{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T21:18:42Z","timestamp":1774646322099,"version":"3.50.1"},"reference-count":100,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2022,7,14]],"date-time":"2022-07-14T00:00:00Z","timestamp":1657756800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea (NRF)","doi-asserted-by":"publisher","award":["2021R1A2C3004938"],"award-info":[{"award-number":["2021R1A2C3004938"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea (NRF)","doi-asserted-by":"publisher","award":["PJ01573502"],"award-info":[{"award-number":["PJ01573502"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003627","name":"Cooperative Research Program for Agriculture Science and Technology Development","doi-asserted-by":"publisher","award":["2021R1A2C3004938"],"award-info":[{"award-number":["2021R1A2C3004938"]}],"id":[{"id":"10.13039\/501100003627","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003627","name":"Cooperative Research Program for Agriculture Science and Technology Development","doi-asserted-by":"publisher","award":["PJ01573502"],"award-info":[{"award-number":["PJ01573502"]}],"id":[{"id":"10.13039\/501100003627","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), which represent the range of dystrophinopathies, account for nearly 80% of muscle dystrophy. DMD and BMD result from the loss of a functional dystrophin protein, and the leading cause of death in these patients is cardiac remodeling and heart failure. The pathogenesis and progression of the more severe form of DMD have been extensively studied and are controlled by many determinants, including microRNAs (miRNAs). The regulatory role of miRNAs in muscle function and the differential miRNA expression in muscular dystrophy indicate the clinical significance of miRNAs. This review discusses the relevant microRNAs as potential biomarkers and therapeutic targets for DMD and DMD cardiomyopathy as examples of dystrophinopathies.<\/jats:p>","DOI":"10.3390\/ijms23147785","type":"journal-article","created":{"date-parts":[[2022,7,15]],"date-time":"2022-07-15T00:17:10Z","timestamp":1657844230000},"page":"7785","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["MicroRNAs in Dystrophinopathy"],"prefix":"10.3390","volume":"23","author":[{"given":"Ahyoung","family":"Lee","sequence":"first","affiliation":[{"name":"Korean Medicine Institute, School of Korean Medicine, Pusan National University, Yangsan 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiwon","family":"Moon","sequence":"additional","affiliation":[{"name":"Urban Agriculture Research Division, National Institute of Horticultural and Herbal Science, Jeollabuk-do 55365, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jin","family":"Yu","sequence":"additional","affiliation":[{"name":"Ginseng Division, Department of Herbal Crop Research, National Institute of Herbal Science, Chungbuk 27709, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7109-7506","authenticated-orcid":false,"given":"Changwon","family":"Kho","sequence":"additional","affiliation":[{"name":"Division of Applied Medicine, School of Korean Medicine, Pusan National University, Yangsan 50612, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1016\/j.ncl.2014.05.002","article-title":"Duchenne and Becker muscular dystrophies","volume":"32","author":"Flanigan","year":"2014","journal-title":"Neurol. Clin."},{"key":"ref_2","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":"ref_3","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/0888-7543(88)90113-9","article-title":"An explanation for the phenotypic differences between patients bearing partial deletions of the DMD locus","volume":"2","author":"Monaco","year":"1988","journal-title":"Genomics"},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Lim, K.R.Q., Sheri, N., Nguyen, Q., and Yokota, T. (2020). Cardiac Involvement in Dystrophin-Deficient Females: Current Understanding and Implications for the Treatment of Dystrophinopathies. Genes, 11.","DOI":"10.3390\/genes11070765"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1186\/s13018-022-02996-8","article-title":"Global prevalence of Duchenne and Becker muscular dystrophy: A systematic review and meta-analysis","volume":"17","author":"Salari","year":"2022","journal-title":"J. Orthop. Surg. Res."