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In Duchenne muscular dystrophy (DMD), the absence of dystrophin leads to the loss of the DGC at the sarcolemma, resulting in sarcolemmal instability and progressive muscle damage. Utrophin (UTRN), an autosomal homolog of dystrophin, is upregulated in dystrophic muscle and partially compensates for the loss of dystrophin in muscle from patients with DMD. Here, we examine the interaction between Utr and sarcospan (SSPN), a small transmembrane protein that is a core component of both UTRN\u2013glycoprotein complex (UGC) and DGC. We show that additional loss of SSPN causes an earlier onset of disease in dystrophin-deficient mdx mice by reducing the expression of the UGC at the sarcolemma. In order to further evaluate the role of SSPN in maintaining therapeutic levels of Utr at the sarcolemma, we tested the effect of Utr transgenic overexpression in mdx mice lacking SSPN (mdx:SSPN \u2212\/\u2212:Utr-Tg). We found that overexpression of Utr restored SSPN to the sarcolemma in mdx muscle but that the ablation of SSPN in mdx muscle reduced Utr at the membrane. Nevertheless, Utr overexpression reduced central nucleation and improved grip strength in both lines. These findings demonstrate that high levels of Utr transgenic overexpression ameliorate the mdx phenotype independently of SSPN expression but that loss of SSPN may impair Utr-based mechanisms that rely on lower levels of Utr protein.<\/jats:p>","DOI":"10.1093\/hmg\/ddaa264","type":"journal-article","created":{"date-parts":[[2021,1,9]],"date-time":"2021-01-09T14:31:25Z","timestamp":1610202685000},"page":"149-159","source":"Crossref","is-referenced-by-count":5,"title":["Loss of sarcospan exacerbates pathology in<i>mdx<\/i>mice, but does not affect utrophin amelioration of disease"],"prefix":"10.1093","volume":"30","author":[{"given":"Elizabeth M","family":"Gibbs","sequence":"first","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California, Los Angeles, CA 90095, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jackie L","family":"McCourt","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California, Los Angeles, CA 90095, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kara M","family":"Shin","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California, Los Angeles, CA 90095, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine G","family":"Hammond","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California, Los Angeles, CA 90095, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie L","family":"Marshall","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California, Los Angeles, CA 90095, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachelle H","family":"Crosbie","sequence":"additional","affiliation":[{"name":"Department of Integrative Biology and Physiology, University of California, Los Angeles, CA 90095, USA"},{"name":"Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA"},{"name":"Molecular Biology Institute, University of California, Los Angeles, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,1,11]]},"reference":[{"key":"2021050309031454200_ref1","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1093\/oxfordjournals.jbchem.a123276","article-title":"Glycoprotein complex anchoring dystrophin to sarcolemma","volume":"108","author":"Yoshida","year":"1990","journal-title":"J. Biochem."},{"key":"2021050309031454200_ref2","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1038\/355696a0","article-title":"Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix","volume":"355","author":"Ibraghimov-Beskrovnaya","year":"1992","journal-title":"Nature"},{"key":"2021050309031454200_ref3","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1083\/jcb.122.4.809","article-title":"A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin","volume":"122","author":"Ervasti","year":"1993","journal-title":"J. Cell Biol."},{"key":"2021050309031454200_ref4","doi-asserted-by":"crossref","first-page":"3710","DOI":"10.1073\/pnas.90.8.3710","article-title":"Dystrophin protects the sarcolemma from stresses developed during muscle contraction","volume":"90","author":"Petrof","year":"1993","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2021050309031454200_ref5","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/0022-510X(90)90005-8","article-title":"Dystrophin-deficient mdx muscle fibers are preferentially vulnerable to necrosis induced by experimental lengthening contractions","volume":"100","author":"Weller","year":"1990","journal-title":"J. Neurol. Sci."},{"key":"2021050309031454200_ref6","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1038\/s41582-019-0203-3","article-title":"Therapeutic developments for Duchenne muscular dystrophy","volume":"15","author":"Verhaart","year":"2019","journal-title":"Nat. Rev. Neurol."},{"key":"2021050309031454200_ref7","doi-asserted-by":"crossref","first-page":"23593","DOI":"10.1016\/S0021-9258(18)54324-6","article-title":"Skeletal myoblasts utilize a novel beta 1-series integrin and not alpha 6 beta 1 for binding to the E8 and T8 fragments of laminin","volume":"266","author":"Mark","year":"1991","journal-title":"J. Biol. Chem."},{"key":"2021050309031454200_ref8","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1083\/jcb.117.3.643","article-title":"H36-alpha 7 is a novel integrin alpha chain that is developmentally regulated during skeletal myogenesis","volume":"117","author":"Song","year":"1992","journal-title":"J. Cell Biol."