utrophin

Summary

Summary: An autosomally-encoded 376-kDa cytoskeletal protein that is similar in structure and function to DYSTROPHIN. It is a ubiquitously-expressed protein that plays a role in anchoring the CYTOSKELETON to the PLASMA MEMBRANE.

Top Publications

  1. Hainsey T, Senapati S, Kuhn D, Rafael J. Cardiomyopathic features associated with muscular dystrophy are independent of dystrophin absence in cardiovasculature. Neuromuscul Disord. 2003;13:294-302 pubmed
    ..We show here that the cardiomyopathic features of the utrophin/dystrophin-deficient mouse can be prevented by the presence of dystrophin in cardiomyocytes but not in ..
  2. Delfin D, Xu Y, Peterson J, Guttridge D, Rafael Fortney J, Janssen P. Improvement of cardiac contractile function by peptide-based inhibition of NF-?B in the utrophin/dystrophin-deficient murine model of muscular dystrophy. J Transl Med. 2011;9:68 pubmed publisher
    ..physiological and histological parameters of cardiac muscle in mice deficient for both dystrophin and its homolog utrophin (double knockout = dko) mice treated with NBD peptide...
  3. Pozzoli U, Sironi M, Cagliani R, Comi G, Bardoni A, Bresolin N. Comparative analysis of the human dystrophin and utrophin gene structures. Genetics. 2002;160:793-8 pubmed
    ..Out-of-frame rod-domain exons have stronger splice sites and are separated by significantly longer introns as compared to in-frame exons. These features are unique for the two homologs and not shared by other spectrin superfamily genes. ..
  4. Fisher I, Abraham D, Bouri K, Hoffmann E, Hoffman E, Muntoni F, et al. Prednisolone-induced changes in dystrophic skeletal muscle. FASEB J. 2005;19:834-6 pubmed
    ..Treatment did not increase muscle regeneration, reduce the number of infiltrating macrophages, or alter utrophin expression or localization...
  5. Gramolini A, Burton E, Tinsley J, Ferns M, Cartaud A, Cartaud J, et al. Muscle and neural isoforms of agrin increase utrophin expression in cultured myotubes via a transcriptional regulatory mechanism. J Biol Chem. 1998;273:736-43 pubmed
    ..Recently, it was demonstrated in a transgenic mouse model that utrophin could functionally compensate for the lack of dystrophin and alleviate the muscle pathology (Tinsley, J. M...
  6. Adams M, Kramarcy N, Krall S, Rossi S, Rotundo R, Sealock R, et al. Absence of alpha-syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin. J Cell Biol. 2000;150:1385-98 pubmed
    ..alpha-Syn(-/)- neuromuscular junctions have undetectable levels of postsynaptic utrophin and reduced levels of acetylcholine receptor and acetylcholinesterase...
  7. Crosbie R, Lebakken C, Holt K, Venzke D, Straub V, Lee J, et al. Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex. J Cell Biol. 1999;145:153-65 pubmed
    ..myotendinous junction (MTJ) and neuromuscular junction (NMJ), where it is a component of both the dystrophin- and utrophin-glycoprotein complexes...
  8. Chakkalakal J, Harrison M, Carbonetto S, Chin E, Michel R, Jasmin B. Stimulation of calcineurin signaling attenuates the dystrophic pathology in mdx mice. Hum Mol Genet. 2004;13:379-88 pubmed
    b>Utrophin has been studied extensively in recent years in an effort to find a cure for Duchenne muscular dystrophy...
  9. Moorwood C, Soni N, Patel G, Wilton S, Khurana T. A cell-based high-throughput screening assay for posttranscriptional utrophin upregulation. J Biomol Screen. 2013;18:400-6 pubmed publisher
    ..b>Utrophin is a homologue of dystrophin that can compensate for its absence when overexpressed in DMD animal models...

Scientific Experts

More Information

Publications62

  1. McCarthy J, Esser K, Andrade F. MicroRNA-206 is overexpressed in the diaphragm but not the hindlimb muscle of mdx mouse. Am J Physiol Cell Physiol. 2007;293:C451-7 pubmed
    ..Previous in vitro analysis found miR-206 was capable of repressing utrophin expression; however, under dystrophic conditions, both utrophin transcript and protein levels were significantly ..
