Utrn

Summary

Gene Symbol: Utrn
Description: utrophin
Alias: AA589569, DRP, Dmdl, utrophin, G-utrophin, utrophin short isoform, Up109
Species: mouse
Products:     Utrn

Top Publications

  1. 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...
  2. Bhosle R, Michele D, Campbell K, Li Z, Robson R. Interactions of intermediate filament protein synemin with dystrophin and utrophin. Biochem Biophys Res Commun. 2006;346:768-77 pubmed
    ..We show herein that tissue-purified avian synemin directly interacts with both dystrophin and utrophin, and that specific expressed regions of both of the mammalian (human) synemin isoforms (alpha-synemin and beta-..
  3. Angus L, Chakkalakal J, Mejat A, Eibl J, Belanger G, Megeney L, et al. Calcineurin-NFAT signaling, together with GABP and peroxisome PGC-1{alpha}, drives utrophin gene expression at the neuromuscular junction. Am J Physiol Cell Physiol. 2005;289:C908-17 pubmed
    ..nuclear factors of activated T cells) signaling plays a role in specifically directing the expression of utrophin in the synaptic compartment of muscle fibers...
  4. Burton E, Tinsley J, Holzfeind P, Rodrigues N, Davies K. A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy. Proc Natl Acad Sci U S A. 1999;96:14025-30 pubmed
    ..We have previously shown that utrophin, the autosomal homologue of dystrophin, is able to compensate for the absence of dystrophin in a mouse model of ..
  5. Crisp A, Yin H, Goyenvalle A, Betts C, Moulton H, Seow Y, et al. Diaphragm rescue alone prevents heart dysfunction in dystrophic mice. Hum Mol Genet. 2011;20:413-21 pubmed publisher
    ..Here, we investigated whether cardiomyopathy could be prevented in animal models of DMD by increasing diaphragm utrophin or dystrophin expression and thereby restoring diaphragm function...
  6. Uchino M, Yoshioka K, Miike T, Tokunaga M, Uyama E, Teramoto H, et al. Dystrophin and dystrophin-related protein in the brains of normal and mdx mice. Muscle Nerve. 1994;17:533-8 pubmed
    To clarify the localization and characterization of dystrophin and dystrophin-related protein (DRP) in the brains of normal and mdx mice, we carried out immunostaining and immunoblotting studies using four region-specific antidystrophin ..
  7. Rafael J, Tinsley J, Potter A, Deconinck A, Davies K. Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice. Nat Genet. 1998;19:79-82 pubmed
    ..Mice deficient for both dystrophin and the dystrophin-related protein, utrophin, (Dmd(mdx);Utrn-/- mice) die between 6 and 20 weeks of age suffering from severe muscle weakness with joint ..
  8. Gautam M, Noakes P, Moscoso L, Rupp F, Scheller R, Merlie J, et al. Defective neuromuscular synaptogenesis in agrin-deficient mutant mice. Cell. 1996;85:525-35 pubmed
  9. Xu R, Camboni M, Martin P. Postnatal overexpression of the CT GalNAc transferase inhibits muscular dystrophy in mdx mice without altering muscle growth or neuromuscular development: evidence for a utrophin-independent mechanism. Neuromuscul Disord. 2007;17:209-20 pubmed
    ..embryonic overexpression, postnatal overexpression of Galgt2 did not reproducibly increase the expression of utrophin, synaptic laminins, or dystrophin-associated glycoproteins along infected myofibers...

More Information

Publications64

  1. Matsumura K, Ervasti J, Ohlendieck K, Kahl S, Campbell K. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature. 1992;360:588-91 pubmed
    ..Here we demonstrate that dystrophin-related protein (DRP, utrophin), an autosomal homologue of dystrophin, is associated with an identical or antigenically similar complex of ..
  2. Burkin D, Wallace G, Milner D, Chaney E, Mulligan J, Kaufman S. Transgenic expression of {alpha}7{beta}1 integrin maintains muscle integrity, increases regenerative capacity, promotes hypertrophy, and reduces cardiomyopathy in dystrophic mice. Am J Pathol. 2005;166:253-63 pubmed
    ..We believe this multiplicity of responses to increased alpha7beta1 integrin collectively inhibits the development of muscle disease and increases longevity in these mice. ..
  3. 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...
