JF, Le RE. Mapping of the lipid-binding and stability properties of the central rod domain of human dystrophin. J Mol Biol 389: 546–558, 2009.19379759Leonoudakis D, Conti LR, Anderson S, Radeke CM, McGuire LM, Adams ME, Froehner SC, Yates JR III, Vandenberg CA. Protein trafficking and anchoring complexes revealed by proteomic analysis of inward rectifier potassium channel (Kir2.x)-associated proteins. J Biol Chem 279: 22331–22346, 2004.15024025Lesault PF, Theret M, Magnan M, Cuvellier S, Niu Y, Gherardi RK, Tremblay JP, Hittinger L, Chazaud B. Macrophages improve survival, proliferation and migration of engrafted myogenic precursor cells into MDX skeletal muscle. PLoS One 7: e46698, 2012.PMC346274723056408Li D, Bareja A, Judge L, Yue Y, Lai Y, Fairclough R, Davies KE, Chamberlain JS, Duan D. Sarcolemmal nNOS anchoring reveals a qualitative difference between dystrophin and utrophin. J Cell Sci 123: 2008–2013, 2010.PMC288001220483958Li D, Shin JH, Duan D. iNOS ablation does not improve specific force of the extensor digitorum longus muscle in dystrophin-deficient mdx4cv mice. PLoS One 6: e21618, 2011.PMC312808821738735Li D, Yue Y, Lai Y, Hakim CH, Duan D. Nitrosative stress elicited by nNOSmicro delocalization inhibits muscle force in dystrophin-null mice. J Pathol 223: 88–98, 2011.PMC310908421125668Lin C, Guo X, Lange S, Liu J, Ouyang K, Yin X, Jiang L, Cai Y, Mu Y, Sheikh F, Ye S, Chen J, Ke Y, Cheng H. Cypher/ZASP is a novel A-kinase anchoring protein. J Biol Chem 288: 29403–29413, 2013.PMC379524123996002Lindahl M, Backman E, Henriksson KG, Gorospe JR, Hoffman EP. Phospholipase A2 activity in dystrophinopathies. Neuromuscul Disord 5: 193–199, 1995.7633184Loufrani L, Dubroca C, You D, Li Z, Levy B, Paulin D, Henrion D. Absence of dystrophin in mice reduces NO-dependent vascular function and vascular density: total recovery after a treatment with the aminoglycoside gentamicin. Arterioscler Thromb Vasc Biol 24: 671–676, 2004.PMC223385114751810Lovering RM, Michaelson L, Ward CW. Malformed mdx myofibers have normal cytoskeletal architecture yet altered EC coupling and stress-induced Ca2+ signaling. Am J Physiol Cell Physiol 297: C571–C580, 2009.PMC274039019605736Lumeng C, Phelps S, Crawford GE, Walden PD, Barald K, Chamberlain JS. Interactions between beta 2-syntrophin and a family of microtubule-associated serine/threonine kinases. Nat Neurosci 2: 611–617, 1999.10404183Lumeng CN, Hauser M, Brown V, Chamberlain JS. Expression of the 71 kDa dystrophin isoform (Dp71) evaluated by gene targeting. Brain Res 830: 174–178, 1999.10350571Luo S, Chen Y, Lai KO, Arevalo JC, Froehner SC, Adams ME, Chao MV, Ip NY. α-Syntrophin regulates ARMS localization at the neuromuscular junction and enhances EphA4 signaling in an ARMS-dependent manner. J Cell Biol 169: 813–824, 2005.PMC217161115939763Lyfenko AD, Dirksen RT. Differential dependence of store-operated and excitation-coupled Ca2+ entry in skeletal muscle on STIM1 and Orai1. J Physiol 586: 4815–4824, 2008.PMC261405918772199Lynch GS, Hinkle RT, Chamberlain JS, Brooks SV, Faulkner JA. Force and power output of fast and slow skeletal muscles from mdx mice 6–28 months old. J Physiol 535: 591–600, 2001.PMC227878211533147Lyssand JS, DeFino MC, Tang XB, Hertz AL, Feller DB, Wacker JL, Adams ME, Hague C. Blood pressure is regulated by an alpha1D-adrenergic receptor/dystrophin signalosome. J Biol Chem 283: 18792–18800, 2008.PMC244155218468998Lyssand JS, Lee KS, DeFino M, Adams ME, Hague C. Syntrophin isoforms play specific functional roles in the alpha1D-adrenergic receptor/DAPC signalosome. Biochem Biophys Res Commun 412: 596–601, 2011.PMC404540921846462Lyssand JS, Whiting JL, Lee KS, Kastl R, Wacker JL, Bruchas MR, Miyatake M, Langeberg LK, Chavkin C, Scott JD, Gardner RG, Adams ME, Hague C. Alpha-dystrobrevin-1 recruits alpha-catulin to the alpha1D-adrenergic receptor/dystrophin-associated protein complex signalosome. Proc Natl Acad Sci USA 107: 21854–21859, 2010.PMC300311221115837Mack NA, Porter AP, Whalley HJ, Schwarz JP, Jones RC, Khaja AS, Bjartell A, Anderson KI, Malliri A. Beta2-syntrophin and Par-3 promote an apicobasal Rac activity gradient at cell-cell junctions by differentially regulating Tiam1 activity. Nat Cell Biol 14: 1169–1180, 2012.PMC349806723103911Madhavan R, Jarrett HW. Phosphorylation of dystrophin and alpha-syntrophin by Ca2+-calmodulin dependent protein kinase II. Biochim Biophys Acta 1434: 260–274, 1999.10525145Mair J, Mayr M, Muller E, Koller A, Haid C, Artner-Dworzak E, Calzolari C, Larue C, Puschendorf B. Rapid adaptation to eccentric exercise-induced muscle damage. Int J Sports Med 16: 352–356, 1995.7591384Malik V, Rodino-Klapac LR, Mendell JR. Emerging drugs for Duchenne muscular dystrophy. Expert Opin Emerg Drugs 17: 261–277, 2012.PMC348643122632414Mann CJ, Perdiguero E, Kharraz Y, Aguilar S, Pessina P, Serrano AL, Muñoz-Cánoves P. Aberrant repair and fibrosis development in skeletal muscle. Skelet Muscle 1: 21, 2011. (doi: 10.1186/2044-5040-1-21).10.1186/2044-5040-1-21PMC315664421798099Maroto R, Raso A, Wood TG, Kurosky A, Martinac B, Hamill OP. TRPC1 forms the stretch-activated cation channel in vertebrate cells. Nat Cell Biol 7: 179–185, 2005.15665854Marshall JL, Chou E, Oh J, Kwok A, Burkin DJ, Crosbie-Watson RH. Dystrophin and utrophin expression require sarcospan: loss of alpha7 integrin exacerbates a newly discovered muscle phenotype in sarcospan-null mice. Hum Mol Genet 21: 4378–4393, 2012.PMC345946222798625Marshall JL, Crosbie-Watson RH. Sarcospan: a small protein with large potential for Duchenne muscular dystrophy. Skeletal Muscle 3: 1, 2013.PMC359965323282144Marshall JL, Holmberg J, Chou E, Ocampo AC, Oh J, Lee J, Peter AK, Martin PT, Crosbie-Watson RH. Sarcospan-dependent Akt activation is required for utrophin expression and muscle regeneration. J Cell Biol 197: 1009–1027, 2012.PMC338441122734004Martin EA, Barresi R, Byrne BJ, Tsimerinov EI, Scott BL, Walker AE, Gurudevan SV, Anene F, Elashoff RM, Thomas GD, Victor RG. Tadalafil alleviates muscle ischemia in patients with Becker muscular dystrophy. Sci Transl Med 4: 162ra155, 2012.PMC393543023197572Martins AS, Shkryl VM, Nowycky MC, Shirokova N. Reactive oxygen species contribute to Ca2+ signals produced by osmotic stress in mouse skeletal muscle fibres. J Physiol 586: 197–210, 2008.PMC237556817974587Masuda-Hirata M, Suzuki A, Amano Y, Yamashita K, Ide M, Yamanaka T, Sakai M, Imamura M, Ohno S. Intracellular polarity protein PAR-1 regulates extracellular laminin assembly by regulating the dystroglycan complex. Genes Cells 14: 835–850, 2009.19549170Mathews KD, Moore SA. Limb-girdle muscular dystrophy. Curr Neurol Neurosci Rep 3: 78–85, 2003.12507416Matsumura K, Ervasti JM, Ohlendieck K, Kahl SD, Campbell KP. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature 360: 588–591, 1992.1461282McConell GK, Rattigan S, Lee-Young RS, Wadley GD, Merry TL. Skeletal muscle nitric oxide signaling and exercise: a focus on glucose metabolism. Am J Physiol Endocrinol Metab 303: E301–E307, 2012.22550064McConell GK, Wadley GD. Potential role of nitric oxide in contraction-stimulated glucose uptake and mitochondrial biogenesis in skeletal muscle. Clin Exp Pharmacol Physiol 35: 1488–1492, 2008.18759853McDonald CM, Henricson EK, Han JJ, Abresch RT, Nicorici A, Atkinson L, Elfring GL, Reha A, Miller LL. The 6-minute walk test in Duchenne/Becker muscular dystrophy: longitudinal observations. Muscle Nerve 42: 966–974, 2010.21038378McNeil PL, Khakee R. Disruptions of muscle fiber plasma membranes. Role in exercise-induced damage. Am J Pathol 140: 1097–1109, 1992.PMC18865181374591McNeil PL, Steinhardt RA. Plasma membrane disruption: repair, prevention, adaptation. Annu Rev Cell Dev Biol 19: 697–731, 2003.14570587Meinen S, Lin S, Ruegg MA, Punga AR. Fatigue and muscle atrophy in a mouse model of myasthenia gravis is paralleled by loss of sarcolemmal nNOS. PLoS One 7: e44148, 2012.PMC342945222952904Mendell JR, Campbell K, Rodino-Klapac L, Sahenk Z, Shilling C, Lewis S, Bowles D, Gray S, Li C, Galloway G, Malik V, Coley B, Clark KR, Li J, Xiao X, Samulski J, McPhee SW, Samulski RJ, Walker CM. Dystrophin immunity in Duchenne's muscular dystrophy. N Engl J Med 363: 1429–1437, 2010.PMC301410620925545Mendell JR, Moxley RT, Griggs RC, Brooke MH, Fenichel GM, Miller JP, King W, Signore L, Pandya S, Florence J. Randomized, double-blind six-month trial of prednisone in Duchenne's muscular dystrophy. N Engl J Med 320: 1592–1597, 1989.2657428Mendell JR, Rodino-Klapac L, Sahenk Z, Malik V, Kaspar BK, Walker CM, Clark KR. Gene therapy for muscular dystrophy: lessons learned and path forward. Neurosci Lett 527: 90–99, 2012.PMC349293622609847Mendell JR, Rodino-Klapac LR, Sahenk Z, Roush K, Bird L, Lowes LP, Alfano L, Gomez AM, Lewis S, Kota J, Malik V, Shontz K, Walker CM, Flanigan KM, Corridore M, Kean JR, Allen HD, Shilling C, Melia KR, Sazani P, Saoud JB, Kaye EM. Eteplirsen for the treatment of Duchenne muscular dystrophy. Ann Neurol 74: 637–647, 2013.23907995Menke A, Jockusch H. Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature 349: 69–71, 1991.1985268Menke A, Jockusch H. Extent of shock-induced membrane leakage in human and mouse myotubes depends on dystrophin. J Cell Sci 108: 727–733, 1995.7769014Mercado ML, Amenta AR, Hagiwara H, Rafii MS, Lechner BE, Owens RT, McQuillan DJ, Froehner SC, Fallon JR. Biglycan regulates the expression and sarcolemmal localization of dystrobrevin, syntrophin, and nNOS. FASEB J 20: 1724–1726, 2006.PMC305644016807372Mercuri E, Muntoni F. The ever-expanding spectrum of congenital muscular dystrophies. Ann Neurol 72: 9–17, 2012.22829265Messina S, Altavilla D, Aguennouz M, Seminara P, Minutoli L, Monici MC, Bitto A, Mazzeo A, Marini H, Squadrito F, Vita G. Lipid peroxidation inhibition blunts nuclear factor-kappaB activation, reduces skeletal muscle degeneration, and enhances muscle function in mdx mice. Am J Pathol 168: 918–926, 2006.PMC160651516507907Messina S, Vita GL, Aguennouz M, Sframeli M, Romeo S, Rodolico C, Vita G. Activation of NF-kappaB pathway in Duchenne muscular dystrophy: relation to age. Acta Myol 30: 16–23, 2011.PMC318583221842588Michaelson LP, Shi G, Ward CW, Rodney GG. Mitochondrial redox potential during contraction in single intact muscle fibers. Muscle Nerve 42: 522–529, 2010.PMC301517920730875Millay DP, Goonasekera SA, Sargent MA, Maillet M, Aronow BJ, Molkentin JD. Calcium influx is sufficient to induce muscular dystrophy through a TRPC-dependent mechanism. Proc Natl Acad Sci USA 106: 19023–19028, 2009.PMC277644119864620Miller G, Moore CJ, Terry R, La RT, Mitchell A, Piggott R, Dear TN, Wells DJ, Winder SJ. Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse. Hum Mol Genet 21: 4508–4520, 2012.PMC588637322810924Miller G, Peter AK, Espinoza E, Heighway J, Crosbie RH. Over-expression of Microspan, a novel component of the sarcoplasmic reticulum, causes severe muscle pathology with triad abnormalities. J Muscle Res Cell Motil 27: 545–558, 2006.16823602Miller G, Wang EL, Nassar KL, Peter AK, Crosbie RH. Structural and functional analysis of the sarcoglycan-sarcospan subcomplex. Exp Cell Res 313: 639–651, 2007.PMC385535117223103Mizuno Y, Thompson TG, Guyon JR, Lidov HG, Brosius M, Imamura M, Ozawa E, Watkins SC, Kunkel LM. Desmuslin, an intermediate filament protein that interacts with alpha-dystrobrevin and desmin. Proc Natl Acad Sci USA 98: 6156–6161, 2001.PMC3343811353857Mizushima N, Komatsu M. Autophagy: renovation of cells and tissues. Cell 147: 728–741, 2011.22078875Moens P, Baatsen PH, Marechal G. Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch. J Muscle Res Cell Motil 14: 446–451, 1993.7693747Mofarrahi M, Brandes RP, Gorlach A, Hanze J, Terada LS, Quinn MT, Mayaki D, Petrof B, Hussain SN. Regulation of proliferation of skeletal muscle precursor cells by NADPH oxidase. Antioxid Redox Signal 10: 559–574, 2008.18092937Mokhtarian A, Lefaucheur JP, Even PC, Sebille A. Hindlimb immobilization applied to 21-day-old mdx mice prevents the occurrence of muscle degeneration. J Appl Physiol 86: 924–931, 1999.10066706Mokri B, Engel AG. Duchenne dystrophy: electron microscopic findings pointing to a basic or early abnormality in the plasma membrane of the muscle fiber. Neurology 25: 1111–1120, 1975.1105232Monaco AP, Neve RL, Collettifeener C, Bertelson CJ, Kurnit DM, Kunkel LM. Isolation of candidate cDNAs for portions of the Duchenne Muscular-Dystrophy gene. Nature 323: 646–650, 1986.3773991Moore CJ, Winder SJ. The inside and out of dystroglycan post-translational modification. Neuromuscul Disord 22: 959–965, 2012.22770978Moorwood C. Syncoilin, an intermediate filament-like protein linked to the dystrophin associated protein complex in skeletal muscle. Cell Mol Life Sci 65: 2957–2963, 2008.PMC1113173118810324Moorwood C, Barton ER. Caspase-12 ablation preserves muscle function in the mdx mouse. Hum Mol Genet 23: 5325–5341, 2014.PMC416882124879640Morales MG, Cabello-Verrugio C, Santander C, Cabrera D, Goldschmeding R, Brandan E. CTGF/CCN-2 over-expression can directly induce features of skeletal muscle dystrophy. J Pathol 225: 490–501, 2011.21826667Morales MG, Gutierrez J, Cabello-Verrugio C, Cabrera D, Lipson KE, Goldschmeding R, Brandan E. Reducing CTGF/CCN2 slows down mdx muscle dystrophy and improves cell therapy. Hum Mol Genet 22: 4938–4951, 2013.23904456Mundy DI, Machleidt T, Ying YS, Anderson RG, Bloom GS. Dual control of caveolar membrane traffic by microtubules and the actin cytoskeleton. J Cell Sci 115: 4327–4339, 2002.12376564Munehira Y, Ohnishi T, Kawamoto S, Furuya A, Shitara K, Imamura M, Yokota T, Takeda S, Amachi T, Matsuo M, Kioka N, Ueda K. Alpha1-syntrophin modulates turnover of ABCA1. J Biol Chem 279: 15091–15095, 2004.14722086Muraki K, Iwata Y, Katanosaka Y, Ito T, Ohya S, Shigekawa M, Imaizumi Y. TRPV2 is a component of osmotically sensitive cation channels in murine aortic myocytes. Circ Res 93: 829–838, 2003.14512441Murphy RM, Dutka TL, Horvath D, Bell JR, Delbridge LM, Lamb GD. Ca2+-dependent proteolysis of junctophilin-1 and junctophilin-2 in skeletal and cardiac muscle. J Physiol 591: 719–729, 2013.PMC357753923148318Murphy RM, Lamb GD. Endogenous calpain-3 activation is primarily governed by small increases in resting cytoplasmic [Ca2+] and is not dependent on stretch. J Biol Chem 284: 7811–7819, 2009.PMC265807519144634Murphy RM, Mollica JP, Lamb GD. Plasma membrane removal in rat skeletal muscle fibers reveals caveolin-3 hot-spots at the necks of transverse tubules. Exp Cell Res 315: 1015–1028, 2009.19101541Murphy RM, Verburg E, Lamb GD. Ca2+-activation of diffusible and bound pools of μ-calpain in rat skeletal muscle. J Physiol 576: 595–612, 2006.PMC189035316857710Murphy S, Henry M, Meleady P, Zweyer M, Mundegar RR, Swandulla D, Ohlendieck K. Simultaneous pathoproteomic evaluation of the dystrophin-glycoprotein complex and secondary changes in the mdx-4cv mouse model of Duchenne Muscular Dystrophy. Biology 4: 397–423, 2015.PMC449830726067837Na S, Collin O, Chowdhury F, Tay B, Ouyang M, Wang Y, Wang N. Rapid signal transduction in living cells is a unique feature of mechanotransduction. Proc Natl Acad Sci USA 105: 6626–6631, 2008.PMC237331518456839Nakae Y, Dorchies OM, Stoward PJ, Zimmermann BF, Ritter C, Ruegg UT. Quantitative evaluation of the beneficial effects in the mdx mouse of epigallocatechin gallate, an antioxidant polyphenol from green tea. Histochem Cell Biol 137: 811–827, 2012.PMC335310922331205Nakajima H, Raben N, Hamaguchi T, Yamasaki T. Phosphofructokinase deficiency; past, present and future. Curr Mol Med 2: 197–212, 2002.11949936Nastase MV, Young MF, Schaefer L. Biglycan: a multivalent proteoglycan providing structure and signals. J Histochem Cytochem 60: 963–975, 2012.PMC352788622821552Neely JD, Amiry-Moghaddam M, Ottersen OP, Froehner SC, Agre P, Adams