  2. Lomnytska M, Dubrovska A, Hellman U, Volodko N, Souchelnytskyi S. Increased expression of cSHMT, Tbx3 and utrophin in plasma of ovarian and breast cancer patients. Int J Cancer. 2006;118:412-21 pubmed
    ..Truncated forms of cytosolic serine hydroxymethyl transferase (cSHMT), T-box transcription factor 3 (Tbx3) and utrophin were aberrantly expressed in samples from cancer patients as compared to samples from noncancerous cases...
  3. Nguyen H, Jayasinha V, Xia B, Hoyte K, Martin P. Overexpression of the cytotoxic T cell GalNAc transferase in skeletal muscle inhibits muscular dystrophy in mdx mice. Proc Natl Acad Sci U S A. 2002;99:5616-21 pubmed
    ..along the sarcolemmal membrane, but the same proteins remain concentrated at the neuromuscular junction where utrophin, a dystrophin homologue, is expressed [Matsumura, K., Ervasti, J. M., Ohlendieck, K., Kahl, K. D...
  4. Ueda H, Baba T, Terada N, Kato Y, Fujii Y, Takayama I, et al. Immunolocalization of dystrobrevin in the astrocytic endfeet and endothelial cells in the rat cerebellum. Neurosci Lett. 2000;283:121-4 pubmed
    ..Previous studies reported that dystrophin, utrophin, syntrophin and beta-dystroglycan were expressed in the cerebellum...
  5. Cerletti M, Negri T, Cozzi F, Colpo R, Andreetta F, Croci D, et al. Dystrophic phenotype of canine X-linked muscular dystrophy is mitigated by adenovirus-mediated utrophin gene transfer. Gene Ther. 2003;10:750-7 pubmed
    b>Utrophin is highly homologous and structurally similar to dystrophin, and in gene delivery experiments in mdx mice was able to functionally replace dystrophin...
  6. Connolly A, Keeling R, Mehta S, Pestronk A, Sanes J. Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin alpha2-deficient mice. Neuromuscul Disord. 2001;11:703-12 pubmed
    ..mutant mouse strains of muscular dystrophy: mdx mice, which lack dystrophin; mdx:utrn-/- mice, which also lack utrophin; and dy/dy mice, which are deficient in laminin alpha2...
  7. Moghadaszadeh B, Albrechtsen R, Guo L, Zaik M, Kawaguchi N, Borup R, et al. Compensation for dystrophin-deficiency: ADAM12 overexpression in skeletal muscle results in increased alpha 7 integrin, utrophin and associated glycoproteins. Hum Mol Genet. 2003;12:2467-79 pubmed
    ..an approximately 2-fold increase in expression, and distinct extrasynaptic localization, of alpha 7B integrin and utrophin, the functional homolog of dystrophin...
  8. Rodova M, Brownback K, Werle M. Okadaic acid augments utrophin in myogenic cells. Neurosci Lett. 2004;363:163-7 pubmed
    ..b>Utrophin is a paralogue of dystrophin and can functionally replace it in skeletal muscle...
  9. Rybakova I, Ervasti J. Identification of spectrin-like repeats required for high affinity utrophin-actin interaction. J Biol Chem. 2005;280:23018-23 pubmed
    ..However, we recently reported evidence suggesting that spectrin-like repeats of utrophin also participate in binding to actin...
  10. Onori A, Desantis A, Buontempo S, Di Certo M, Fanciulli M, Salvatori L, et al. The artificial 4-zinc-finger protein Bagly binds human utrophin promoter A at the endogenous chromosomal site and activates transcription. Biochem Cell Biol. 2007;85:358-65 pubmed
    Our aim is to upregulate the expression of the dystrophin-related gene utrophin in Duchenne muscular dystrophy, in this way complementing the lack of dystrophin function...
  11. Urasawa N, Wada M, Machida N, Yuasa K, Shimatsu Y, Wakao Y, et al. Selective vacuolar degeneration in dystrophin-deficient canine Purkinje fibers despite preservation of dystrophin-associated proteins with overexpression of Dp71. Circulation. 2008;117:2437-48 pubmed publisher
    ..b>Utrophin was highly upregulated in the earlier stage of CXMD(J) Purkinje fibers, but the expression was dislocated when ..