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/S1474-4422(09)70271-6","article-title":"Diagnosis and management of Duchenne muscular dystrophy, part 1: Diagnosis, and pharmacological and psychosocial management","volume":"9","author":"Bushby","year":"2010","journal-title":"Lancet Neurol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2116","DOI":"10.1016\/S0140-6736(98)10028-4","article-title":"Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in The Netherlands: A cohort study","volume":"353","author":"Hoogerwaard","year":"1999","journal-title":"Lancet"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1126\/science.3659917","article-title":"Conservation of the Duchenne muscular dystrophy gene in mice and humans","volume":"238","author":"Hoffman","year":"1987","journal-title":"Science"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1016\/S1474-4422(03)00585-4","article-title":"Dystrophin and mutations: One gene, several proteins, multiple phenotypes","volume":"2","author":"Muntoni","year":"2003","journal-title":"Lancet Neurol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1083\/jcb.135.3.661","article-title":"A new model for the interaction of dystrophin with F-actin","volume":"135","author":"Rybakova","year":"1996","journal-title":"J. Cell Biol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"27305","DOI":"10.1074\/jbc.270.45.27305","article-title":"Identification and characterization of the dystrophin anchoring site on beta-dystroglycan","volume":"270","author":"Jung","year":"1995","journal-title":"J. Biol. Chem."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"12573","DOI":"10.1073\/pnas.0906545106","article-title":"Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan","volume":"106","author":"Han","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_13","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. USA"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1038\/s41467-020-14895-9","article-title":"A signaling hub of insulin receptor, dystrophin glycoprotein complex and plakoglobin regulates muscle size","volume":"11","author":"Aweida","year":"2020","journal-title":"Nat. Commun."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1038\/nature22979","article-title":"Dystrophin-glycoprotein complex sequesters Yap to inhibit cardiomyocyte proliferation","volume":"547","author":"Morikawa","year":"2017","journal-title":"Nature"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1016\/j.cell.2012.04.005","article-title":"Roles for microRNAs in conferring robustness to biological processes","volume":"149","author":"Ebert","year":"2012","journal-title":"Cell"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1016\/S0092-8674(04)00045-5","article-title":"MicroRNAs: Genomics, biogenesis, mechanism, and function","volume":"116","author":"Bartel","year":"2004","journal-title":"Cell"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/j.jmb.2004.03.065","article-title":"Molecular evolution of a microRNA cluster","volume":"339","author":"Tanzer","year":"2004","journal-title":"J. Mol. Biol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1016\/j.cell.2009.01.002","article-title":"MicroRNAs: Target recognition and regulatory functions","volume":"136","author":"Bartel","year":"2009","journal-title":"Cell"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1089\/dna.2006.0545","article-title":"Evolutionary conservation of microRNA regulatory circuits: An examination of microRNA gene complexity and conserved microRNA-target interactions through metazoan phylogeny","volume":"26","author":"Lee","year":"2007","journal-title":"DNA Cell Biol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1172","DOI":"10.1016\/j.cell.2012.02.005","article-title":"MicroRNAs in stress signaling and human disease","volume":"148","author":"Mendell","year":"2012","journal-title":"Cell"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"3353","DOI":"10.1093\/nar\/gkz097","article-title":"An estimate of the total number of true human miRNAs","volume":"47","author":"Alles","year":"2019","journal-title":"Nucleic Acids Res."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/nrc1840","article-title":"Oncomirs\u2014microRNAs with a role in cancer","volume":"6","author":"Slack","year":"2006","journal-title":"Nat. Rev. Cancer"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1111\/j.1365-2141.2008.07077.x","article-title":"Detection of elevated levels of tumour-associated microRNAs in serum of patients with diffuse large B-cell lymphoma","volume":"141","author":"Lawrie","year":"2008","journal-title":"Br. J. Haematol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1148\/radiol.10091547","article-title":"T2 mapping in Duchenne muscular dystrophy: Distribution of disease activity and correlation with clinical assessments","volume":"255","author":"Kim","year":"2010","journal-title":"Radiology"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1016\/j.molmed.2017.09.002","article-title":"Biomarker Potential of Extracellular miRNAs in Duchenne Muscular Dystrophy","volume":"23","author":"Wood","year":"2017","journal-title":"Trends Mol. Med."