ME. Syntrophin-dependent expression and localization of Aquaporin-4 water channel protein. Proc Natl Acad Sci USA 98: 14108–14113, 2001.PMC6117611717465Neumeier M, Krautbauer S, Schmidhofer S, Hader Y, Eisinger K, Eggenhofer E, Froehner SC, Adams ME, Mages W, Buechler C. Adiponectin receptor 1 C-terminus interacts with PDZ-domain proteins such as syntrophins. Exp Mol Pathol 95: 180–186, 2013.PMC384137823860432Newbell BJ, Anderson JT, Jarrett HW. Ca2+-calmodulin binding to mouse alpha1 syntrophin: syntrophin is also a Ca2+-binding protein. Biochemistry 36: 1295–1305, 1997.9063877Newey SE, Benson MA, Ponting CP, Davies KE, Blake DJ. Alternative splicing of dystrobrevin regulates the stoichiometry of syntrophin binding to the dystrophin protein complex. Curr Biol 10: 1295–1298, 2000.11069112Newey SE, Howman EV, Ponting CP, Benson MA, Nawrotzki R, Loh NY, Davies KE, Blake DJ. Syncoilin, a novel member of the intermediate filament superfamily that interacts with alpha-dystrobrevin in skeletal muscle. J Biol Chem 276: 6645–6655, 2001.11053421Ng R, Banks GB, Hall JK, Muir LA, Ramos JN, Wicki J, Odom GL, Konieczny P, Seto J, Chamberlain JR, Chamberlain JS. Animal models of muscular dystrophy. Prog Mol Biol Transl Sci 105: 83–111, 2012.PMC487262222137430Nichol JA, Hutter OF. Ca2+ loading reduces the tensile strength of sarcolemmal vesicles shed from rabbit muscle. J Physiol 493: 199–209, 1996.PMC11589618735705Nordmann C, Strokin M, Schonfeld P, Reiser G. Putative roles of Ca-independent phospholipase A in respiratory chain-associated ROS production in brain mitochondria: influence of docosahexaenoic acid and bromoenol lactone. J Neurochem 131: 163–176, 2014.24923354Nuss JE, Amaning JK, Bailey CE, DeFord JH, Dimayuga VL, Rabek JP, Papaconstantinou J. Oxidative modification and aggregation of creatine kinase from aged mouse skeletal muscle. Aging 1: 557–572, 2009.PMC283007920195383O'Neill A, Williams MW, Resneck WG, Milner DJ, Capetanaki Y, Bloch RJ. Sarcolemmal organization in skeletal muscle lacking desmin: evidence for cytokeratins associated with the membrane skeleton at costameres. Mol Biol Cell 13: 2347–2359, 2002.PMC11731812134074Oak SA, Russo K, Petrucci TC, Jarrett HW. Mouse alpha1-syntrophin binding to Grb2: further evidence of a role for syntrophin in cell signaling. Biochemistry 40: 11270–11278, 2001.11551227Oddoux S, Zaal KJ, Tate V, Kenea A, Nandkeolyar SA, Reid E, Liu W, Ralston E. Microtubules that form the stationary lattice of muscle fibers are dynamic and nucleated at Golgi elements. J Cell Biol 203: 205–213, 2013.PMC381296424145165Oh HJ, Abraham LS, van HJ, Stove C, Proszynski TJ, Gevaert K, DiMario JX, Sanes JR, van RF, Kim H. Interaction of alpha-catulin with dystrobrevin contributes to integrity of dystrophin complex in muscle. J Biol Chem 287: 21717–21728, 2012.PMC338113422577143Okuhira K, Fitzgerald ML, Sarracino DA, Manning JJ, Bell SA, Goss JL, Freeman MW. Purification of ATP-binding cassette transporter A1 and associated binding proteins reveals the importance of beta1-syntrophin in cholesterol efflux. J Biol Chem 280: 39653–39664, 2005.16192269Ort T, Maksimova E, Dirkx R, Kachinsky AM, Berghs S, Froehner SC, Solimena M. The receptor tyrosine phosphatase-like protein ICA512 binds the PDZ domains of beta2-syntrophin and nNOS in pancreatic beta-cells. Eur J Cell Biol 79: 621–630, 2000.11043403Ort T, Voronov S, Guo J, Zawalich K, Froehner SC, Zawalich W, Solimena M. Dephosphorylation of beta2-syntrophin and Ca2+/mu-calpain-mediated cleavage of ICA512 upon stimulation of insulin secretion. EMBO J 20: 4013–4023, 2001.PMC14914011483505Ozawa E, Mizuno Y, Hagiwara Y, Sasaoka T, Yoshida M. Molecular and cell biology of the sarcoglycan complex. Muscle Nerve 32: 563–576, 2005.15937871Pal R, Palmieri M, Loehr JA, Li S, Abo-Zahrah R, Monroe TO, Thakur PB, Sardiello M, Rodney GG. Src-dependent impairment of autophagy by oxidative stress in a mouse model of Duchenne muscular dystrophy. Nat Commun 5: 4425, 2014.PMC410181125028121Parton RG, del Pozo MA. Caveolae as plasma membrane sensors, protectors and organizers. Nat Rev Mol Cell Biol 14: 98–112, 2013.23340574Partridge TA. The mdx mouse model as a surrogate for Duchenne muscular dystrophy. FEBS J 280: 4177–4186, 2013.PMC414794923551987Pasternak C, Wong S, Elson EL. Mechanical function of dystrophin in muscle cells. J Cell Biol 128: 355–361, 1995.PMC21203427844149Paulin D, Li Z. Desmin: a major intermediate filament protein essential for the structural integrity and function of muscle. Exp Cell Res 301: 1–7, 2004.15501438Pauly M, Daussin F, Burelle Y, Li T, Godin R, Fauconnier J, Koechlin-Ramonatxo C, Hugon G, Lacampagne A, Coisy-Quivy M, Liang F, Hussain S, Matecki S, Petrof BJ. AMPK activation stimulates autophagy and ameliorates muscular dystrophy in the mdx mouse diaphragm. Am J Pathol 181: 583–592, 2012.22683340Peng HM, Morishima Y, Pratt WB, Osawa Y. Modulation of heme/substrate binding cleft of neuronal nitric-oxide synthase (nNOS) regulates binding of Hsp90 and Hsp70 proteins and nNOS ubiquitination. J Biol Chem 287: 1556–1565, 2012.PMC325688922128174Percival JM, Adamo CM, Beavo JA, Froehner SC. Evaluation of the therapeutic utility of phosphodiesterase 5A inhibition in the mdx mouse model of duchenne muscular dystrophy. Handb Exp Pharmacol 323–344, 2011.PMC406312021695647Percival JM, Anderson KN, Gregorevic P, Chamberlain JS, Froehner SC. Functional deficits in nNOSmu-deficient skeletal muscle: myopathy in nNOS knockout mice. PLoS One 3: e3387, 2008.PMC255986218852886Percival JM, Anderson KN, Huang P, Adams ME, Froehner SC. Golgi and sarcolemmal neuronal NOS differentially regulate contraction-induced fatigue and vasoconstriction in exercising mouse skeletal muscle. J Clin Invest 120: 816–826, 2010.PMC282795820124730Percival JM, Froehner SC. Golgi complex organization in skeletal muscle: a role for Golgi-mediated glycosylation in muscular dystrophies? Traffic 8: 184–194, 2007.17319799Percival JM, Gregorevic P, Odom GL, Banks GB, Chamberlain JS, Froehner SC. rAAV6-microdystrophin rescues aberrant Golgi complex organization in mdx skeletal muscles. Traffic 8: 1424–1439, 2007.17714427Percival JM, Siegel MP, Knowels G, Marcinek DJ. Defects in mitochondrial localization and ATP synthesis in the mdx mouse model of Duchenne muscular dystrophy are not alleviated by PDE5 inhibition. Hum Mol Genet 22: 153–167, 2013.PMC352240423049075Percival JM, Whitehead NP, Adams ME, Adamo CM, Beavo JA, Froehner SC. Sildenafil reduces respiratory muscle weakness and fibrosis in the mdx mouse model of Duchenne muscular dystrophy. J Pathol 228: 77–87, 2012.PMC406745522653783Pessina P, Cabrera D, Morales MG, Riquelme CA, Gutierrez J, Serrano AL, Brandan E, Munoz-Canoves P. Novel and optimized strategies for inducing fibrosis in vivo: focus on Duchenne Muscular Dystrophy. Skelet Muscle 4: 7, 2014.PMC414239125157321Peter AK, Marshall JL, Crosbie RH. Sarcospan reduces dystrophic pathology: stabilization of the utrophin-glycoprotein complex. J Cell Biol 183: 419–427, 2008.PMC257577318981229Peters MF, Adams ME, Froehner SC. Differential association of syntrophin pairs with the dystrophin complex. J Cell Biol 138: 81–93, 1997.PMC21399479214383Peters MF, O'Brien KF, Sadoulet-Puccio HM, Kunkel LM, Adams ME, Froehner SC. Beta-dystrobrevin, a new member of the dystrophin family. Identification, cloning, and protein associations. J Biol Chem 272: 31561–31569, 1997.9395493Peters MF, Sadoulet-Puccio HM, Grady MR, Kramarcy NR, Kunkel LM, Sanes JR, Sealock R, Froehner SC. Differential membrane localization and intermolecular associations of alpha-dystrobrevin isoforms in skeletal muscle. J Cell Biol 142: 1269–1278, 1998.PMC21493399732287Peterson JM, Kline W, Canan BD, Ricca DJ, Kaspar B, Delfin DA, DiRienzo K, Clemens PR, Robbins PD, Baldwin AS, Flood P, Kaumaya P, Freitas M, Kornegay JN, Mendell JR, Rafael-Fortney JA, Guttridge DC, Janssen PM. Peptide-based