  12. Nguyen T, Ellis J, Love D, Davies K, Gatter K, Dickson G, et al. Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proli. J Cell Biol. 1991;115:1695-700 pubmed
    ..This contrasts with the low levels of DMDL protein in adult brain tissue. ..
  13. Côté P, Moukhles H, Lindenbaum M, Carbonetto S. Chimaeric mice deficient in dystroglycans develop muscular dystrophy and have disrupted myoneural synapses. Nat Genet. 1999;23:338-42 pubmed
    ..each myofibre and docks with beta-Dg, a transmembrane protein, which in turn interacts with dystrophin or utrophin in the subplasmalemmal cytoskeleton...
  14. Wakefield P, Tinsley J, Wood M, Gilbert R, Karpati G, Davies K. Prevention of the dystrophic phenotype in dystrophin/utrophin-deficient muscle following adenovirus-mediated transfer of a utrophin minigene. Gene Ther. 2000;7:201-4 pubmed
    ..We have previously shown that the dystrophin-related protein, utrophin is able to compensate for the lack of dystrophin in the mdx mouse, the mouse model for DMD...
  15. Gramolini A, Belanger G, Jasmin B. Distinct regions in the 3' untranslated region are responsible for targeting and stabilizing utrophin transcripts in skeletal muscle cells. J Cell Biol. 2001;154:1173-83 pubmed
    In this study, we have sought to determine whether utrophin transcripts are targeted to a distinct subcellular compartment in skeletal muscle cells, and have examined the role of the 3' untranslated region (UTR) in regulating the ..
  16. Marshall J, Chou E, Oh J, Kwok A, Burkin D, Crosbie Watson R. Dystrophin and utrophin expression require sarcospan: loss of ?7 integrin exacerbates a newly discovered muscle phenotype in sarcospan-null mice. Hum Mol Genet. 2012;21:4378-93 pubmed
    Sarcospan (SSPN) is a core component of the major adhesion complexes in skeletal muscle, the dystrophin- and utrophin (Utr)-glycoprotein complexes (DGC and UGC)...
  17. Basu U, Lozynska O, Moorwood C, Patel G, Wilton S, Khurana T. Translational regulation of utrophin by miRNAs. PLoS ONE. 2011;6:e29376 pubmed publisher
    b>Utrophin is the autosomal homolog of dystrophin, the product of the Duchenne Muscular Dystrophy (DMD) locus. Its regulation is of therapeutic interest as its overexpression can compensate for dystrophin's absence in animal models of DMD...
  18. Albesa M, Ogrodnik J, Rougier J, Abriel H. Regulation of the cardiac sodium channel Nav1.5 by utrophin in dystrophin-deficient mice. Cardiovasc Res. 2011;89:320-8 pubmed publisher
    ..Disease progression in the mdx mouse only modestly reflects that of DMD patients, possibly due to utrophin up-regulation...
  19. Miura P, Coriati A, Belanger G, De Repentigny Y, Lee J, Kothary R, et al. The utrophin A 5'-UTR drives cap-independent translation exclusively in skeletal muscles of transgenic mice and interacts with eEF1A2. Hum Mol Genet. 2010;19:1211-20 pubmed publisher
    The molecular mechanisms regulating expression of utrophin A are of therapeutic interest since upregulating its expression at the sarcolemma can compensate for the lack of dystrophin in animal models of Duchenne Muscular Dystrophy (DMD)...
  20. Guo W, Nichol M, Merlie J. Cloning and expression of full length mouse utrophin: the differential association of utrophin and dystrophin with AChR clusters. FEBS Lett. 1996;398:259-64 pubmed
    We have cloned and sequenced mouse utrophin cDNA, and successfully expressed full length utrophin (400 kDa) in both muscle and non-muscle cells...
  21. Voisin V, de la Porte S. Therapeutic strategies for Duchenne and Becker dystrophies. Int Rev Cytol. 2004;240:1-30 pubmed
    ..of three potential goals: improvement of dystrophic phenotype, expression of dystrophin, and overexpression of utrophin. Utrophin exhibits 80% homology with dystrophin and is able to perform similar functions...