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"R13","DOI":"10.1186\/gb-2004-5-3-r13","article-title":"Expression profiling of mammalian microRNAs uncovers a subset of brain-expressed microRNAs with possible roles in murine and human neuronal differentiation","volume":"5","author":"Sempere","year":"2004","journal-title":"Genome Biol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1126\/science.1139089","article-title":"Control of stress-dependent cardiac growth and gene expression by a microRNA","volume":"316","author":"Sutherland","year":"2007","journal-title":"Science"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1016\/j.bbagrm.2008.03.001","article-title":"MicroRNA-206: The skeletal muscle-specific myomiR","volume":"1779","author":"McCarthy","year":"2008","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1016\/j.devcel.2009.10.013","article-title":"A family of microRNAs encoded by myosin genes governs myosin expression and muscle performance","volume":"17","author":"Quiat","year":"2009","journal-title":"Dev. Cell"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1038\/ng1725","article-title":"The role of microRNA-1 and microRNA-133 in skeletal muscle proliferation and differentiation","volume":"38","author":"Chen","year":"2006","journal-title":"Nat. Genet."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1083\/jcb.200911036","article-title":"microRNA-1 and microRNA-206 regulate skeletal muscle satellite cell proliferation and differentiation by repressing Pax7","volume":"190","author":"Chen","year":"2010","journal-title":"J. Cell Biol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"17016","DOI":"10.1073\/pnas.0708115104","article-title":"Distinctive patterns of microRNA expression in primary muscular disorders","volume":"104","author":"Eisenberg","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"3335","DOI":"10.1096\/fj.08-128579","article-title":"Common micro-RNA signature in skeletal muscle damage and regeneration induced by Duchenne muscular dystrophy and acute ischemia","volume":"23","author":"Greco","year":"2009","journal-title":"FASEB J."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e39","DOI":"10.1038\/mtna.2012.26","article-title":"Expression analysis in multiple muscle groups and serum reveals complexity in the microRNA transcriptome of the mdx mouse with implications for therapy","volume":"1","author":"Roberts","year":"2012","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1038\/embor.2010.208","article-title":"miR-31 modulates dystrophin expression: New implications for Duchenne muscular dystrophy therapy","volume":"12","author":"Cacchiarelli","year":"2011","journal-title":"EMBO Rep."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1002\/emmm.201100133","article-title":"miRNAs as serum biomarkers for Duchenne muscular dystrophy","volume":"3","author":"Cacchiarelli","year":"2011","journal-title":"EMBO Mol. Med."},{"key":"ref_38","doi-asserted-by":"crossref","unstructured":"Anaya-Segura, M.A., Rangel-Villalobos, H., Martinez-Cortes, G., Gomez-Diaz, B., Coral-Vazquez, R.M., Zamora-Gonzalez, E.O., Garcia, S., and Lopez-Hernandez, L.B. (2016). Serum Levels of MicroRNA-206 and Novel Mini-STR Assays for Carrier Detection in Duchenne Muscular Dystrophy. Int. J. Mol. Sci., 17.","DOI":"10.3390\/ijms17081334"},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Zaharieva, I.T., Calissano, M., Scoto, M., Preston, M., Cirak, S., Feng, L., Collins, J., Kole, R., Guglieri, M., and Straub, V. (2013). Dystromirs as serum biomarkers for monitoring the disease severity in Duchenne muscular Dystrophy. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0080263"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.nmd.2016.11.003","article-title":"Digital PCR quantification of miR-30c and miR-181a as serum biomarkers for Duchenne muscular dystrophy","volume":"27","author":"Ortez","year":"2017","journal-title":"Neuromuscul. Disord."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"2885","DOI":"10.1016\/j.ajpath.2014.07.021","article-title":"Serum profiling identifies novel muscle miRNA and cardiomyopathy-related miRNA biomarkers in Golden Retriever muscular dystrophy dogs and Duchenne muscular dystrophy patients","volume":"184","author":"Lameth","year":"2014","journal-title":"Am. J. Pathol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"304","DOI":"10.3389\/fneur.2020.00304","article-title":"Longitudinal Study of Three microRNAs in Duchenne Muscular Dystrophy and Becker Muscular Dystrophy","volume":"11","author":"Trifunov","year":"2020","journal-title":"Front. Neurol."