inhibition of NF-kappaB rescues diaphragm muscle contractile dysfunction in a murine model of Duchenne muscular dystrophy. Mol Med 17: 508–515, 2011.PMC310512721267511Petrof BJ, Shrager JB, Stedman HH, Kelly AM, Sweeney HL. Dystrophin protects the sarcolemma from stresses developed during muscle contraction. Proc Natl Acad Sci USA 90: 3710–3714, 1993.PMC463718475120Petrof BJ, Stedman HH, Shrager JB, Eby J, Sweeney HL, Kelly AM. Adaptations in myosin heavy chain expression and contractile function in dystrophic mouse diaphragm. Am J Physiol Cell Physiol 265: C834–C841, 1993.8214039Pierno S, Nico B, Burdi R, Liantonio A, Didonna MP, Cippone V, Fraysse B, Rolland JF, Mangieri D, Andreetta F, Ferro P, Camerino C, Zallone A, Confalonieri P, De Luca A. Role of tumour necrosis factor alpha, but not of cyclo-oxygenase-2-derived eicosanoids, on functional and morphological indices of dystrophic progression in mdx mice: a pharmacological approach. Neuropathol Appl Neurobiol 33: 344–359, 2007.17493014Piluso G, Mirabella M, Ricci E, Belsito A, Abbondanza C, Servidei S, Puca AA, Tonali P, Puca GA, Nigro V. Gamma1- and gamma2-syntrophins, two novel dystrophin-binding proteins localized in neuronal cells. J Biol Chem 275: 15851–15860, 2000.10747910Plant DR, Lynch GS. Depolarization-induced contraction and SR function in mechanically skinned muscle fibers from dystrophic mdx mice. Am J Physiol Cell Physiol 285: C522–C528, 2003.12724137Poon E, Howman EV, Newey SE, Davies KE. Association of syncoilin and desmin: linking intermediate filament proteins to the dystrophin-associated protein complex. J Biol Chem 277: 3433–3439, 2002.11694502Porter GA, Dmytrenko GM, Winkelmann JC, Bloch RJ. Dystrophin colocalizes with beta-spectrin in distinct subsarcolemmal domains in mammalian skeletal muscle. J Cell Biol 117: 997–1005, 1992.PMC22894901577872Prins KW, Humston JL, Mehta A, Tate V, Ralston E, Ervasti JM. Dystrophin is a microtubule-associated protein. J Cell Biol 186: 363–369, 2009.PMC272840519651889Prins KW, Lowe DA, Ervasti JM. Skeletal muscle-specific ablation of gamma(cyto)-actin does not exacerbate the mdx phenotype. PLoS One 3: e2419, 2008.PMC240907518545671Prior TW, Bridgeman SJ. Experience and strategy for the molecular testing of Duchenne muscular dystrophy. J Mol Diagn 7: 317–326, 2005.PMC186754216049303Prosser BL, Ward CW, Lederer WJ. X-ROS signaling: rapid mechano-chemo transduction in heart. Science 333: 1440–1445, 2011.21903813Putney JW., Jr A model for receptor-regulated calcium entry. Cell Calcium 7: 1–12, 1986.2420465Quinlan JG, Wong BL, Niemeier RT, McCullough AS, Levin L, Emanuele M. Poloxamer 188 failed to prevent exercise-induced membrane breakdown in mdx skeletal muscle fibers. Neuromuscular Disorders 16: 855–864, 2006.17118658Rafii MS, Hagiwara H, Mercado ML, Seo NS, Xu T, Dugan T, Owens RT, Hook M, McQuillan DJ, Young MF, Fallon JR. Biglycan binds to alpha- and gamma-sarcoglycan and regulates their expression during development. J Cell Physiol 209: 439–447, 2006.PMC292967216883602Raith M, Valencia RG, Fischer I, Orthofer M, Penninger JM, Spuler S, Rezniczek GA, Wiche G. Linking cytoarchitecture to metabolism: sarcolemma-associated plectin affects glucose uptake by destabilizing microtubule networks in mdx myofibers. Skeletal Muscle 3: 14, 2013.PMC369581023758845Rall S, Grimm T. Survival in Duchenne muscular dystrophy. Acta Myol 31: 117–120, 2012.PMC347685523097602Ralston E, Ploug T. Caveolin-3 is associated with the T-tubules of mature skeletal muscle fibers. Exp Cell Res 246: 510–515, 1999.9925767Ramaswamy KS, Palmer ML, van der Meulen JH, Renoux A, Kostrominova TY, Michele DE, Faulkner JA. Lateral transmission of force is impaired in skeletal muscles of dystrophic mice and very old rats. J Physiol 589: 1195–1208, 2011.PMC306059621224224Rando TA. Role of nitric oxide in the pathogenesis of muscular dystrophies: a “two hit” hypothesis of the cause of muscle necrosis. Microsc Res Tech 55: 223–235, 2001.11748861Rando TA. Oxidative stress and the pathogenesis of muscular dystrophies. Am J Phys Med Rehabil 81: S175–S186, 2002.12409822Rando TA, Disatnik MH, Yu Y, Franco A. Muscle cells from mdx mice have an increased susceptibility to oxidative stress. Neuromuscular Disorders 8: 14–21, 1998.9565986Rayavarapu S, Coley W, Cakir E, Jahnke V, Takeda S, Aoki Y, Grodish-Dressman H, Jaiswal JK, Hoffman EP, Brown KJ, Hathout Y, Nagaraju K. Identification of disease specific pathways using in vivo SILAC proteomics in dystrophin deficient mdx mouse. Mol Cell Proteomics 12: 1061–1073, 2013.PMC365032123297347Reay DP, Yang M, Watchko JF, Daood M, O'Day TL, Rehman KK, Guttridge DC, Robbins PD, Clemens PR. Systemic delivery of NEMO binding domain/IKKgamma inhibitory peptide to young mdx mice improves dystrophic skeletal muscle histopathology. Neurobiol Dis 43: 598–608, 2011.PMC314563321624467Reddy A, Caler EV, Andrews NW. Plasma membrane repair is mediated by Ca2+-regulated exocytosis of lysosomes. Cell 106: 157–169, 2001.11511344Reid MB. Invited Review: redox modulation of skeletal muscle contraction: what we know and what we don't. J Appl Physiol 90: 724–731, 2001.11160074Renjini R, Gayathri N, Nalini A, Srinivas Bharath MM. Oxidative damage in muscular dystrophy correlates with the severity of the pathology: role of glutathione metabolism. Neurochem Res 37: 885–898, 2012.22219131Repetto S, Bado M, Broda P, Lucania G, Masetti E, Sotgia F, Carbone I, Pavan A, Bonilla E, Cordone G, Lisanti MP, Minetti C. Increased number of caveolae and caveolin-3 overexpression in Duchenne muscular dystrophy. Biochem Biophys Res Commun 261: 547–550, 1999.10441463Rezniczek GA, Konieczny P, Nikolic B, Reipert S, Schneller D, Abrahamsberg C, Davies KE, Winder SJ, Wiche G. Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with beta-dystroglycan. J Cell Biol 176: 965–977, 2007.PMC206408217389230Richard I, Broux O, Allamand V, Fougerousse F, Chiannilkulchai N, Bourg N, Brenguier L, Devaud C, Pasturaud P, Roudaut C. Mutations in the proteolytic enzyme calpain 3 cause limb-girdle muscular dystrophy type 2A. Cell 81: 27–40, 1995.7720071Riefler GM, Firestein BL. Binding of neuronal nitric-oxide synthase (nNOS) to carboxyl-terminal-binding protein (CtBP) changes the localization of CtBP from the nucleus to the cytosol: a novel function for targeting by the PDZ domain of nNOS. J Biol Chem 276: 48262–48268, 2001.11590170Rios E. The cell boundary theorem: a simple law of the control of cytosolic calcium concentration. J Physiol Sci 60: 81–84, 2010.PMC282183419937486Roberts RG, Coffey AJ, Bobrow M, Bentley DR. Exon structure of the human dystrophin gene. Genomics 16: 536–538, 1993.8314593Robin G, Berthier C, Allard B. Sarcoplasmic reticulum Ca2+ permeation explored from the lumen side in mdx muscle fibers under voltage control. J Gen Physiol 139: 209–218, 2012.PMC328996122371362Rodino-Klapac LR, Mendell JR, Sahenk Z. Update on the treatment of Duchenne muscular dystrophy. Curr Neurol Neurosci Rep 13: 332, 2013.PMC601461723328943Rooney JE, Gurpur PB, Burkin DJ. Laminin-111 protein therapy prevents muscle disease in the mdx mouse model for Duchenne muscular dystrophy. Proc Natl Acad Sci USA 106: 7991–7996, 2009.PMC268311319416897Rooney JE, Welser JV, Dechert MA, Flintoff-Dye NL, Kaufman SJ, Burkin DJ. Severe muscular dystrophy in mice that lack dystrophin and alpha7 integrin. J Cell Sci 119: 2185–2195, 2006.16684813Ruegg UT. Pharmacological prospects in the treatment of Duchenne muscular dystrophy. Curr Opin Neurol 26: 577–584, 2013.23995279Russell MA, Lund LM, Haber R, McKeegan K, Cianciola N, Bond M. The intermediate filament protein, synemin, is an AKAP in the heart. Arch Biochem Biophys 456: 204–215, 2006.16934740Rybakova IN, Ervasti JM. Dystrophin-glycoprotein complex is monomeric and stabilizes actin filaments in vitro through a lateral association. J Biol Chem 272: 28771–28778, 1997.9353348Sabourin J, Cognard C, Constantin