  4. Rafael J, Trickett J, Potter A, Davies K. Dystrophin and utrophin do not play crucial roles in nonmuscle tissues in mice. Muscle Nerve. 1999;22:517-9 pubmed
    ..Both Dp71, a C-terminal dystrophin isoform, and the dystrophin-related protein, utrophin, are present at high levels in many nonmuscle tissues...
  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. Capote J, DiFranco M, Vergara J. Excitation-contraction coupling alterations in mdx and utrophin/dystrophin double knockout mice: a comparative study. Am J Physiol Cell Physiol. 2010;298:C1077-86 pubmed publisher
    The double knockout mouse for utrophin and dystrophin (utr(-/-)/mdx) has been proposed to be a better model of Duchenne Muscular Dystrophy (DMD) than the mdx mouse because the former displays more similar muscle pathology to that of the ..
  7. 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...
  8. Blake D, Schofield J, Zuellig R, Gorecki D, Phelps S, Barnard E, et al. G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain. Proc Natl Acad Sci U S A. 1995;92:3697-701 pubmed
    The utrophin gene is closely related to the dystrophin gene in both sequence and genomic structure...
  9. Rezniczek G, Konieczny P, Nikolic B, Reipert S, Schneller D, Abrahamsberg C, et al. Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with beta-dystroglycan. J Cell Biol. 2007;176:965-77 pubmed
    ..in vitro binding assays using recombinant protein fragments revealed the direct binding of plectin to dystrophin (utrophin) and beta-dystroglycan, the key components of the dystrophin-glycoprotein complex...
  10. Peter A, Ko C, Kim M, Hsu N, Ouchi N, Rhie S, et al. Myogenic Akt signaling upregulates the utrophin-glycoprotein complex and promotes sarcolemma stability in muscular dystrophy. Hum Mol Genet. 2009;18:318-27 pubmed publisher
    ..Here we show that myogenic Akt signaling in mouse models of dystrophy promotes increased expression of utrophin, which replaces the function of dystrophin thereby preventing sarcolemma damage and muscle wasting...
  11. Deconinck A, Rafael J, Skinner J, Brown S, Potter A, Metzinger L, et al. Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy. Cell. 1997;90:717-27 pubmed
    ..We describe mice deficient for both dystrophin and the dystrophin-related protein utrophin. These mice show many signs typical of DMD in humans: they show severe progressive muscular dystrophy that ..
  12. Grady R, Teng H, Nichol M, Cunningham J, Wilkinson R, Sanes J. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Cell. 1997;90:729-38 pubmed
    ..mdx mice also lack dystrophin, but are only mildly dystrophic. Utrophin, a homolog of dystrophin, is confined to the postsynaptic membrane at skeletal neuromuscular junctions and has ..
  13. Rybakova I, Patel J, Davies K, Yurchenco P, Ervasti J. Utrophin binds laterally along actin filaments and can couple costameric actin with sarcolemma when overexpressed in dystrophin-deficient muscle. Mol Biol Cell. 2002;13:1512-21 pubmed
    ..Although the dystrophin homolog utrophin can functionally compensate for dystrophin in mice, recent studies question whether utrophin can bind laterally ..
  14. Love D, Hill D, Dickson G, Spurr N, Byth B, Marsden R, et al. An autosomal transcript in skeletal muscle with homology to dystrophin. Nature. 1989;339:55-8 pubmed
    ..The dystrophin-related sequence identifies a 13-kb transcript in human fetal muscle and maps to chromosome 6. Thus, dystrophin may be a member of a family of functionally related large structural proteins in muscle. ..
  15. Grady R, Grange R, Lau K, Maimone M, Nichol M, Stull J, et al. Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies. Nat Cell Biol. 1999;1:215-20 pubmed
    ..These results indicate that both signalling and structural functions of the DGC are required for muscle stability, and implicate alpha-dystrobrevin in the former...
  16. Galvagni F, Cantini M, Oliviero S. The utrophin gene is transcriptionally up-regulated in regenerating muscle. J Biol Chem. 2002;277:19106-13 pubmed
    The utrophin gene codes for a large cytoskeletal protein closely related to dystrophin, the gene mutated in Duchenne's muscular dystrophy...