},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"Mizuno, H., Nakamura, A., Aoki, Y., Ito, N., Kishi, S., Yamamoto, K., Sekiguchi, M., Takeda, S., and Hashido, K. (2011). Identification of muscle-specific microRNAs in serum of muscular dystrophy animal models: Promising novel blood-based markers for muscular dystrophy. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0018388"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"e177","DOI":"10.1038\/mtna.2014.29","article-title":"Circulating Muscle-specific miRNAs in Duchenne Muscular Dystrophy Patients","volume":"3","author":"Li","year":"2014","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1567","DOI":"10.1161\/CIRCULATIONAHA.108.769984","article-title":"Conditional dicer gene deletion in the postnatal myocardium provokes spontaneous cardiac remodeling","volume":"118","author":"Bourajjaj","year":"2008","journal-title":"Circulation"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1161\/CIRCRESAHA.109.200451","article-title":"Loss of cardiac microRNA-mediated regulation leads to dilated cardiomyopathy and heart failure","volume":"105","author":"Rao","year":"2009","journal-title":"Circ. Res."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1038\/nm1569","article-title":"The muscle-specific microRNA miR-1 regulates cardiac arrhythmogenic potential by targeting GJA1 and KCNJ2","volume":"13","author":"Yang","year":"2007","journal-title":"Nat. Med."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1161\/CIRCRESAHA.108.181651","article-title":"miR-1 overexpression enhances Ca(2+) release and promotes cardiac arrhythmogenesis by targeting PP2A regulatory subunit B56alpha and causing CaMKII-dependent hyperphosphorylation of RyR2","volume":"104","author":"Terentyev","year":"2009","journal-title":"Circ. Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1038\/nm1582","article-title":"MicroRNA-133 controls cardiac hypertrophy","volume":"13","author":"Care","year":"2007","journal-title":"Nat. Med."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"e000078","DOI":"10.1161\/JAHA.113.000078","article-title":"Therapeutic cardiac-targeted delivery of miR-1 reverses pressure overload-induced cardiac hypertrophy and attenuates pathological remodeling","volume":"2","author":"Karakikes","year":"2013","journal-title":"J. Am. Heart Assoc."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1161\/CIRCRESAHA.108.182535","article-title":"miR-133 and miR-30 regulate connective tissue growth factor: Implications for a role of microRNAs in myocardial matrix remodeling","volume":"104","author":"Duisters","year":"2009","journal-title":"Circ. Res."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1002\/jcp.22939","article-title":"MiR-133a regulates collagen 1A1: Potential role of miR-133a in myocardial fibrosis in angiotensin II-dependent hypertension","volume":"227","author":"Castoldi","year":"2012","journal-title":"J. Cell Physiol."},{"key":"ref_53","first-page":"8588","article-title":"Role of miR-133a in regulating TGF-beta1 signaling pathway in myocardial fibrosis after acute myocardial infarction in rats","volume":"23","author":"Yu","year":"2019","journal-title":"Eur. Rev. Med. Pharmacol. Sci."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1016\/j.cell.2011.09.028","article-title":"A long noncoding RNA controls muscle differentiation by functioning as a competing endogenous RNA","volume":"147","author":"Cesana","year":"2011","journal-title":"Cell"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"2772","DOI":"10.1172\/JCI36154","article-title":"MicroRNA-208a is a regulator of cardiac hypertrophy and conduction in mice","volume":"119","author":"Callis","year":"2009","journal-title":"J. Clin. Invest."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1093\/eurheartj\/ehq013","article-title":"Circulating microRNA: A novel potential biomarker for early diagnosis of acute myocardial infarction in humans","volume":"31","author":"Wang","year":"2010","journal-title":"Eur. Heart J."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1002\/ejhf.2096","article-title":"Circulating cardiovascular microRNAs in critically ill COVID-19 patients","volume":"23","author":"Garg","year":"2021","journal-title":"Eur. J. Heart Fail."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/j.hrtlng.2020.07.010","article-title":"Diagnostic value of circulating microRNA-208a in differentiation of preserved from reduced ejection fraction heart failure","volume":"50","author":"Li","year":"2021","journal-title":"Heart Lung"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"2347","DOI":"10.1093\/hmg\/ddab199","article-title":"Cardiomyocyte-produced miR-339-5p mediates pathology in Duchenne muscular dystrophy cardiomyopathy","volume":"30","author":"Gartz","year":"2021","journal-title":"Hum. Mol. Genet."