  22. Porter J, Rafael J, Ragusa R, Brueckner J, Trickett J, Davies K. The sparing of extraocular muscle in dystrophinopathy is lost in mice lacking utrophin and dystrophin. J Cell Sci. 1998;111 ( Pt 13):1801-11 pubmed
    ..extraocular muscles of mdx mice exhibited increased levels of a dystrophin analog, dystrophin-related protein or utrophin. These data suggest, but do not provide mechanistic evidence, that utrophin mediates eye muscle protection...
  23. Fisher R, Tinsley J, Phelps S, Squire S, Townsend E, Martin J, et al. Non-toxic ubiquitous over-expression of utrophin in the mdx mouse. Neuromuscul Disord. 2001;11:713-21 pubmed
    ..We have previously demonstrated that the dystrophin related protein, utrophin is able to compensate for the loss of dystrophin in the mdx mouse, the mouse model of the disease...
  24. Peters M, O Brien K, Sadoulet Puccio H, Kunkel L, Adams M, Froehner S. beta-dystrobrevin, a new member of the dystrophin family. Identification, cloning, and protein associations. J Biol Chem. 1997;272:31561-9 pubmed
    ..Three dystrophin-related proteins (utrophin, dystrophin-related protein-2 (DRP2), and dystrobrevin) have been described...
  25. Xu R, Chandrasekharan K, Yoon J, Camboni M, Martin P. Overexpression of the cytotoxic T cell (CT) carbohydrate inhibits muscular dystrophy in the dyW mouse model of congenital muscular dystrophy 1A. Am J Pathol. 2007;171:181-99 pubmed
    ..These experiments demonstrate that Galgt2 overexpression is effective in altering disease progression in skeletal muscles of dy(W) mice and should be considered as a therapeutic target in MDC1A. ..
  26. Sewry C, Sansome A, Matsumura K, Campbell K, Dubowitz V. Deficiency of the 50 kDa dystrophin-associated glycoprotein and abnormal expression of utrophin in two south Asian cousins with variable expression of severe childhood autosomal recessive muscular dystrophy. Neuromuscul Disord. 1994;4:121-9 pubmed
    ..Abnormal expression of utrophin, the dystrophin-related protein, was observed on the surface of several non-regenerating muscle fibres, with less ..
  27. Grady R, Merlie J, Sanes J. Subtle neuromuscular defects in utrophin-deficient mice. J Cell Biol. 1997;136:871-82 pubmed
    b>Utrophin is a large cytoskeletal protein that is homologous to dystrophin, the protein mutated in Duchenne and Becker muscular dystrophy...
  28. Sonnemann K, Heun Johnson H, Turner A, Baltgalvis K, Lowe D, Ervasti J. Functional substitution by TAT-utrophin in dystrophin-deficient mice. PLoS Med. 2009;6:e1000083 pubmed publisher
    ..Increased expression of the dystrophin homolog utrophin by gene delivery or pharmacologic up-regulation has been demonstrated to restore membrane integrity and improve ..
  29. Yue Y, Liu M, Duan D. C-terminal-truncated microdystrophin recruits dystrobrevin and syntrophin to the dystrophin-associated glycoprotein complex and reduces muscular dystrophy in symptomatic utrophin/dystrophin double-knockout mice. Mol Ther. 2006;14:79-87 pubmed
    ..Evaluating DeltaC microdystrophin in a symptomatic model will be of significant relevance to human trials. Utrophin/dystrophin double-knockout (u-dko) mice were developed to model severe dystrophic changes in human patients...
  30. Lin A, Prochniewicz E, James Z, Svensson B, Thomas D. Large-scale opening of utrophin's tandem calponin homology (CH) domains upon actin binding by an induced-fit mechanism. Proc Natl Acad Sci U S A. 2011;108:12729-33 pubmed publisher
    ..spin labeling and pulsed electron paramagnetic resonance to resolve a controversy concerning the structure of the utrophin-actin complex, with implications for the pathophysiology of muscular dystrophy...
  31. Ramirez Sanchez I, Mendoza Lorenzo P, Zentella Dehesa A, Méndez Bolaina E, Lara Padilla E, Ceballos Reyes G, et al. Caveolae and non-caveolae lipid raft microdomains of human umbilical vein endothelial cells contain utrophin-associated protein complexes. Biochimie. 2012;94:1884-90 pubmed publisher
    ..In vascular endothelium, dystrophin is substituted for utrophin (autosomal homolog of dystrophin); however, its role in this tissue is unknown...
