{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T19:46:21Z","timestamp":1788291981467,"version":"build-2803163510"},"reference-count":78,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T00:00:00Z","timestamp":1667260800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T00:00:00Z","timestamp":1667260800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,7,18]],"date-time":"2022-07-18T00:00:00Z","timestamp":1658102400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Fundamental Research"],"published-print":{"date-parts":[[2022,11]]},"DOI":"10.1016\/j.fmre.2022.07.004","type":"journal-article","created":{"date-parts":[[2022,7,21]],"date-time":"2022-07-21T13:36:20Z","timestamp":1658410580000},"page":"885-893","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":16,"title":["Promising therapeutic approaches of utrophin replacing dystrophin in the treatment of Duchenne muscular dystrophy"],"prefix":"10.1016","volume":"2","author":[{"given":"Ruo","family":"Wu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yafeng","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiwen","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yongchang","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.fmre.2022.07.004_bib0001","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1002\/humu.22758","article-title":"The TREAT-NMD DMD global database: analysis of more than 7000 Duchenne muscular dystrophy mutations","volume":"36","author":"Bladen","year":"2015","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.fmre.2022.07.004_bib0002","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1016\/S1474-4422(18)30025-5","article-title":"Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management","volume":"17","author":"Birnkrant","year":"2018","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0003","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/0092-8674(87)90579-4","article-title":"Dystrophin: the protein product of the Duchenne muscular dystrophy locus","volume":"51","author":"Hoffman","year":"1987","journal-title":"Cell"},{"key":"10.1016\/j.fmre.2022.07.004_bib0004","series-title":"Molecular and cellular basis of genetically inherited skeletal muscle disorders","author":"Dowling","year":"2021"},{"key":"10.1016\/j.fmre.2022.07.004_bib0005","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1002\/mus.20586","article-title":"Entries in the Leiden Duchenne muscular dystrophy mutation database: an overview of mutation types and paradoxical cases that confirm the reading-frame rule","volume":"34","author":"Aartsma-Rus","year":"2006","journal-title":"Muscle Nerve"},{"key":"10.1016\/j.fmre.2022.07.004_bib0006","doi-asserted-by":"crossref","first-page":"988","DOI":"10.7150\/ijms.8391","article-title":"Identification of de novo mutations of Duch\u00e9nn\u00e8\/Becker muscular dystrophies in southern Spain","volume":"11","author":"Garcia","year":"2014","journal-title":"Int. J. Med. Sci."},{"key":"10.1016\/j.fmre.2022.07.004_bib0007","doi-asserted-by":"crossref","first-page":"341","DOI":"10.2217\/cer-2019-0171","article-title":"Safety and effectiveness of ataluren: comparison of results from the STRIDE registry and CINRG DMD natural history study","volume":"9","author":"Mercuri","year":"2020","journal-title":"J. Comp. Eff. Res."},{"key":"10.1016\/j.fmre.2022.07.004_bib0008","doi-asserted-by":"crossref","first-page":"e2270","DOI":"10.1212\/WNL.0000000000009233","article-title":"Increased dystrophin production with golodirsen in patients with Duchenne muscular dystrophy","volume":"94","author":"Frank","year":"2020","journal-title":"Neurology"},{"key":"10.1016\/j.fmre.2022.07.004_bib0009","doi-asserted-by":"crossref","first-page":"533","DOI":"10.2147\/DDDT.S97635","article-title":"Eteplirsen in the treatment of Duchenne muscular dystrophy","volume":"11","author":"Lim","year":"2017","journal-title":"Drug Des. Dev. Ther."},{"key":"10.1016\/j.fmre.2022.07.004_bib0010","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1080\/00207454.2020.1740218","article-title":"Common therapeutic advances for Duchenne muscular dystrophy (DMD)","volume":"131","author":"Salmaninejad","year":"2021","journal-title":"Int. J. Neurosci."