  17. Zhou L, Rafael Fortney J, Huang P, Zhao X, Cheng G, Zhou X, et al. Haploinsufficiency of utrophin gene worsens skeletal muscle inflammation and fibrosis in mdx mice. J Neurol Sci. 2008;264:106-11 pubmed
    To address whether mdx mice with haploinsufficiency of utrophin (mdx/utrn+/-) develop more severe skeletal muscle inflammation and fibrosis than mdx mice, to represent a better model for Duchenne muscular dystrophy (DMD), we performed ..
  18. Love D, Morris G, Ellis J, Fairbrother U, Marsden R, Bloomfield J, et al. Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse. Proc Natl Acad Sci U S A. 1991;88:3243-7 pubmed
    We have previously reported a dystrophin-related locus (DMDL for Duchenne muscular dystrophy-like) on human chromosome 6 that maps close to the dy mutation on mouse chromosome 10...
  19. Goyenvalle A, Babbs A, Wright J, Wilkins V, Powell D, Garcia L, et al. Rescue of severely affected dystrophin/utrophin-deficient mice through scAAV-U7snRNA-mediated exon skipping. Hum Mol Genet. 2012;21:2559-71 pubmed publisher
    ..In this study, we report for the first time the efficiency of the AAV-mediated exon skipping approach in the utrophin/dystrophin double-knockout (dKO) mouse which is a very severe and progressive mouse model of DMD...
  20. Miura P, Thompson J, Chakkalakal J, Holcik M, Jasmin B. The utrophin A 5'-untranslated region confers internal ribosome entry site-mediated translational control during regeneration of skeletal muscle fibers. J Biol Chem. 2005;280:32997-3005 pubmed
    b>Utrophin up-regulation in muscle fibers of Duchenne muscular dystrophy patients represents a potential therapeutic strategy...
  21. Verma M, Asakura Y, Hirai H, Watanabe S, Tastad C, Fong G, et al. Flt-1 haploinsufficiency ameliorates muscular dystrophy phenotype by developmentally increased vasculature in mdx mice. Hum Mol Genet. 2010;19:4145-59 pubmed publisher
    ..Consequently, the mdx:utrophin(-/-):Flt-1(+/-) mice display improved muscle histology and significantly higher survival rates compared with the ..
  22. Prins K, Humston J, Mehta A, Tate V, Ralston E, Ervasti J. Dystrophin is a microtubule-associated protein. J Cell Biol. 2009;186:363-9 pubmed publisher
    ..5-fold in mdx skeletal muscle without altering the tubulin-MT equilibrium. Collectively, these data suggest dystrophin directly organizes and/or stabilizes costameric MTs and classifies dystrophin as a cytolinker in skeletal muscle. ..
  23. 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...
  24. Law D, Allen D, Tidball J. Talin, vinculin and DRP (utrophin) concentrations are increased at mdx myotendinous junctions following onset of necrosis. J Cell Sci. 1994;107 ( Pt 6):1477-83 pubmed
    ..Blots also showed an increase (143%) in the dystrophin-related protein called utrophin, another myotendinous junction constituent, which may be able to substitute for dystrophin directly...
  25. Grady R, Zhou H, Cunningham J, Henry M, Campbell K, Sanes J. Maturation and maintenance of the neuromuscular synapse: genetic evidence for roles of the dystrophin--glycoprotein complex. Neuron. 2000;25:279-93 pubmed
    ..We also analyzed double and triple mutants lacking other cytoskeletal DGC components (utrophin and dystrophin) and myotubes lacking a alpha-DB or a transmembrane DGC component (dystroglycan)...
  26. Galvagni F, Oliviero S. Utrophin transcription is activated by an intronic enhancer. J Biol Chem. 2000;275:3168-72 pubmed
    The utrophin gene codes for a large cytoskeletal protein closely related to dystrophin. Its transcription is driven by a TATA-less promoter...
  27. Pons F, Robert A, Marini J, Leger J. Does utrophin expression in muscles of mdx mice during postnatal development functionally compensate for dystrophin deficiency?. J Neurol Sci. 1994;122:162-70 pubmed
    We correlated utrophin expression with the physiopathological course in mdx mice. Evolution of the pathology was assessed by monitoring expression of developmental MHC in mdx mice versus control...
  28. Odom G, Gregorevic P, Allen J, Finn E, Chamberlain J. Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin/utrophin-deficient mice. Mol Ther. 2008;16:1539-45 pubmed publisher
    ..Several lines of evidence have suggested that methods to increase expression of utrophin, a dystrophin paralog, show promise as a treatment for DMD...