  32. Gramolini A, Belanger G, Thompson J, Chakkalakal J, Jasmin B. Increased expression of utrophin in a slow vs. a fast muscle involves posttranscriptional events. Am J Physiol Cell Physiol. 2001;281:C1300-9 pubmed
    ..Because utrophin is a structural protein expressed at the neuromuscular junction, we hypothesize that its expression may be ..
  33. Khurana T, Kunkel L, Frederickson A, Carbonetto S, Watkins S. Interaction of chromosome-6-encoded dystrophin related protein with the extracellular matrix. J Cell Sci. 1995;108 ( Pt 1):173-85 pubmed
    Dystrophin-related protein/utrophin is a large, cytoskeletal protein that shares significant sequence similarity with dystrophin...
  34. Deconinck N, Tinsley J, De Backer F, Fisher R, Kahn D, Phelps S, et al. Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice. Nat Med. 1997;3:1216-21 pubmed
    Dystrophin-deficient mice (mdx) expressing a truncated (trc) utrophin transgene show amelioration of the dystrophic phenotype...
  35. Galvagni F, Capo S, Oliviero S. Sp1 and Sp3 physically interact and co-operate with GABP for the activation of the utrophin promoter. J Mol Biol. 2001;306:985-96 pubmed
    The utrophin gene codes for a large cytoskeletal protein closely related to dystrophin which, in the absence of dystrophin, can functionally substitute it. Utrophin is transcribed by two independently regulated promoters about 50 kb apart...
  36. Haenggi T, Soontornmalai A, Schaub M, Fritschy J. The role of utrophin and Dp71 for assembly of different dystrophin-associated protein complexes (DPCs) in the choroid plexus and microvasculature of the brain. Neuroscience. 2004;129:403-13 pubmed
    In the brain, utrophin is present in the choroid plexus epithelium and vascular endothelial cells, whereas the short C-terminal isoform of dystrophin (Dp71) is localized in the glial end-feet surrounding blood vessels...
  37. Burkin D, Wallace G, Nicol K, Kaufman D, Kaufman S. Enhanced expression of the alpha 7 beta 1 integrin reduces muscular dystrophy and restores viability in dystrophic mice. J Cell Biol. 2001;152:1207-18 pubmed
    ..the absence of dystrophin, we expressed the rat alpha 7 chain in mdx/utr(-/-) mice that lack both dystrophin and utrophin. These mice develop a severe muscular dystrophy highly akin to that in DMD, and they also die prematurely...
  38. Galkin V, Orlova A, VanLoock M, Egelman E. Do the utrophin tandem calponin homology domains bind F-actin in a compact or extended conformation?. J Mol Biol. 2003;331:967-72 pubmed
    ..A recent paper has used electron microscopy and three-dimensional reconstruction to examine the complex of the utrophin tandem CH domain with F-actin...
  39. Li D, Bareja A, Judge L, Yue Y, Lai Y, Fairclough R, et al. Sarcolemmal nNOS anchoring reveals a qualitative difference between dystrophin and utrophin. J Cell Sci. 2010;123:2008-13 pubmed publisher
    ..A particularly attractive approach is to increase utrophin expression. Utrophin shares considerable sequence, structural and functional similarity with dystrophin...
  40. Gyrd Hansen M, Krag T, Rosmarin A, Khurana T. Sp1 and the ets-related transcription factor complex GABP alpha/beta functionally cooperate to activate the utrophin promoter. J Neurol Sci. 2002;197:27-35 pubmed
    Duchenne muscular dystrophy (DMD) is a fatal neuromuscular disease caused by the absence of dystrophin. Utrophin is the autosomal homolog of dystrophin and capable of compensating for the absence of dystrophin, when overexpressed...
  41. Zuellig R, Bornhauser B, Knuesel I, Heller F, Fritschy J, Schaub M. Identification and characterisation of transcript and protein of a new short N-terminal utrophin isoform. J Cell Biochem. 2000;77:418-31 pubmed
    Dystrophin and utrophin are known to link the intracellular cytoskeleton to the extracellular matrix via a transmembraneous glycoprotein complex...
  42. Courdier Fruh I, Barman L, Wettstein P, Meier T. Detection of glucocorticoid-like activity in traditional Chinese medicine used for the treatment of Duchenne muscular dystrophy. Neuromuscul Disord. 2003;13:699-704 pubmed
    ..extracts from this Chinese medicine activate a prototype glucocorticoid-response element, increase the level of utrophin protein in human muscle cells and activate the utrophin promoter A...