},{"key":"10.1016\/j.fmre.2022.07.004_bib0011","doi-asserted-by":"crossref","DOI":"10.3390\/jpm9010016","article-title":"Mutation-based therapeutic strategies for Duchenne muscular dystrophy: from genetic diagnosis to therapy","author":"Nakamura","year":"2019","journal-title":"J. Pers. Med. 9"},{"key":"10.1016\/j.fmre.2022.07.004_bib0012","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1038\/s41551-021-00774-1","article-title":"Restoration of dystrophin expression in mice by suppressing a nonsense mutation through the incorporation of unnatural amino acids","volume":"6","author":"Shi","year":"2022","journal-title":"Nat. Biomed. Eng."},{"key":"10.1016\/j.fmre.2022.07.004_bib0013","first-page":"e15858","volume":"98","author":"Alfano","year":"2019","journal-title":"Long-term treatment with eteplirsen in nonambulatory patients with Duchenne muscular dystrophy"},{"key":"10.1016\/j.fmre.2022.07.004_bib0014","doi-asserted-by":"crossref","first-page":"1760","DOI":"10.1161\/CIRCULATIONAHA.121.054628","article-title":"Therapeutic exon skipping through a CRISPR-guided cytidine deaminase rescues dystrophic cardiomyopathy in vivo","volume":"144","author":"Li","year":"2021","journal-title":"Circulation."},{"key":"10.1016\/j.fmre.2022.07.004_bib0015","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.1001\/jamaneurol.2020.1484","article-title":"Assessment of systemic delivery of rAAVrh74.MHCK7.micro-dystrophin in children with Duchenne muscular dystrophy: a nonrandomized controlled trial","volume":"77","author":"Mendell","year":"2020","journal-title":"JAMA Neurol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0016","doi-asserted-by":"crossref","first-page":"2337","DOI":"10.1016\/j.ymthe.2018.07.011","article-title":"Systemic AAV micro-dystrophin gene therapy for duchenne muscular dystrophy","volume":"26","author":"Duan","year":"2018","journal-title":"Mol. Ther."},{"key":"10.1016\/j.fmre.2022.07.004_bib0017","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":"10.1016\/j.fmre.2022.07.004_bib0018","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/S0165-3806(99)00165-0","article-title":"Utrophin may be a precursor of dystrophin during skeletal muscle development","volume":"119","author":"Lin","year":"2000","journal-title":"Brain Res. Dev. Brain Res."},{"key":"10.1016\/j.fmre.2022.07.004_bib0019","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1007\/BF02388063","article-title":"Dystrophin-related protein, utrophin, in normal and dystrophic human fetal skeletal muscle","volume":"25","author":"Clerk","year":"1993","journal-title":"Histochem. J."},{"key":"10.1016\/j.fmre.2022.07.004_bib0020","doi-asserted-by":"crossref","first-page":"45285","DOI":"10.1074\/jbc.M205177200","article-title":"A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle","volume":"277","author":"Weir","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.fmre.2022.07.004_bib0021","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1152\/physrev.00028.2001","article-title":"Function and genetics of dystrophin and dystrophin-related proteins in muscle","volume":"82","author":"Blake","year":"2002","journal-title":"Physiol. Rev."},{"key":"10.1016\/j.fmre.2022.07.004_bib0022","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1007\/978-3-319-49674-0_12","article-title":"Dystrophin and spectrin, two highly dissimilar sisters of the same family","volume":"82","author":"Delalande","year":"2017","journal-title":"Subcell Biochem."},{"key":"10.1016\/j.fmre.2022.07.004_bib0023","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":"10.1016\/j.fmre.2022.07.004_bib0024","first-page":"4951","article-title":"Independent variability of microtubule perturbations associated with dystrophinopathy","volume":"25","author":"Belanto","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0025","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1016\/S0092-8674(00)80532-2","article-title":"Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy","volume":"90","author":"Deconinck","year":"1997","journal-title":"Cell"},{"key":"10.1016\/j.fmre.2022.07.004_bib0026","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":"10.1016\/j.fmre.2022.07.004_bib0027","doi-asserted-by":"crossref","first-page":"1505","DOI":"10.1038\/s41591-019-0594-0","article-title":"Non-immunogenic utrophin gene therapy for the treatment of muscular dystrophy animal models","volume":"25","author":"Song","year":"2019","journal-title":"Nat. Med."