B. Regulation by scaffolding proteins of canonical transient receptor potential channels in striated muscle. J Muscle Res Cell Motil 30: 289–297, 2009.20195709Sabourin J, Lamiche C, Vandebrouck A, Magaud C, Rivet J, Cognard C, Bourmeyster N, Constantin B. Regulation of TRPC1 and TRPC4 cation channels requires an alpha1-syntrophin-dependent complex in skeletal mouse myotubes. J Biol Chem 284: 36248–36261, 2009.PMC279474119812031Sacco A, Mourkioti F, Tran R, Choi J, Llewellyn M, Kraft P, Shkreli M, Delp S, Pomerantz JH, Artandi SE, Blau HM. Short telomeres and stem cell exhaustion model Duchenne muscular dystrophy in mdx/mTR mice. Cell 143: 1059–1071, 2010.PMC302560821145579Sakellariou GK, Vasilaki A, Palomero J, Kayani A, Zibrik L, McArdle A, Jackson MJ. Studies of mitochondrial and nonmitochondrial sources implicate nicotinamide adenine dinucleotide phosphate oxidase(s) in the increased skeletal muscle superoxide generation that occurs during contractile activity. Antioxid Redox Signal 18: 603–621, 2013.PMC354921223050834Salanova M, Schiffl G, Rittweger J, Felsenberg D, Blottner D. Ryanodine receptor type-1 (RyR1) expression and protein S-nitrosylation pattern in human soleus myofibres following bed rest and exercise countermeasure. Histochem Cell Biol 130: 105–118, 2008.18283481Salminen A, Kainulainen H, Arstila AU, Vihko V. Vitamin E deficiency and the susceptibility to lipid peroxidation of mouse cardiac and skeletal muscles. Acta Physiol Scand 122: 565–570, 1984.6524397Saltin B. Exercise hyperaemia: magnitude and aspects on regulation in humans. J Physiol 583: 819–823, 2007.PMC227719717640931Samuni Y, Goldstein S, Dean OM, Berk M. The chemistry and biological activities of N-acetylcysteine. Biochim Biophys Acta 1830: 4117–4129, 2013.23618697Sander M, Chavoshan B, Harris SA, Iannaccone ST, Stull JT, Thomas GD, Victor RG. Functional muscle ischemia in neuronal nitric oxide synthase-deficient skeletal muscle of children with Duchenne muscular dystrophy. Proc Natl Acad Sci USA 97: 13818–13823, 2000.PMC1765911087833Sandona D, Betto R. Sarcoglycanopathies: molecular pathogenesis and therapeutic prospects. Expert Rev Mol Med 11: e28, 2009.PMC327995619781108Sato O, Nonomura Y, Kimura S, Maruyama K. Molecular shape of dystrophin. J Biochem 112: 631–636, 1992.1478922Sato Y, Sagami I, Shimizu T. Identification of caveolin-1-interacting sites in neuronal nitric-oxide synthase. Molecular mechanism for inhibition of NO formation. J Biol Chem 279: 8827–8836, 2004.14681230Schessl J, Zou Y, Bonnemann CG. Congenital muscular dystrophies and the extracellular matrix. Semin Pediatr Neurol 13: 80–89, 2006.17027857Schlormann W, Steiniger F, Richter W, Kaufmann R, Hause G, Lemke C, Westermann M. The shape of caveolae is omega-like after glutaraldehyde fixation and cup-like after cryofixation. Histochem Cell Biol 133: 223–228, 2010.19851779Schmidt N, Akaaboune M, Gajendran N, Martinez P, Wakefield S, Thurnheer R, Brenner HR. Neuregulin/ErbB regulate neuromuscular junction development by phosphorylation of alpha-dystrobrevin. J Cell Biol 195: 1171–1184, 2011.PMC324689722184199Schubert S, Knoch KP, Ouwendijk J, Mohammed S, Bodrov Y, Jager M, Altkruger A, Wegbrod C, Adams ME, Kim Y, Froehner SC, Jensen ON, Kalaidzidis Y, Solimena M. Beta2-Syntrophin is a Cdk5 substrate that restrains the motility of insulin secretory granules. PLoS One 5: e12929, 2010.PMC294484920886068Segawa M, Fukada S, Yamamoto Y, Yahagi H, Kanematsu M, Sato M, Ito T, Uezumi A, Hayashi S, Miyagoe-Suzuki Y, Takeda S, Tsujikawa K, Yamamoto H. Suppression of macrophage functions impairs skeletal muscle regeneration with severe fibrosis. Exp Cell Res 314: 3232–3244, 2008.18775697Selsby J, Pendrak K, Zadel M, Tian Z, Pham J, Carver T, Acosta P, Barton E, Sweeney HL. Leupeptin-based inhibitors do not improve the mdx phenotype. Am J Physiol Regul Integr Comp Physiol 299: R1192–R1201, 2010.PMC377425220844259Selsby JT. Increased catalase expression improves muscle function in mdx mice. Exp Physiol 96: 194–202, 2011.PMC451983021041317Serrander L, Cartier L, Bedard K, Banfi B, Lardy B, Plastre O, Sienkiewicz A, Forro L, Schlegel W, Krause KH. NOX4 activity is determined by mRNA levels and reveals a unique pattern of ROS generation. Biochem J 406: 105–114, 2007.PMC194899017501721Serrano AL, Mann CJ, Vidal B, Ardite E, Perdiguero E, Munoz-Canoves P. Cellular and molecular mechanisms regulating fibrosis in skeletal muscle repair and disease. Curr Top Dev Biol 96: 167–201, 2011.21621071Serrano AL, Munoz-Canoves P. Regulation and dysregulation of fibrosis in skeletal muscle. Exp Cell Res 316: 3050–3058, 2010.20570674Servitja JM, Marinissen MJ, Sodhi A, Bustelo XR, Gutkind JS. Rac1 function is required for Src-induced transformation. Evidence of a role for Tiam1 and Vav2 in Rac activation by Src. J Biol Chem 278: 34339–34346, 2003.12810717Sevanian A, Kim E. Phospholipase A2 dependent release of fatty acids from peroxidized membranes. J Free Radic Biol Med 1: 263–271, 1985.3836246Sevanian A, Wratten ML, McLeod LL, Kim E. Lipid peroxidation and phospholipase A2 activity in liposomes composed of unsaturated phospholipids: a structural basis for enzyme activation. Biochim Biophys Acta 961: 316–327, 1988.3401498Shkryl VM, Martins AS, Ullrich ND, Nowycky MC, Niggli E, Shirokova N. Reciprocal amplification of ROS and Ca2+ signals in stressed mdx dystrophic skeletal muscle fibers. Pflügers Arch 458: 915–928, 2009.19387681Silvagno F, Xia H, Bredt DS. Neuronal nitric-oxide synthase-mu, an alternatively spliced isoform expressed in differentiated skeletal muscle. J Biol Chem 271: 11204–11208, 1996.8626668Song KS, Scherer PE, Tang Z, Okamoto T, Li S, Chafel M, Chu C, Kohtz DS, Lisanti MP. Expression of caveolin-3 in skeletal, cardiac, and smooth muscle cells. Caveolin-3 is a component of the sarcolemma and co-fractionates with dystrophin and dystrophin-associated glycoproteins. J Biol Chem 271: 15160–15165, 1996.8663016Sonnemann KJ, Fitzsimons DP, Patel JR, Liu Y, Schneider MF, Moss RL, Ervasti JM. Cytoplasmic gamma-actin is not required for skeletal muscle development but its absence leads to a progressive myopathy. Dev Cell 11: 387–397, 2006.16950128Sorimachi H, Ono Y, Suzuki K. Skeletal muscle-specific calpain, p94, and connectin/titin: their physiological functions and relationship to limb-girdle muscular dystrophy type 2A. Adv Exp Med Biol 481: 383–395, 2000.10987085Sotgia F, Bonuccelli G, Bedford M, Brancaccio A, Mayer U, Wilson MT, Campos-Gonzalez R, Brooks JW, Sudol M, Lisanti MP. Localization of phospho-beta-dystroglycan (pY892) to an intracellular vesicular compartment in cultured cells and skeletal muscle fibers in vivo. Biochemistry 42: 7110–7123, 2003.12795607Sotgia F, Lee H, Bedford MT, Petrucci T, Sudol M, Lisanti MP. Tyrosine phosphorylation of beta-dystroglycan at its WW domain binding motif, PPxY, recruits SH2 domain containing proteins. Biochemistry 40: 14585–14592, 2001.11724572Sotgia F, Lee JK, Das K, Bedford M, Petrucci TC, Macioce P, Sargiacomo M, Bricarelli FD, Minetti C, Sudol M, Lisanti MP. Caveolin-3 directly interacts with the C-terminal tail of beta-dystroglycan. Identification of a central WW-like domain within caveolin family members. J Biol Chem 275: 38048–38058, 2000.10988290Spencer MJ, Mellgren RL. Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology. Hum Mol Genet 11: 2645–2655, 2002.12354790Spencer MJ, Tidball JG. Calpain concentration is elevated although net calcium-dependent proteolysis is suppressed in dystrophin-deficient muscle. Exp Cell Res 203: 107–114, 1992.1426033Spurney C, Shimizu R, Hache LP, Kolski H, Gordish-Dressman H, Clemens PR. CINRG Duchenne Natural History Study demonstrates insufficient diagnosis and treatment of cardiomyopathy in Duchenne muscular dystrophy. Muscle Nerve 50: 250–256, 2014.PMC408152324395289Squire S, Raymackers JM, Vandebrouck C, Potter A, Tinsley J, Fisher R, Gillis JM, Davies KE. Prevention of pathology in mdx mice by expression of utrophin: analysis using an inducible transgenic expression system. Hum Mol Genet 11: 3333–3344, 2002.12471059St Pierre J, Buckingham JA, Roebuck SJ, Brand MD. Topology of superoxide production from different sites in the mitochondrial electron transport chain. J Biol Chem 277: 44784–44790, 2002.12237311Stamler JS, Meissner G. Physiology of nitric oxide in skeletal muscle. Physiol Rev 81: 209–237, 2001.11152758Stedman HH, Sweeney HL, Shrager JB, Maguire HC, Panettieri RA, Petrof B, Narusawa M, Leferovich JM, Sladky JT, Kelly AM. The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy. Nature 352: 536–539, 1991.1865908Steinberger M, Foller M, Vogelgesang S, Krautwald M, Landsberger M, Winkler CK, Kasch J, Fuchtbauer EM, Kuhl D, Voelkl J, Lang F, Brinkmeier H. Lack of the serum- and glucocorticoid-inducible kinase SGK1 improves muscle force characteristics and attenuates fibrosis in dystrophic mdx mouse muscle. Pflügers Arch 467: 1965–1974, 2015.25394886Stern LZ, Ringel SP, Ziter FA, Menander-Huber KB, Ionasescu V, Pellegrino RJ, Snyder RD. Drug trial of superoxide dismutase in Duchenne's muscular dystrophy. Arch Neurol 39: 342–346, 1982.7046702Stiber JA, Rosenberg PB. The role of store-operated calcium influx in skeletal muscle signaling. Cell Calcium 49: 341–349, 2011.PMC371421121176846Stiber JA, Zhang ZS, Burch J, Eu JP, Zhang S, Truskey GA, Seth M, Yamaguchi N, Meissner G, Shah R, Worley PF, Williams RS, Rosenberg PB. Mice lacking Homer 1 exhibit a skeletal myopathy characterized by abnormal transient receptor potential channel activity. Mol Cell Biol 28: 2637–2647, 2008.PMC229311618268005Stone MR, O'Neill A, Catino D, Bloch RJ. Specific interaction of the actin-binding domain of dystrophin with intermediate filaments containing keratin 19. Mol Biol Cell 16: 4280–4293, 2005.PMC119633716000376Stone MR, O'Neill A, Lovering RM, Strong J, Resneck WG, Reed PW, Toivola DM, Ursitti JA, Omary MB, Bloch RJ. Absence of keratin 19 in mice causes skeletal myopathy with mitochondrial and sarcolemmal reorganization. J Cell Sci 120: 3999–4008, 2007.PMC920244417971417Street SF. Lateral transmission of tension in frog myofibers: a myofibrillar network and transverse cytoskeletal connections are possible transmitters. J Cell Physiol 114: 346–364, 1983.6601109Stroissnigg H, Trancikova A, Descovich L, Fuhrmann J, Kutschera W, Kostan J, Meixner A, Nothias F, Propst F. S-nitrosylation of microtubule-associated protein 1B mediates nitric-oxide-induced axon retraction. Nat Cell Biol 9: 1035–1045, 2007.17704770Suchyna TM, Sachs F. Mechanosensitive channel properties and membrane mechanics in mouse dystrophic myotubes. J Physiol 581: 369–387, 2007.PMC207520817255168Suhr F, Gehlert S, Grau M, Bloch W. Skeletal muscle function during exercise-fine-tuning of diverse subsystems by nitric oxide. Int J Mol Sci 14: 7109–7139, 2013.PMC364567923538841Sun QA, Hess DT, Nogueira L, Yong S, Bowles DE, Eu J, Laurita KR, Meissner G, Stamler JS. Oxygen-coupled redox regulation of the skeletal muscle ryanodine receptor-Ca2+ release channel by NADPH oxidase 4. Proc Natl Acad Sci USA 108: 16098–16103, 2011.PMC317912721896730Suzuki A, Yoshida M, Ozawa E. Mammalian alpha 1- and beta 1-syntrophin bind to the alternative splice-prone region of the dystrophin COOH terminus. J Cell Biol 128: 373–381, 1995.PMC21203477844151Suzuki N, Motohashi N, Uezumi A, Fukada S, Yoshimura T, Itoyama Y, Aoki M, Miyagoe-Suzuki Y, Takeda S. NO production results in suspension-induced muscle atrophy through dislocation of neuronal NOS. J Clin Invest 117: 2468–2476, 2007.PMC195262217786240Swiderski K, Shaffer SA, Gallis B, Odom GL, Arnett AL, Scott EJ, Baum DM, Chee A, Naim T, Gregorevic P, Murphy KT, Moody J, Goodlett DR, Lynch GS, Chamberlain JS. Phosphorylation within the cysteine-rich region of dystrophin enhances its association with beta-dystroglycan and identifies a potential novel therapeutic target for skeletal muscle wasting. Hum Mol Genet 23: 6697–6711, 2014.PMC424504025082828Tahallah N, Brunelle A, De La Porte S, Laprevote O. Lipid mapping in human dystrophic muscle by cluster-time-of-flight secondary ion mass spectrometry imaging. J Lipid Res 49: 438–454, 2008.PMC243827618025000Takagi A, Kojima S, Ida M, Araki M. Increased leakage of calcium ion from the sarcoplasmic reticulum of the mdx mouse. J Neurol Sci 110: 160–164, 1992.1506855Takahashi J, Itoh Y, Fujimori K, Imamura M, Wakayama Y, Miyagoe-Suzuki Y, Takeda S. The utrophin promoter A drives high expression of the transgenic LacZ gene in liver, testis, colon, submandibular gland, and small intestine. J Gene Med 7: 237–248, 2005.15538725Thomas GD, Sander M, Lau KS, Huang PL, Stull JT, Victor RG. Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscle. Proc Natl Acad Sci USA 95: 15090–15095, 1998.PMC245809844020Thomas GD, Shaul PW, Yuhanna IS, Froehner SC, Adams ME. Vasomodulation by skeletal muscle-derived nitric oxide requires alpha-syntrophin-mediated sarcolemmal localization of neuronal nitric oxide synthase. Circ Res 92: 554–560, 2003.12600881Thomas GD, Victor RG. Nitric oxide mediates contraction-induced attenuation of sympathetic vasoconstriction in rat skeletal muscle. J Physiol 506: 817–826, 1998.PMC22307499503340Thompson O, Kleino I, Crimaldi L, Gimona M, Saksela K, Winder SJ. Dystroglycan, Tks5 and Src mediated assembly of podosomes in myoblasts. PLoS One 3: e3638, 2008.PMC257284018982058Thompson TG, Chan YM, Hack AA, Brosius M, Rajala M, Lidov HG, McNally EM, Watkins S, Kunkel LM. Filamin 2 (FLN2): a muscle-specific sarcoglycan interacting protein. J Cell Biol 148: 115–126, 2000.PMC320714210629222Tidball JG, Wehling-Henricks M. Expression of a NOS transgene in dystrophin-deficient muscle reduces muscle membrane damage without increasing the expression of membrane-associated cytoskeletal proteins. Mol Genet Metab 82: 312–320, 2004.15308129Tinsley J, Deconinck N, Fisher R, Kahn D, Phelps S, Gillis JM, Davies K. Expression of full-length utrophin prevents muscular dystrophy in mdx mice. Nat Med 4: 1441–1444, 1998.9846586Tinsley JM, Fairclough RJ, Storer R, Wilkes FJ, Potter AC, Squire SE, Powell DS, Cozzoli A, Capogrosso RF, Lambert A, Wilson FX, Wren SP, De LA, Davies KE. Daily treatment with SMTC1100, a novel small molecule utrophin upregulator, dramatically reduces the dystrophic symptoms in the mdx mouse. PLoS One 6: e19189, 2011.PMC308959821573153Tochio H, Ohki S, Zhang Q, Li M, Zhang M. Solution structure of a protein inhibitor of neuronal nitric oxide synthase. Nat Struct Biol 5: 965–969, 1998.9808041Tomasi LG. Reversibility of human myopathy caused by vitamin E deficiency. Neurology 29: 1182–1186, 1979.572510Tonon E, Ferretti R, Shiratori JH, Santo NH, Marques MJ, Minatel E. Ascorbic acid protects the diaphragm muscle against myonecrosis in mdx mice. Nutrition 28: 686–690, 2012.22189191Torelli S, Brown SC, Jimenez-Mallebrera C, Feng L, Muntoni F, Sewry CA. Absence of neuronal nitric oxide synthase (nNOS) as a pathological marker for the diagnosis of Becker muscular dystrophy with rod domain deletions. Neuropathol Appl Neurobiol 30: 540–545, 2004.15488030Touyz RM, Yao G, Schiffrin EL. c-Src induces phosphorylation and translocation of p47phox: role in superoxide generation by angiotensin II in human vascular smooth muscle cells. Arterioscler Thromb Vasc Biol 23: 981–987, 2003.12663375Townsend D, Yasuda S, Li S, Chamberlain JS, Metzger JM. Emergent dilated cardiomyopathy caused by targeted repair of dystrophic skeletal muscle. Mol Ther 16: 832–835, 2008.PMC259284618414480Turgeman T, Hagai Y, Huebner K, Jassal DS, Anderson JE, Genin O, Nagler A, Halevy O, Pines M. Prevention of muscle fibrosis and improvement in muscle performance in the mdx mouse by halofuginone. Neuromuscular Disorders 18: 857–868, 2008.18672370Turner PR, Westwood T, Regen CM, Steinhardt RA. Increased protein degradation results from elevated