  43. Perkins K, Basu U, Budak M, Ketterer C, Baby S, Lozynska O, et al. Ets-2 repressor factor silences extrasynaptic utrophin by N-box mediated repression in skeletal muscle. Mol Biol Cell. 2007;18:2864-72 pubmed
    b>Utrophin is the autosomal homologue of dystrophin, the protein product of the Duchenne's muscular dystrophy (DMD) locus...
  44. Kleopa K, Drousiotou A, Mavrikiou E, Ormiston A, Kyriakides T. Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophy. Hum Mol Genet. 2006;15:1623-8 pubmed
    Although there is good experimental data that utrophin, the autosomal analog of dystrophin, can ameliorate the phenotype in dystrophinopathies, there is scant evidence from human data to support this hypothesis...
  45. Ebihara S, Guibinga G, Gilbert R, Nalbantoglu J, Massie B, Karpati G, et al. Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy (mdx) mice. Physiol Genomics. 2000;3:133-44 pubmed
    ..In contrast, a closely related protein called utrophin is not foreign to DMD patients and is able to compensate for dystrophin deficiency when overexpressed throughout ..
  46. Deconinck A, Potter A, Tinsley J, Wood S, Vater R, Young C, et al. Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice. J Cell Biol. 1997;136:883-94 pubmed
    b>Utrophin is a dystrophin-related cytoskeletal protein expressed in many tissues. It is thought to link F-actin in the internal cytoskeleton to a transmembrane protein complex similar to the dystrophin protein complex (DPC)...
  47. Janssen P, Hiranandani N, Mays T, Rafael Fortney J. Utrophin deficiency worsens cardiac contractile dysfunction present in dystrophin-deficient mdx mice. Am J Physiol Heart Circ Physiol. 2005;289:H2373-8 pubmed
    ..mice have a much milder phenotype, whereas double knockout (DKO) mice lacking both dystrophin and its homolog, utrophin, exhibit the clinical signs observed in DMD patients...
  48. Lu Y, Tian C, Danialou G, Gilbert R, Petrof B, Karpati G, et al. Targeting artificial transcription factors to the utrophin A promoter: effects on dystrophic pathology and muscle function. J Biol Chem. 2008;283:34720-7 pubmed publisher
    ..b>Utrophin is a close analogue of dystrophin...
  49. Li D, Long C, Yue Y, Duan D. Sub-physiological sarcoglycan expression contributes to compensatory muscle protection in mdx mice. Hum Mol Genet. 2009;18:1209-20 pubmed publisher
    ..Enhanced muscle regeneration and the up-regulation of utrophin and integrin are thought to protect mdx muscle...
  50. Perkins K, Davies K. The role of utrophin in the potential therapy of Duchenne muscular dystrophy. Neuromuscul Disord. 2002;12 Suppl 1:S78-89 pubmed
    ..to three proteins that constitute the dystrophin related protein family, including the autosomal homologue, utrophin. An alternative strategy circumventing many problems associated with somatic gene therapies for Duchenne muscular ..
  51. Squire S, Raymackers J, Vandebrouck C, Potter A, Tinsley J, Fisher R, et al. Prevention of pathology in mdx mice by expression of utrophin: analysis using an inducible transgenic expression system. Hum Mol Genet. 2002;11:3333-44 pubmed
    ..a transgenic mouse model of the disease (mdx) that high levels of expression of the dystrophin-related protein, utrophin can prevent pathology...
  52. Weir A, Morgan J, Davies K. A-utrophin up-regulation in mdx skeletal muscle is independent of regeneration. Neuromuscul Disord. 2004;14:19-23 pubmed
    ..b>Utrophin is a paralogue of dystrophin and can functionally replace it in skeletal muscle...
  53. Khurana T, Watkins S, Kunkel L. The subcellular distribution of chromosome 6-encoded dystrophin-related protein in the brain. J Cell Biol. 1992;119:357-66 pubmed
    ..The distribution of DRP is conserved across a large evolutionary distance, from mammals to elasmobranchs, suggesting that DRP may play a role in the maintenance of regional specializations in the brain. ..