},{"key":"10.1016\/j.fmre.2022.07.004_bib0028","doi-asserted-by":"crossref","first-page":"1872","DOI":"10.1016\/j.ymthe.2019.10.011","article-title":"Micro-utrophin therapy for duchenne muscular dystrophy","volume":"27","author":"Duan","year":"2019","journal-title":"Mol. Ther."},{"key":"10.1016\/j.fmre.2022.07.004_bib0029","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/j.nmd.2005.03.008","article-title":"A 1.3 kb promoter fragment confers spatial and temporal expression of utrophin A mRNA in mouse skeletal muscle fibers","volume":"15","author":"Stocksley","year":"2005","journal-title":"Neuromuscul. Disord."},{"key":"10.1016\/j.fmre.2022.07.004_bib0030","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1006\/jmbi.2000.4335","article-title":"Sp1 and Sp3 physically interact and co-operate with GABP for the activation of the utrophin promoter","author":"Galvagni","year":"2001","journal-title":"J. Mol. Biol. 306"},{"key":"10.1016\/j.fmre.2022.07.004_bib0031","doi-asserted-by":"crossref","first-page":"19106","DOI":"10.1074\/jbc.M109642200","article-title":"The utrophin gene is transcriptionally up-regulated in regenerating muscle","volume":"277","author":"Galvagni","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.fmre.2022.07.004_bib0032","doi-asserted-by":"crossref","first-page":"2864","DOI":"10.1091\/mbc.e06-12-1069","article-title":"Ets-2 repressor factor silences extrasynaptic utrophin by N-box mediated repression in skeletal muscle","volume":"18","author":"Perkins","year":"2007","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.fmre.2022.07.004_bib0033","doi-asserted-by":"crossref","DOI":"10.1155\/2010\/721219","article-title":"The functional role of calcineurin in hypertrophy, regeneration, and disorders of skeletal muscle","volume":"2010","author":"Sakuma","year":"2010","journal-title":"J. Biomed. Biotechnol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0034","doi-asserted-by":"crossref","first-page":"2950","DOI":"10.1096\/fj.201700868RRR","article-title":"The role of AMP-activated protein kinase in the expression of the dystrophin-associated protein complex in skeletal muscle","volume":"32","author":"Dial","year":"2018","journal-title":"Faseb J."},{"key":"10.1016\/j.fmre.2022.07.004_bib0035","doi-asserted-by":"crossref","first-page":"2075","DOI":"10.1091\/mbc.10.6.2075","article-title":"Activation of utrophin promoter by heregulin via the ets-related transcription factor complex GA-binding protein alpha\/beta","volume":"10","author":"Khurana","year":"1999","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.fmre.2022.07.004_bib0036","doi-asserted-by":"crossref","first-page":"C908","DOI":"10.1152\/ajpcell.00196.2005","article-title":"Calcineurin-NFAT signaling, together with GABP and peroxisome PGC-1{alpha}, drives utrophin gene expression at the neuromuscular junction","volume":"289","author":"Angus","year":"2005","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0037","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1016\/j.apsb.2020.01.001","article-title":"Natural products, PGC-1 \u03b1, and Duchenne muscular dystrophy","volume":"10","author":"Suntar","year":"2020","journal-title":"Acta Pharm. Sin. B"},{"key":"10.1016\/j.fmre.2022.07.004_bib0038","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1093\/hmg\/ddl065","article-title":"Targeted inhibition of Ca2+ \/calmodulin signaling exacerbates the dystrophic phenotype in mdx mouse muscle","volume":"15","author":"Chakkalakal","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0039","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1113\/jphysiol.2006.108472","article-title":"Activated calcineurin ameliorates contraction-induced injury to skeletal muscles of mdx dystrophic mice","volume":"575","author":"Stupka","year":"2006","journal-title":"J. Physiol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0040","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1016\/j.tem.2018.02.010","article-title":"The role of AMPK in neuromuscular biology and disease","volume":"29","author":"Dial","year":"2018","journal-title":"Trends Endocrinol. Metab."