free calcium levels found in muscle from mdx mice. Nature 335: 735–738, 1988.3173492Tutdibi O, Brinkmeier H, Rudel R, Fohr KJ. Increased calcium entry into dystrophin-deficient muscle fibres of MDX and ADR-MDX mice is reduced by ion channel blockers. J Physiol 515: 859–868, 1999.PMC226918910066910Ueda K, Valdivia C, Medeiros-Domingo A, Tester DJ, Vatta M, Farrugia G, Ackerman MJ, Makielski JC. Syntrophin mutation associated with long QT syndrome through activation of the nNOS-SCN5A macromolecular complex. Proc Natl Acad Sci USA 105: 9355–9360, 2008.PMC244212718591664Vaghy PL, Fang J, Wu W, Vaghy LP. Increased caveolin-3 levels in mdx mouse muscles. FEBS Lett 431: 125–127, 1998.9684879Valentine BA, Cooper BJ, de LA, O'Quinn R, Blue JT. Canine X-linked muscular dystrophy. An animal model of Duchenne muscular dystrophy: clinical studies. J Neurol Sci 88: 69–81, 1988.3225630Valko M, Leibfritz D, Moncol J, Cronin MT, Mazur M, Telser J. Free radicals and antioxidants in normal physiological functions and human disease. Int J Biochem Cell Biol 39: 44–84, 2007.16978905Vallejo-Illarramendi A, Toral-Ojeda I, Aldanondo G, Lopez de MA. Dysregulation of calcium homeostasis in muscular dystrophies. Expert Rev Mol Med 16: e16, 2014.25293420Vandebrouck A, Sabourin J, Rivet J, Balghi H, Sebille S, Kitzis A, Raymond G, Cognard C, Bourmeyster N, Constantin B. Regulation of capacitative calcium entries by alpha1-syntrophin: association of TRPC1 with dystrophin complex and the PDZ domain of alpha1-syntrophin. FASEB J 21: 608–617, 2007.17202249Vandebrouck C, Duport G, Cognard C, Raymond G. Cationic channels in normal and dystrophic human myotubes. Neuromuscul Disord 11: 72–79, 2001.11166168Vandebrouck C, Martin D, Colson-Van Schoor M, Debaix H, Gailly P. Involvement of TRPC in the abnormal calcium influx observed in dystrophic (mdx) mouse skeletal muscle fibers. J Cell Biol 158: 1089–1096, 2002.PMC217322512235126Venema VJ, Ju H, Zou R, Venema RC. Interaction of neuronal nitric-oxide synthase with caveolin-3 in skeletal muscle. Identification of a novel caveolin scaffolding/inhibitory domain. J Biol Chem 272: 28187–28190, 1997.9353265Verburg E, Murphy RM, Richard I, Lamb GD. Involvement of calpains in Ca2+-induced disruption of excitation-contraction coupling in mammalian skeletal muscle fibers. Am J Physiol Cell Physiol 296: C1115–C1122, 2009.19295178Vetrone SA, Montecino-Rodriguez E, Kudryashova E, Kramerova I, Hoffman EP, Liu SD, Miceli MC, Spencer MJ. Osteopontin promotes fibrosis in dystrophic mouse muscle by modulating immune cell subsets and intramuscular TGF-beta. J Clin Invest 119: 1583–1594, 2009.PMC268911219451692Vidal B, Ardite E, Suelves M, Ruiz-Bonilla V, Janue A, Flick MJ, Degen JL, Serrano AL, Munoz-Canoves P. Amelioration of Duchenne muscular dystrophy in mdx mice by elimination of matrix-associated fibrin-driven inflammation coupled to the alphaMbeta2 leukocyte integrin receptor. Hum Mol Genet 21: 1989–2004, 2012.PMC331520622381526Vidal B, Serrano AL, Tjwa M, Suelves M, Ardite E, De MR, Baeza-Raja B, Martinez de LM, Lafuste P, Ruiz-Bonilla V, Jardi M, Gherardi R, Christov C, Dierssen M, Carmeliet P, Degen JL, Dewerchin M, Munoz-Canoves P. Fibrinogen drives dystrophic muscle fibrosis via a TGFbeta/alternative macrophage activation pathway. Genes Dev 22: 1747–1752, 2008.PMC249266118593877Vilhardt F, van Deurs B. The phagocyte NADPH oxidase depends on cholesterol-enriched membrane microdomains for assembly. EMBO J 23: 739–748, 2004.PMC38099014765128Voet NB, Geurts AC, Bleijenberg G, Zwarts MJ, Padberg GW, Van Engelen BG. Muscle fatigue in muscular dystophies. In: Human Muscle Fatigue, edited by Williams CA, Ratel S. London: Routledge, 2009.Vorgerd M, Karitzky J, Ristow M, Van SE, Tegenthoff M, Jerusalem F, Malin JP. Muscle phosphofructokinase deficiency in two generations. J Neurol Sci 141: 95–99, 1996.8880699Wagner KR, Cohen JB, Huganir RL. The 87K postsynaptic membrane protein from Torpedo is a protein-tyrosine kinase substrate homologous to dystrophin. Neuron 10: 511–522, 1993.8461138Wagner KR, Huganir RL. Tyrosine and serine phosphorylation of dystrophin and the 58-kDa protein in the postsynaptic membrane of Torpedo electric organ. J Neurochem 62: 1947–1952, 1994.7512621Wakayama Y, Shibuya S, Jimi T, Takeda A, Oniki H. Size and localization of dystrophin molecule: immunoelectron microscopic and freeze etching studies of muscle plasma membranes of murine skeletal myofibers. Acta Neuropathol 86: 567–577, 1993.8310812Wallace GQ, McNally EM. Mechanisms of muscle degeneration, regeneration, and repair in the muscular dystrophies. Annu Rev Physiol 71: 37–57, 2009.18808326Wang X, Weisleder N, Collet C, Zhou J, Chu Y, Hirata Y, Zhao X, Pan Z, Brotto M, Cheng H, Ma J. Uncontrolled calcium sparks act as a dystrophic signal for mammalian skeletal muscle. Nat Cell Biol 7: 525–530, 2005.15834406Watchko J, O'Day T, Wang B, Zhou L, Tang Y, Li J, Xiao X. Adeno-associated virus vector-mediated minidystrophin gene therapy improves dystrophic muscle contractile function in mdx mice. Hum Gene Ther 13: 1451–1460, 2002.12215266Watchko JF, O'Day TL, Hoffman EP. Functional characteristics of dystrophic skeletal muscle: insights from animal models. J Appl Physiol 93: 407–417, 2002.12133845Watkins SC, Cullen MJ. Histochemical fibre typing and ultrastructure of the small fibres in Duchenne muscular dystrophy. Neuropathol Appl Neurobiol 11: 447–460, 1985.2936970Watkins SC, Cullen MJ. A quantitative comparison of satellite cell ultrastructure in Duchenne muscular dystrophy, polymyositis, and normal controls. Muscle Nerve 9: 724–730, 1986.3785283Watkins SC, Hoffman EP, Slayter HS, Kunkel LM. Immunoelectron microscopic localization of dystrophin in myofibres. Nature 333: 863–866, 1988.3290684Webster C, Blau HM. Accelerated age-related decline in replicative life-span of Duchenne muscular dystrophy myoblasts: implications for cell and gene therapy. Somat Cell Mol Genet 16: 557–565, 1990.2267630Webster C, Silberstein L, Hays AP, Blau HM. Fast muscle fibers are preferentially affected in Duchenne muscular dystrophy. Cell 52: 503–513, 1988.3342447Wehling M, Spencer MJ, Tidball JG. A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice. J Cell Biol 155: 123–131, 2001.PMC215080011581289Wehling-Henricks M, Oltmann M, Rinaldi C, Myung KH, Tidball JG. Loss of positive allosteric interactions between neuronal nitric oxide synthase and phosphofructokinase contributes to defects in glycolysis and increased fatigability in muscular dystrophy. Hum Mol Genet 18: 3439–3451, 2009.PMC272966619542095Wehling-Henricks M, Tidball JG. Neuronal nitric oxide synthase-rescue of dystrophin/utrophin double knockout mice does not require nNOS localization to the cell membrane. PLoS ONE 6: e25071, 2011.PMC318917722003386Weisleder N, Takizawa N, Lin P, Wang X, Cao C, Zhang Y, Tan T, Ferrante C, Zhu H, Chen PJ, Yan R, Sterling M, Zhao X, Hwang M, Takeshima M, Cai C, Cheng H, Takeshima H, Xiao RP, Ma J. Recombinant MG53 protein modulates therapeutic cell membrane repair in treatment of muscular dystrophy. Sci Transl Med 4: 139ra85, 2012.PMC377762322723464Welch EM, Barton ER, Zhuo J, Tomizawa Y, Friesen WJ, Trifillis P, Paushkin S, Patel M, Trotta CR, Hwang S, Wilde RG, Karp G, Takasugi J, Chen G, Jones S, Ren H, Moon YC, Corson D, Turpoff AA, Campbell JA, Conn MM, Khan A, Almstead NG, Hedrick J, Mollin A, Risher N, Weetall M, Yeh S, Branstrom AA, Colacino JM, Babiak J, Ju WD, Hirawat S, Northcutt VJ, Miller LL, Spatrick P, He F, Kawana M, Feng H, Jacobson A, Peltz SW, Sweeney HL. PTC124 targets genetic disorders caused by nonsense mutations. Nature 447: 87–91, 2007.17450125Wells KE, Torelli S, Lu Q, Brown SC, Partridge T, Muntoni F, Wells DJ. Relocalization of neuronal nitric oxide synthase (nNOS) as a marker for complete restoration of the dystrophin associated protein complex in skeletal muscle. Neuromuscular Disorders 13: 21–31, 