  29. Dennis C, Tinsley J, Deconinck A, Davies K. Molecular and functional analysis of the utrophin promoter. Nucleic Acids Res. 1996;24:1646-52 pubmed
    b>Utrophin is a ubiquitously expressed cytoskeletal protein which is an important structural component of the mammalian neuromuscular junction...
  30. 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...
  31. Haenggi T, Fritschy J. Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue. Cell Mol Life Sci. 2006;63:1614-31 pubmed
    ..protein assembly associated with either the X-linked cytoskeletal protein dystrophin or its autosomal homologue utrophin. In striated muscle cells, the DGC links the extracellular matrix to the actin cytoskeleton and mediates three ..
  32. Schofield J, Houzelstein D, Davies K, Buckingham M, Edwards Y. Expression of the dystrophin-related protein (utrophin) gene during mouse embryogenesis. Dev Dyn. 1993;198:254-64 pubmed
    The utrophin (UTRN) locus is the autosomal homologue of the DMD (Duchenne muscular dystrophy) gene and encodes a protein, utrophin which is thought to be upregulated in the absence of dystrophin...
  33. Kamakura K, Tadano Y, Kawai M, Ishiura S, Nakamura R, Miyamoto K, et al. Dystrophin-related protein is found in the central nervous system of mice at various developmental stages, especially at the postsynaptic membrane. J Neurosci Res. 1994;37:728-34 pubmed
    ..This autosome-derived gene product is called dystrophin-related protein (DRP)...
  34. Bia B, Cassidy P, Young M, Rafael J, Leighton B, Davies K, et al. Decreased myocardial nNOS, increased iNOS and abnormal ECGs in mouse models of Duchenne muscular dystrophy. J Mol Cell Cardiol. 1999;31:1857-62 pubmed
    ..that the dystrophin-deficient mdx mouse and a mouse lacking both dystrophin and the dystrophin-related protein, utrophin (dko), have abnormal electrocardiograms (ECGs)...
  35. Krag T, Bogdanovich S, Jensen C, Fischer M, Hansen Schwartz J, Javazon E, et al. Heregulin ameliorates the dystrophic phenotype in mdx mice. Proc Natl Acad Sci U S A. 2004;101:13856-60 pubmed
    Duchenne's muscular dystrophy (DMD) is a fatal neuromuscular disease caused by absence of dystrophin. Utrophin is a chromosome 6-encoded dystrophin-related protein (DRP), sharing functional motifs with dystrophin...
  36. Rafael J, Townsend E, Squire S, Potter A, Chamberlain J, Davies K. Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure. Hum Mol Genet. 2000;9:1357-67 pubmed
    ..associated with the double knockout mouse deficient for both dystrophin and the dystrophin-related protein utrophin. We show that restoration of the DAPC with Dp71 does not prevent the structural abnormalities of the post-..
  37. Perkins K, Burton E, Davies K. The role of basal and myogenic factors in the transcriptional activation of utrophin promoter A: implications for therapeutic up-regulation in Duchenne muscular dystrophy. Nucleic Acids Res. 2001;29:4843-50 pubmed
    ..b>Utrophin is the autosomal homologue of dystrophin...
  38. Gramolini A, Dennis C, Tinsley J, Robertson G, Cartaud J, Davies K, et al. Local transcriptional control of utrophin expression at the neuromuscular synapse. J Biol Chem. 1997;272:8117-20 pubmed
    ..the use of a transgenic mouse model system for Duchenne muscular dystrophy has demonstrated the ability of utrophin to functionally replace dystrophin and alleviate the muscle pathology (see Tinsley, J. M., Potter, A. C...
  39. Liu J, Milner D, Boppart M, Ross R, Kaufman S. ?1D chain increases ?7?1 integrin and laminin and protects against sarcolemmal damage in mdx mice. Hum Mol Genet. 2012;21:1592-603 pubmed publisher
    ..of ?7 chain may alleviate development of muscular dystrophy and extend the lifespan of severely dystrophic mdx/utrn(-/-) mice, further enhancing levels of ?7 chain provided little additional membrane integrin and negligible ..
  40. van Putten M, Kumar D, Hulsker M, Hoogaars W, Plomp J, van Opstal A, et al. Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains. Neuromuscul Disord. 2012;22:406-17 pubmed publisher
    ..By contrast, mice lacking utrophin and dystrophin (mdx/utrn -/-) are severely affected and die prematurely...