},{"key":"10.1016\/j.fmre.2022.07.004_bib0041","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1093\/abbs\/gmy028","article-title":"Negative regulation of TGF-\u03b2 by AMPK and implications in the treatment of associated disorders","volume":"50","author":"Gao","year":"2018","journal-title":"Acta Biochim. Biophys. Sin. (Shanghai)"},{"key":"10.1016\/j.fmre.2022.07.004_bib0042","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1093\/hmg\/ddv444","article-title":"Combinatorial therapeutic activation with heparin and AICAR stimulates additive effects on utrophin A expression in dystrophic muscles","volume":"25","author":"P\u00e9ladeau","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0043","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1002\/mus.24692","article-title":"Metformin increases peroxisome proliferator-activated receptor \u03b3 Co-activator-1\u03b1 and utrophin a expression in dystrophic skeletal muscle","volume":"52","author":"Ljubicic","year":"2015","journal-title":"Muscle Nerve"},{"key":"10.1016\/j.fmre.2022.07.004_bib0044","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1002\/jcsm.12531","article-title":"AdipoRon, a new therapeutic prospect for Duchenne muscular dystrophy","volume":"11","author":"Abou-Samra","year":"2020","journal-title":"J. Cachexia Sarcopenia Muscle"},{"key":"10.1016\/j.fmre.2022.07.004_bib0045","doi-asserted-by":"crossref","first-page":"13856","DOI":"10.1073\/pnas.0405972101","article-title":"Heregulin ameliorates the dystrophic phenotype in mdx mice","volume":"101","author":"Krag","year":"2004","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.fmre.2022.07.004_bib0046","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1139\/O07-015","article-title":"The artificial 4-zinc-finger protein Bagly binds human utrophin promoter A at the endogenous chromosomal site and activates transcription","volume":"85","author":"Onori","year":"2007","journal-title":"Biochem. Cell Biol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0047","doi-asserted-by":"crossref","first-page":"5090","DOI":"10.1096\/fj.201800081R","article-title":"Celecoxib treatment improves muscle function in mdx mice and increases utrophin A expression","volume":"32","author":"Peladeau","year":"2018","journal-title":"Faseb J."},{"key":"10.1016\/j.fmre.2022.07.004_bib0048","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1111\/cts.12562","article-title":"Anisomycin activates utrophin upregulation through a p38 signaling pathway","volume":"11","author":"Hadwen","year":"2018","journal-title":"Clin. Transl. Sci."},{"key":"10.1016\/j.fmre.2022.07.004_bib0049","doi-asserted-by":"crossref","first-page":"e00140-19","DOI":"10.1128\/MCB.00140-19","article-title":"Soluble Heparin binding epidermal growth factor-like growth factor is a regulator of GALGT2 expression and GALGT2-dependent muscle and neuromuscular phenotypes","volume":"39","author":"Cramer","year":"2019","journal-title":"Mol. Cell Biol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0050","doi-asserted-by":"crossref","first-page":"2813","DOI":"10.1093\/hmg\/ddx117","article-title":"RhoA\/ROCK inhibition improves the beneficial effects of glucocorticoid treatment in dystrophic muscle: implications for stem cell depletion","volume":"26","author":"Mu","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0051","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1074\/jbc.273.2.736","article-title":"Muscle and neural isoforms of agrin increase utrophin expression in cultured myotubes via a transcriptional regulatory mechanism","volume":"273","author":"Gramolini","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.fmre.2022.07.004_bib0052","doi-asserted-by":"crossref","first-page":"1579","DOI":"10.1093\/hmg\/ddab135","article-title":"Increasing LRP4 diminishes neuromuscular deficits in a mouse model of Duchenne muscular dystrophy","volume":"30","author":"Hui","year":"2021","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0053","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1002\/cpdd.642","article-title":"A phase 1b trial to assess the pharmacokinetics of ezutromid in pediatric duchenne muscular dystrophy patients on a balanced diet","volume":"8","author":"Muntoni","year":"2019","journal-title":"Clin. Pharmacol. Drug Dev."},{"key":"10.1016\/j.fmre.2022.07.004_bib0054","doi-asserted-by":"crossref","DOI":"10.1016\/j.bioorg.2019.103395","article-title":"Characterisation of utrophin modulator SMT C1100 as a non-competitive inhibitor of firefly luciferase","volume":"94","author":"Wilkinson","year":"2020","journal-title":"Bioorg. Chem."