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"2545","DOI":"10.2169\/internalmedicine.2714-19","article-title":"Rapidly Progressive Heart Failure in a Female Carrier of Becker Muscular Dystrophy with No Skeletal Muscle Symptoms","volume":"58","author":"Komoriyama","year":"2019","journal-title":"Intern. Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1016\/j.ijcard.2008.06.084","article-title":"Cardiac involvement in a female carrier of Duchenne muscular dystrophy","volume":"138","author":"Walcher","year":"2010","journal-title":"Int. J. Cardiol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"95","DOI":"10.5582\/irdr.2017.01024","article-title":"Heart transplantation in patients with dystrophinopathic cardiomyopathy: Review of the literature and personal series","volume":"6","author":"Papa","year":"2017","journal-title":"Intractable Rare Dis. Res."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1770","DOI":"10.3389\/fphys.2018.01770","article-title":"Identification of Cardiomyopathy-Associated Circulating miRNA Biomarkers in Muscular Dystrophy Female Carriers Using a Complementary Cardiac Imaging and Plasma Profiling Approach","volume":"9","author":"Florian","year":"2018","journal-title":"Front. Physiol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1186\/s12968-016-0244-3","article-title":"Identification of cardiomyopathy associated circulating miRNA biomarkers in patients with muscular dystrophy using a complementary cardiovascular magnetic resonance and plasma profiling approach","volume":"18","author":"Becker","year":"2016","journal-title":"J. Cardiovasc. Magn. Reson."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"875","DOI":"10.2217\/epi-2018-0022","article-title":"Downregulation of miRNA-29, -23 and -21 in urine of Duchenne muscular dystrophy patients","volume":"10","author":"Catapano","year":"2018","journal-title":"Epigenomics"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.stem.2012.03.011","article-title":"Muscle satellite cells are primed for myogenesis but maintain quiescence with sequestration of Myf5 mRNA targeted by microRNA-31 in mRNP granules","volume":"11","author":"Crist","year":"2012","journal-title":"Cell Stem Cell"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"340ra374","DOI":"10.1126\/scitranslmed.aac4296","article-title":"Up-regulation of miR-31 in human atrial fibrillation begets the arrhythmia by depleting dystrophin and neuronal nitric oxide synthase","volume":"8","author":"Reilly","year":"2016","journal-title":"Sci. Transl. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.yjmcc.2017.08.013","article-title":"MicroRNA-31 promotes adverse cardiac remodeling and dysfunction in ischemic heart disease","volume":"112","author":"Martinez","year":"2017","journal-title":"J. Mol. Cell Cardiol."},{"key":"ref_69","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":"ref_70","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":"ref_71","doi-asserted-by":"crossref","unstructured":"Banks, G.B., Combs, A.C., Odom, G.L., Bloch, R.J., and Chamberlain, J.S. (2014). Muscle structure influences utrophin expression in mdx mice. PLoS Genet., 10.","DOI":"10.1371\/journal.pgen.1004431"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"1623","DOI":"10.1093\/hmg\/ddl083","article-title":"Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophy","volume":"15","author":"Kleopa","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"ref_73","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":"ref_74","doi-asserted-by":"crossref","unstructured":"Basu, U., Lozynska, O., Moorwood, C., Patel, G., Wilton, S.D., and Khurana, T.S. (2011). Translational regulation of utrophin by miRNAs. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0029376"},{"key":"ref_75","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":"ref_76","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":"Fontelonga","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1161\/CIRCRESAHA.115.306624","article-title":"miR-206 Mediates YAP-Induced Cardiac Hypertrophy and Survival","volume":"117","author":"Yang","year":"2015","journal-title":"Circ. Res."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1001\/jamacardio.2017.1048","article-title":"Myocardial Fibrosis in Duchenne and Becker Muscular Dystrophy-Reply","volume":"2","author":"Silva","year":"2017","journal-title":"JAMA Cardiol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1016\/j.bbadis.2015.04.013","article-title":"Opposing roles of miR-21 and miR-29 in the progression of fibrosis in Duchenne muscular dystrophy","volume":"1852","author":"Zanotti","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"563166","DOI":"10.3389\/fgene.2020.563166","article-title":"The Cell Type-Specific Functions of miR-21 in Cardiovascular Diseases","volume":"11","author":"Dai","year":"2020","journal-title":"Front. Genet."