},{"key":"2021050309031454200_ref9","doi-asserted-by":"crossref","first-page":"C1660","DOI":"10.1152\/ajpcell.00317.2005","article-title":"Alpha7beta1-integrin regulates mechanotransduction and prevents skeletal muscle injury","volume":"290","author":"Boppart","year":"2006","journal-title":"Am J Physiol Cell Physiol"},{"key":"2021050309031454200_ref10","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1038\/339055a0","article-title":"An autosomal transcript in skeletal muscle with homology to dystrophin","volume":"339","author":"Love","year":"1989","journal-title":"Nature"},{"key":"2021050309031454200_ref11","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/338259a0","article-title":"Association of dystrophin and an integral membrane glycoprotein","volume":"338","author":"Campbell","year":"1989","journal-title":"Nature"},{"key":"2021050309031454200_ref12","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1038\/345315a0","article-title":"Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle","volume":"345","author":"Ervasti","year":"1990","journal-title":"Nature"},{"key":"2021050309031454200_ref13","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.1093\/hmg\/2.11.1765","article-title":"The utrophin and dystrophin genes share similarities in genomic structure","volume":"2","author":"Pearce","year":"1993","journal-title":"Hum. Mol. Genet."},{"key":"2021050309031454200_ref14","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1093\/hmg\/9.7.1033","article-title":"Biochemical evidence for association of dystrobrevin with the sarcoglycan-sarcospan complex as a basis for understanding sarcoglycanopathy","volume":"9","author":"Yoshida","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"2021050309031454200_ref15","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1083\/jcb.145.1.153","article-title":"Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan subcomplex","volume":"145","author":"Crosbie","year":"1999","journal-title":"J. Cell Biol."},{"key":"2021050309031454200_ref16","doi-asserted-by":"crossref","first-page":"31221","DOI":"10.1074\/jbc.272.50.31221","article-title":"Sarcospan: the 25kDa transmembrane component of the dystrophin-glycoprotein complex","volume":"272","author":"Crosbie","year":"1997","journal-title":"J. Biol. Chem."},{"key":"2021050309031454200_ref17","doi-asserted-by":"crossref","first-page":"691","DOI":"10.4103\/0028-3886.72164","article-title":"Sarcoglycanopathy: clinical and histochemical characteristics in 66 patients","volume":"58","author":"Nalini","year":"2010","journal-title":"Neurol. India"},{"key":"2021050309031454200_ref18","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1038\/ng1096-195","article-title":"Autosomal recessive limb-girdle muscular dystrophy, LGMD2F, is caused by a mutation in the delta-sarcoglycan gene","volume":"14","author":"Nigro","year":"1996","journal-title":"Nat. Genet."},{"key":"2021050309031454200_ref19","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1038\/ng1195-257","article-title":"Beta-sarcoglycan: characterization and role in limb-girdle muscular dystrophy linked to 4q12","volume":"11","author":"Lim","year":"1995","journal-title":"Nat. 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Genet."},{"key":"2021050309031454200_ref24","first-page":"1","article-title":"Genetic basis of limb-girdle muscular dystrophies: the 2014 update","volume":"33","author":"Nigro","year":"2014","journal-title":"Acta Myol."},{"key":"2021050309031454200_ref25","doi-asserted-by":"crossref","first-page":"2019","DOI":"10.1093\/hmg\/9.13.2019","article-title":"Molecular and genetic characterization of sarcospan: insights into sarcoglycan-sarcospan interactions","volume":"9","author":"Crosbie","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"2021050309031454200_ref26","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1083\/jcb.200808027","article-title":"Sarcospan reduces dystrophic pathology: stabilization of the utrophin-glycoprotein complex","volume":"183","author":"Peter","year":"2008","journal-title":"J. Cell Biol."},{"key":"2021050309031454200_ref27","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1083\/jcb.201110032","article-title":"Sarcospan-dependent Akt activation is required for utrophin expression and muscle regeneration","volume":"197","author":"Marshall","year":"2012","journal-title":"J. Cell Biol."},{"key":"2021050309031454200_ref28","first-page":"5395","article-title":"High levels of sarcospan are well tolerated and act as a sarcolemmal stabilizer to address skeletal muscle and pulmonary dysfunction in DMD","volume":"25","author":"Gibbs","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"2021050309031454200_ref29","doi-asserted-by":"crossref","first-page":"1669","DOI":"10.1128\/MCB.20.5.1669-1677.2000","article-title":"Sarcospan-deficient mice maintain normal muscle function","volume":"20","author":"Lebakken","year":"2000","journal-title":"Mol. Cell. Biol."},{"key":"2021050309031454200_ref30","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.nbd.2008.03.008","article-title":"Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy","volume":"31","author":"Grounds","year":"2008","journal-title":"Neurobiol. Dis."},{"key":"2021050309031454200_ref31","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1016\/j.ymthe.2004.07.026","article-title":"Targeted expression of insulin-like growth factor-I reduces early myofiber necrosis in dystrophic mdx mice","volume":"10","author":"Shavlakadze","year":"2004","journal-title":"Mol. Ther."