},{"key":"10.1016\/j.fmre.2022.07.004_bib0055","doi-asserted-by":"crossref","DOI":"10.1016\/j.tet.2019.130819","article-title":"Synthesis of SMT022357 enantiomers and in vivo evaluation in a Duchenne muscular dystrophy mouse model","volume":"76","author":"Babbs","year":"2020","journal-title":"Tetrahedron"},{"key":"10.1016\/j.fmre.2022.07.004_bib0056","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1093\/hmg\/ddv466","article-title":"Sarcolemmal targeting of nNOS\u03bc improves contractile function of mdx muscle","volume":"25","author":"Rebolledo","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0057","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1186\/s10020-019-0101-6","article-title":"Dystrophin R16\/17 protein therapy restores sarcolemmal nNOS in trans and improves muscle perfusion and function","volume":"25","author":"Zhao","year":"2019","journal-title":"Mol. Med."},{"key":"10.1016\/j.fmre.2022.07.004_bib0058","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1093\/hmg\/ddaa264","article-title":"Loss of sarcospan exacerbates pathology in mdx mice, but does not affect utrophin amelioration of disease","volume":"30","author":"Gibbs","year":"2021","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0059","doi-asserted-by":"crossref","first-page":"2011","DOI":"10.1093\/hmg\/ddu615","article-title":"Sarcospan integration into laminin-binding adhesion complexes that ameliorate muscular dystrophy requires utrophin and alpha7 integrin","volume":"24","author":"Marshall","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0060","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1073\/pnas.1013067108","article-title":"Biglycan recruits utrophin to the sarcolemma and counters dystrophic pathology in mdx mice","volume":"108","author":"Amenta","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.fmre.2022.07.004_bib0061","doi-asserted-by":"crossref","first-page":"2686","DOI":"10.1093\/hmg\/ddz086","article-title":"Laminin-111 protein therapy enhances muscle regeneration and repair in the GRMD dog model of Duchenne muscular dystrophy","volume":"28","author":"Barraza-Flores","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.fmre.2022.07.004_bib0062","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1038\/mt.2013.246","article-title":"Vascular delivery of rAAVrh74.MCK.GALGT2 to the gastrocnemius muscle of the rhesus macaque stimulates the expression of dystrophin and laminin alpha2 surrogates","volume":"22","author":"Chicoine","year":"2014","journal-title":"Mol. Ther."},{"key":"10.1016\/j.fmre.2022.07.004_bib0063","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. Rep."},{"key":"10.1016\/j.fmre.2022.07.004_bib0064","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. Commun."},{"issue":"1","key":"10.1016\/j.fmre.2022.07.004_bib0065","doi-asserted-by":"crossref","first-page":"S95","DOI":"10.1016\/S0960-8966(02)00089-5","article-title":"Glucocorticoid-mediated regulation of utrophin levels in human muscle fibers","volume":"12","author":"Courdier-Fruh","year":"2002","journal-title":"Neuromuscul. Disord."},{"key":"10.1016\/j.fmre.2022.07.004_bib0066","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1177\/147323001103900117","article-title":"A method of utrophin up-regulation through RNAi-mediated knockdown of the transcription factor EN1","volume":"39","author":"Wang","year":"2011","journal-title":"J. Int. Med. Res."},{"key":"10.1016\/j.fmre.2022.07.004_bib0067","doi-asserted-by":"crossref","first-page":"e29376","DOI":"10.1371\/journal.pone.0029376","article-title":"Translational regulation of utrophin by miRNAs","volume":"6","author":"Basu","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.fmre.2022.07.004_bib0068","doi-asserted-by":"crossref","first-page":"21492","DOI":"10.1038\/s41598-020-76338-1","article-title":"PMO-based let-7c site blocking oligonucleotide (SBO) mediated utrophin upregulation in mdx mice, a therapeutic approach for Duchenne muscular dystrophy (DMD)","volume":"10","author":"Sengupta","year":"2020","journal-title":"Sci. Rep."