  41. 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)...
  42. Gregorevic P, Allen J, Minami E, Blankinship M, Haraguchi M, Meuse L, et al. rAAV6-microdystrophin preserves muscle function and extends lifespan in severely dystrophic mice. Nat Med. 2006;12:787-9 pubmed
    Mice carrying mutations in both the dystrophin and utrophin genes die prematurely as a consequence of severe muscular dystrophy...
  43. Rybakova I, Humston J, Sonnemann K, Ervasti J. Dystrophin and utrophin bind actin through distinct modes of contact. J Biol Chem. 2006;281:9996-10001 pubmed
    ..epitopes of dystrophin-actin interaction and to directly compare the actin binding properties of dystrophin and utrophin. According to our data, dystrophin and utrophin both bound alongside actin filaments with submicromolar ..
  44. 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...
  45. Bonet Kerrache A, Fortier M, Comunale F, Gauthier Rouvière C. The GTPase RhoA increases utrophin expression and stability, as well as its localization at the plasma membrane. Biochem J. 2005;391:261-8 pubmed
    ..We report in the present study that the active form of RhoA increases the expression of utrophin, the autosomal homologue of dystrophin in the mouse C2C12 and rat L8 myoblastic cell lines...
  46. Grange R, Gainer T, Marschner K, Talmadge R, Stull J. Fast-twitch skeletal muscles of dystrophic mouse pups are resistant to injury from acute mechanical stress. Am J Physiol Cell Physiol. 2002;283:C1090-101 pubmed
    ..mechanical injury in the onset of DMD, extensor digitorum longus (EDL) muscles from C57 (control), mdx, and mdx-utrophin-deficient [mdx:utrn(-/-); dystrophic] pups aged 9-12 days were subjected to an acute stretch-injury or no-stretch ..
  47. Bostick B, Yue Y, Long C, Duan D. Prevention of dystrophin-deficient cardiomyopathy in twenty-one-month-old carrier mice by mosaic dystrophin expression or complementary dystrophin/utrophin expression. Circ Res. 2008;102:121-30 pubmed
    ..Interestingly, utrophin was upregulated in dystrophin-negative heart cells in carrier mice...
  48. Rosenberg M, Georges S, Asawachaicharn A, Analau E, Tapscott S. MyoD inhibits Fstl1 and Utrn expression by inducing transcription of miR-206. J Cell Biol. 2006;175:77-85 pubmed
    ..For example, the expression of utrophin (Utrn) is suppressed during skeletal muscle differentiation, and it is replaced at the sarcolemma by the related ..
  49. 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 ..
  50. 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...
  51. Mázala D, Grange R, Chin E. The role of proteases in excitation-contraction coupling failure in muscular dystrophy. Am J Physiol Cell Physiol. 2015;308:C33-40 pubmed publisher
    ..E-C) coupling disruption following repeated contractions is greater in animals lacking both dystrophin and utrophin (mdx/Utr(-/-)) compared with mice lacking only dystrophin (mdx); and 2) to assess whether protease inhibition can ..
  52. Voit A, Patel V, Pachon R, Shah V, Bakhutma M, Kohlbrenner E, et al. Reducing sarcolipin expression mitigates Duchenne muscular dystrophy and associated cardiomyopathy in mice. Nat Commun. 2017;8:1068 pubmed publisher
    ..Here we show that reducing SLN levels ameliorates dystrophic pathology in the severe dystrophin/utrophin double mutant (mdx:utr -/-) mouse model of DMD...
  53. 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...
  54. Lei K, Zhang X, Ding X, Guo X, Chen M, Zhu B, et al. SUN1 and SUN2 play critical but partially redundant roles in anchoring nuclei in skeletal muscle cells in mice. Proc Natl Acad Sci U S A. 2009;106:10207-12 pubmed publisher
    ..These results clearly indicate that SUN1 and SUN2 function critically in skeletal muscle cells for Syne-1 localization at the NE, which is essential for proper myonuclear positioning. ..
  55. Schofield J, Gorecki D, Blake D, Davies K, Edwards Y. Dystroglycan mRNA expression during normal and mdx mouse embryogenesis: a comparison with utrophin and the apo-dystrophins. Dev Dyn. 1995;204:178-85 pubmed
    ..In contrast, the major sites of utrophin expression do not co-localize with those of dystroglycan suggesting that utrophin may interact with a distinct ..