2003.12467729Wells L. The o-mannosylation pathway: glycosyltransferases and proteins implicated in congenital muscular dystrophy. J Biol Chem 288: 6930–6935, 2013.PMC359160323329833Westerblad H, Duty S, Allen DG. Intracellular calcium concentration during low-frequency fatigue in isolated single fibers of mouse skeletal muscle. J Appl Physiol 75: 382–388, 1993.8397180White AT, Schenk S. NAD+/NADH and skeletal muscle mitochondrial adaptations to exercise. Am J Physiol Endocrinol Metab 303: E308–E321, 2012.PMC342312322436696Whitehead NP, Pham C, Gervasio OL, Allen DG. N-acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice. J Physiol 586: 2003–2014, 2008.PMC237571718258657Whitehead NP, Streamer M, Lusambili LI, Sachs F, Allen DG. Streptomycin reduces stretch-induced membrane permeability in muscles from mdx mice. Neuromuscular Disorders 16: 845–854, 2006.17005404Whitehead NP, Yeung EW, Allen DG. Muscle damage in mdx (dystrophic) mice: role of calcium and reactive oxygen species. Clin Exp Pharmacol Physiol 33: 657–662, 2006.16789936Whitehead NP, Yeung EW, Froehner SC, Allen DG. Skeletal muscle NADPH oxidase is increased and triggers stretch-induced muscle damage in the mdx mouse. PLoS One 5: e15354, 2010.PMC300486421187957Wickstrom SA, Lange A, Hess MW, Polleux J, Spatz JP, Kruger M, Pfaller K, Lambacher A, Bloch W, Mann M, Huber LA, Fassler R. Integrin-linked kinase controls microtubule dynamics required for plasma membrane targeting of caveolae. Dev Cell 19: 574–588, 2010.PMC297707820951348Williams DA, Head SI, Lynch GS, Stephenson DG. Contractile properties of skinned muscle fibres from young and adult normal and dystrophic (mdx) mice. J Physiol 460: 51–67, 1993.PMC11752008487206Williams IA, Allen DG. The role of reactive oxygen species in the hearts of dystrophin-deficient mdx mice. Am J Physiol Heart Circ Physiol 293: H1969–H1977, 2007.17573457Williams JJ, Palmer TM. Cavin-1: caveolae-dependent signalling and cardiovascular disease. Biochem Soc Trans 42: 284–288, 2014.24646232Williams MW, Bloch RJ. Differential distribution of dystrophin and beta-spectrin at the sarcolemma of fast twitch skeletal muscle fibers. J Muscle Res Cell Motil 20: 383–393, 1999.10531619Winter L, Wiche G. The many faces of plectin and plectinopathies: pathology and mechanisms. Acta Neuropathol 125: 77–93, 2013.22864774Woods CE, Novo D, DiFranco M, Capote J, Vergara JL. Propagation in the transverse tubular system and voltage dependence of calcium release in normal and mdx mouse muscle fibres. J Physiol 568: 867–880, 2005.PMC146416716123111Woods CE, Novo D, DiFranco M, Vergara JL. The action potential-evoked sarcoplasmic reticulum calcium release is impaired in mdx mouse muscle fibres. J Physiol 557: 59–75, 2004.PMC166505215004213Worton RG, Duff C, Sylvester JE, Schmickel RD, Willard HF. Duchenne muscular dystrophy involving translocation of the dmd gene next to ribosomal RNA genes. Science 224: 1447–1449, 1984.6729462Wu G, Ai T, Kim JJ, Mohapatra B, Xi Y, Li Z, Abbasi S, Purevjav E, Samani K, Ackerman MJ, Qi M, Moss AJ, Shimizu W, Towbin JA, Cheng J, Vatta M. Alpha-1-syntrophin mutation and the long-QT syndrome: a disease of sodium channel disruption. Circ Arrhythm Electrophysiol 1: 193–201, 2008.PMC272671719684871Xiong Y, Zhou Y, Jarrett HW. Dystrophin glycoprotein complex-associated Gbetagamma subunits activate phosphatidylinositol-3-kinase/Akt signaling in skeletal muscle in a laminin-dependent manner. J Cell Physiol 219: 402–414, 2009.PMC282087519117013Yamada T, Fedotovskaya O, Cheng AJ, Cornachione AS, Minozzo FC, Aulin C, Friden C, Turesson C, Andersson DC, Glenmark B, Lundberg IE, Rassier DE, Westerblad H, Lanner JT. Nitrosative modifications of the Ca2+ release complex and actin underlie arthritis-induced muscle weakness. Ann Rheum Dis 74: 1907–1914, 2015.PMC460226224854355Yamashita K, Suzuki A, Satoh Y, Ide M, Amano Y, Masuda-Hirata M, Hayashi YK, Hamada K, Ogata K, Ohno S. The 8th and 9th tandem spectrin-like repeats of utrophin cooperatively form a functional unit to interact with polarity-regulating kinase PAR-1b. Biochem Biophys Res Commun 391: 812–817, 2010.19945424Yang B, Rizzo V. TNF-alpha potentiates protein-tyrosine nitration through activation of NADPH oxidase and eNOS localized in membrane rafts and caveolae of bovine aortic endothelial cells. Am J Physiol Heart Circ Physiol 292: H954–H962, 2007.17028163Yano R, Yap CC, Yamazaki Y, Muto Y, Kishida H, Okada D, Hashikawa T. Sast124, a novel splice variant of syntrophin-associated serine/threonine kinase (SAST), is specifically localized in the restricted brain regions. Neuroscience 117: 373–381, 2003.12614677Yeung EW, Whitehead NP, Suchyna TM, Gottlieb PA, Sachs F, Allen DG. Effects of stretch-activated channel blockers on [Ca2+]i and muscle damage in the mdx mouse. J Physiol 562: 367–380, 2005.PMC166549915528244Yoshida M, Hama H, Ishikawa-Sakurai M, Imamura M, Mizuno Y, Araishi K, Wakabayashi-Takai E, Noguchi S, Sasaoka T, Ozawa E. Biochemical evidence for association of dystrobrevin with the sarcoglycan-sarcospan complex as a basis for understanding sarcoglycanopathy. Hum Mol Genet 9: 1033–1040, 2000.10767327Young MF, Fallon JR. Biglycan: a promising new therapeutic for neuromuscular and musculoskeletal diseases. Curr Opin Genet Dev 22: 398–400, 2012.22841370Yuan JP, Kiselyov K, Shin DM, Chen J, Shcheynikov N, Kang SH, Dehoff MH, Schwarz MK, Seeburg PH, Muallem S, Worley PF. Homer binds TRPC family channels and is required for gating of TRPC1 by IP3 receptors. Cell 114: 777–789, 2003.14505576Yun BW, Feechan A, Yin M, Saidi NB, Le BT, Yu M, Moore JW, Kang JG, Kwon E, Spoel SH, Pallas JA, Loake GJ. S-nitrosylation of NADPH oxidase regulates cell death in plant immunity. Nature 478: 264–268, 2011.21964330Yurchenco PD. Basement membranes: cell scaffoldings and signaling platforms. Cold Spring Harb Perspect Biol 3: 2011.PMC303952821421915Zanou N, Shapovalov G, Louis M, Tajeddine N, Gallo C, Van SM, Anguish I, Cao ML, Schakman O, Dietrich A, Lebacq J, Ruegg U, Roulet E, Birnbaumer L, Gailly P. Role of TRPC1 channel in skeletal muscle function. Am J Physiol Cell Physiol 298: C149–C162, 2010.PMC280615719846750Zatz M, Betti RT, Levy JA. Benign Duchenne muscular dystrophy in a patient with growth hormone deficiency. Am J Med Genet 10: 301–304, 1981.7304674Zatz M, Rapaport D, Vainzof M, Passos-Bueno MR, Bortolini ER, Pavanello RC, Peres CA. Serum creatine-kinase (CK) and pyruvate-kinase (PK) activities in Duchenne (DMD) as compared with Becker (BMD) muscular dystrophy. J Neurol Sci 102: 190–196, 1991.2072118Zatz M, Rapaport D, Vainzof M, Rocha JM, Pavanello RC, Colletto GM, Peres CA. Relation between height and clinical course in Duchenne muscular dystrophy. Am J Med Genet 29: 405–410, 1988.3354613Zhan Y, Tremblay MR, Melian N, Carbonetto S. Evidence that dystroglycan is associated with dynamin and regulates endocytosis. J Biol Chem 280: 18015–18024, 2005.15728588Zhang BT, Whitehead NP, Gervasio OL, Reardon TF, Vale M, Fatkin D, Dietrich A, Yeung EW, Allen DG. Pathways of Ca2+ entry and cytoskeletal damage following eccentric contractions in mouse skeletal muscle. J Appl Physiol 112: 2077–2086, 2012.PMC337839222461447Zhao X, Moloughney JG, Zhang S, Komazaki S, Weisleder N. Orai1 mediates exacerbated Ca2+ entry in dystrophic skeletal muscle. PLoS One 7: e49862, 2012.PMC350146023185465Zhou L, Lu H. Targeting fibrosis in Duchenne muscular dystrophy. J Neuropathol Exp Neurol 69: 771–776, 2010.PMC291696820613637Zhou YW, Thomason DB, Gullberg D, Jarrett HW. Binding of laminin alpha1-chain LG4-5 domain to alpha-dystroglycan causes tyrosine phosphorylation of syntrophin to initiate Rac1 signaling. Biochemistry 45: 2042–2052, 2006.16475793Zhuang W, Eby JC, Cheong M, Mohapatra PK, Bredt DS, Disatnik MH, Rando TA. The susceptibility of muscle cells to oxidative stress is independent of nitric oxide synthase expression. Muscle Nerve 24: 502–511, 2001.11268022