},{"key":"2021050309031454200_ref32","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1111\/j.1440-1681.2006.04394.x","article-title":"Muscle damage in mdx (dystrophic) mice: role of calcium and reactive oxygen species","volume":"33","author":"Whitehead","year":"2006","journal-title":"Clin. Exp. Pharmacol. Physiol."},{"key":"2021050309031454200_ref33","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1083\/jcb.139.2.375","article-title":"Animal models for muscular dystrophy show different patterns of sarcolemmal disruption","volume":"139","author":"Straub","year":"1997","journal-title":"J. Cell Biol."},{"key":"2021050309031454200_ref34","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1111\/j.1749-6632.1957.tb35384.x","article-title":"The contribution of I 131-labeled proteins to measurements of blood volume","volume":"70","author":"Reeve","year":"1957","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"2021050309031454200_ref35","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1093\/jb\/118.5.959","article-title":"Visualization of dystrophic muscle fibers in mdx mouse by vital staining with Evans blue: evidence of apoptosis in dystrophin-deficient muscle","volume":"118","author":"Matsuda","year":"1995","journal-title":"J. Biochem."},{"key":"2021050309031454200_ref36","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.nmd.2011.08.001","article-title":"Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies","volume":"22","author":"Kobayashi","year":"2012","journal-title":"Neuromuscul. Disord."},{"key":"2021050309031454200_ref37","first-page":"56772","article-title":"A simple and low-cost assay for measuring ambulation in mouse models of muscular dystrophy","volume":"(130)","author":"Gibbs","year":"2017","journal-title":"J. Vis. Exp."},{"key":"2021050309031454200_ref38","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1038\/4033","article-title":"Expression of full-length utrophin prevents muscular dystrophy in mdx mice","volume":"4","author":"Tinsley","year":"1998","journal-title":"Nat. Med."},{"key":"2021050309031454200_ref39","first-page":"307","article-title":"Embryonic myosin is a regeneration marker to monitor utrophin-based therapies for DMD","volume":"28","author":"Guiraud","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"2021050309031454200_ref40","doi-asserted-by":"crossref","first-page":"5723","DOI":"10.1073\/pnas.1323842111","article-title":"Microtubule binding distinguishes dystrophin from utrophin","volume":"111","author":"Belanto","year":"2014","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2021050309031454200_ref41","doi-asserted-by":"crossref","first-page":"S90","DOI":"10.1016\/S0960-8966(02)00088-3","article-title":"Multivariate evaluation of the functional recovery obtained by the overexpression of utrophin in skeletal muscles of the mdx mouse","volume":"12","author":"Gillis","year":"2002","journal-title":"Neuromuscul. Disord."},{"key":"2021050309031454200_ref42","doi-asserted-by":"crossref","first-page":"2008","DOI":"10.1242\/jcs.064808","article-title":"Sarcolemmal nNOS anchoring reveals a qualitative difference between dystrophin and utrophin","volume":"123","author":"Li","year":"2010","journal-title":"J. Cell Sci."},{"key":"2021050309031454200_ref43","doi-asserted-by":"crossref","first-page":"38371","DOI":"10.1038\/srep38371","article-title":"Impaired regenerative capacity and lower revertant fibre expansion in dystrophin-deficient mdx muscles on DBA\/2 background","volume":"6","author":"Rodrigues","year":"2016","journal-title":"Sci. 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Heart"},{"key":"2021050309031454200_ref47","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1038\/88865","article-title":"Mutant glycosyltransferase and altered glycosylation of alpha-dystroglycan in the myodystrophy mouse","volume":"28","author":"Grewal","year":"2001","journal-title":"Nat. Genet."},{"key":"2021050309031454200_ref48","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/s13395-019-0218-x","article-title":"Development of a high-throughput screen to identify small molecule enhancers of sarcospan for the treatment of Duchenne muscular dystrophy","volume":"9","author":"Shu","year":"2019","journal-title":"Skelet. Muscle"},{"key":"2021050309031454200_ref49","doi-asserted-by":"crossref","first-page":"2132","DOI":"10.1038\/s41598-020-58737-6","article-title":"High-throughput identification of post-transcriptional utrophin up-regulators for Duchenne muscle dystrophy (DMD) therapy","volume":"10","author":"Loro","year":"2020","journal-title":"Sci. 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Drug Dev."},{"key":"2021050309031454200_ref52","doi-asserted-by":"crossref","first-page":"7880","DOI":"10.1021\/acs.jmedchem.0c00807","article-title":"2-Arylbenzo[d]oxazole phosphinate esters as second-generation modulators of utrophin for the treatment of Duchenne muscular dystrophy","volume":"2020 Jul 23;63(14)","author":"Babbs","year":"2020","journal-title":"J. Med. Chem."},{"key":"2021050309031454200_ref53","doi-asserted-by":"crossref","first-page":"4212","DOI":"10.1093\/hmg\/ddv154","article-title":"Second-generation compound for the modulation of utrophin in the therapy of DMD","volume":"24","author":"Guiraud","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"2021050309031454200_ref54","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1038\/s41467-020-15971-w","article-title":"Identification of therapeutics that target eEF1A2 and upregulate utrophin A translation in dystrophic muscles","volume":"11","author":"Peladeau","year":"2020","journal-title":"Nat. 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