},{"key":"10.1016\/j.fmre.2022.07.004_bib0069","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/j.omtn.2018.05.011","article-title":"MicroRNA-206 downregulation improves therapeutic gene expression and motor function in mdx mice","volume":"12","author":"Bulaklak","year":"2018","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"10.1016\/j.fmre.2022.07.004_bib0070","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1038\/s41419-019-1307-9","article-title":"sPIF promotes myoblast differentiation and utrophin expression while inhibiting fibrosis in Duchenne muscular dystrophy via the H19\/miR-675\/let-7 and miR-21 pathways","volume":"10","author":"Morgoulis","year":"2019","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.fmre.2022.07.004_bib0071","doi-asserted-by":"crossref","first-page":"1283","DOI":"10.1002\/jcp.24567","article-title":"Novel adeno-associated viral vector delivering the utrophin gene regulator jazz counteracts dystrophic pathology in mdx mice","volume":"229","author":"Strimpakos","year":"2014","journal-title":"J. Cell Physiol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0072","doi-asserted-by":"crossref","first-page":"2281","DOI":"10.1038\/nprot.2013.143","article-title":"Genome engineering using the CRISPR-Cas9 system","volume":"8","author":"Ran","year":"2013","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.fmre.2022.07.004_bib0073","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1016\/j.cell.2013.06.044","article-title":"CRISPR-mediated modular RNA-guided regulation of transcription in eukaryotes","volume":"154","author":"Gilbert","year":"2013","journal-title":"Cell"},{"key":"10.1016\/j.fmre.2022.07.004_bib0074","doi-asserted-by":"crossref","first-page":"1495","DOI":"10.1016\/j.cell.2017.10.025","article-title":"In vivo target gene activation via CRISPR\/Cas9-mediated trans-epigenetic modulation","volume":"171","author":"Liao","year":"2017","journal-title":"Cell"},{"key":"10.1016\/j.fmre.2022.07.004_bib0075","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1038\/mt.2008.149","article-title":"Microutrophin delivery through rAAV6 increases lifespan and improves muscle function in dystrophic dystrophin\/utrophin-deficient mice","volume":"16","author":"Odom","year":"2008","journal-title":"Mol. Ther."},{"key":"10.1016\/j.fmre.2022.07.004_bib0076","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pmed.1000083","article-title":"Functional substitution by TAT-utrophin in dystrophin-deficient mice","volume":"6","author":"Sonnemann","year":"2009","journal-title":"PLoS Med."},{"key":"10.1016\/j.fmre.2022.07.004_bib0077","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1152\/japplphysiol.00248.2011","article-title":"TAT-\u03bcUtrophin mitigates the pathophysiology of dystrophin and utrophin double-knockout mice","volume":"111","author":"Call","year":"2011","journal-title":"J. Appl. Physiol."},{"key":"10.1016\/j.fmre.2022.07.004_bib0078","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1042\/BJ20050024","article-title":"The GTPase RhoA increases utrophin expression and stability, as well as its localization at the plasma membrane","volume":"391","author":"Bonet-Kerrache","year":"2005","journal-title":"Biochem. J."}],"container-title":["Fundamental Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S266732582200293X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S266732582200293X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,9]],"date-time":"2025-09-09T09:07:01Z","timestamp":1757408821000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S266732582200293X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11]]},"references-count":78,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2022,11]]}},"alternative-id":["S266732582200293X"],"URL":"https:\/\/doi.org\/10.1016\/j.fmre.2022.07.004","relation":{},"ISSN":["2667-3258"],"issn-type":[{"value":"2667-3258","type":"print"}],"subject":[],"published":{"date-parts":[[2022,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Promising therapeutic approaches of utrophin replacing dystrophin in the treatment of Duchenne muscular dystrophy","name":"articletitle","label":"Article Title"},{"value":"Fundamental Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.fmre.2022.07.004","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 The Authors. Publishing Services by Elsevier B.V. on behalf of KeAi Communications Co. Ltd.","name":"copyright","label":"Copyright"}]}}