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"767064","DOI":"10.3389\/fcvm.2021.767064","article-title":"miR-21 in Human Cardiomyopathies","volume":"8","author":"Surina","year":"2021","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1222","DOI":"10.1038\/mt.2012.35","article-title":"Loss of miR-29 in myoblasts contributes to dystrophic muscle pathogenesis","volume":"20","author":"Wang","year":"2012","journal-title":"Mol. Ther."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"e93309","DOI":"10.1172\/jci.insight.93309","article-title":"MicroRNA-29 overexpression by adeno-associated virus suppresses fibrosis and restores muscle function in combination with micro-dystrophin","volume":"2","author":"Heller","year":"2017","journal-title":"JCI Insight"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"105510","DOI":"10.1016\/j.phrs.2021.105510","article-title":"miR-29 family: A potential therapeutic target for cardiovascular disease","volume":"166","author":"Liu","year":"2021","journal-title":"Pharmacol. Res."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"647010","DOI":"10.3389\/fphys.2021.647010","article-title":"Abnormal Calcium Handling in Duchenne Muscular Dystrophy: Mechanisms and Potential Therapies","volume":"12","author":"Mareedu","year":"2021","journal-title":"Front. Physiol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"2159","DOI":"10.1021\/bi00655a020","article-title":"A Ca2+-activated protease possibly involved in myofibrillar protein turnover. Partial characterization of the purified enzyme","volume":"15","author":"Dayton","year":"1976","journal-title":"Biochemistry"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/0009-8981(76)90175-3","article-title":"A calcium-activated neutral protease in normal and dystrophic human muscle","volume":"73","author":"Kar","year":"1976","journal-title":"Clin. Chim. Acta"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1016\/j.yjmcc.2008.08.012","article-title":"Calpain mediates cardiac troponin degradation and contractile dysfunction in atrial fibrillation","volume":"45","author":"Ke","year":"2008","journal-title":"J. Mol. Cell Cardiol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"2115","DOI":"10.1016\/j.biocel.2004.12.012","article-title":"Calpains in muscle wasting","volume":"37","author":"Bartoli","year":"2005","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"2197","DOI":"10.1242\/jcs.108.6.2197","article-title":"Apoptosis precedes necrosis of dystrophin-deficient muscle","volume":"108","author":"Tidball","year":"1995","journal-title":"J. Cell Sci."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"3655","DOI":"10.1038\/s41467-018-06057-9","article-title":"Necroptosis mediates myofibre death in dystrophin-deficient mice","volume":"9","author":"Morgan","year":"2018","journal-title":"Nat. Commun."},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Law, M.L., Cohen, H., Martin, A.A., Angulski, A.B.B., and Metzger, J.M. (2020). Dysregulation of Calcium Handling in Duchenne Muscular Dystrophy-Associated Dilated Cardiomyopathy: Mechanisms and Experimental Therapeutic Strategies. J. Clin. Med., 9.","DOI":"10.3390\/jcm9020520"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1038\/nm.1916","article-title":"Hypernitrosylated ryanodine receptor calcium release channels are leaky in dystrophic muscle","volume":"15","author":"Bellinger","year":"2009","journal-title":"Nat. Med."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1096\/fj.201700182RRR","article-title":"Ryanodine channel complex stabilizer compound S48168\/ARM210 as a disease modifier in dystrophin-deficient mdx mice: Proof-of-concept study and independent validation of efficacy","volume":"32","author":"Capogrosso","year":"2018","journal-title":"FASEB J."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1016\/j.omtn.2019.09.020","article-title":"Targeting RyR Activity Boosts Antisense Exon 44 and 45 Skipping in Human DMD Skeletal or Cardiac Muscle Culture Models","volume":"18","author":"Barthelemy","year":"2019","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1016\/j.ymthe.2019.12.011","article-title":"Single SERCA2a Therapy Ameliorated Dilated Cardiomyopathy for 18 Months in a Mouse Model of Duchenne Muscular Dystrophy","volume":"28","author":"Wasala","year":"2020","journal-title":"Mol. Ther."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1038\/nature13073","article-title":"Inhibition of miR-25 improves cardiac contractility in the failing heart","volume":"508","author":"Wahlquist","year":"2014","journal-title":"Nature"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"1178","DOI":"10.1016\/S0140-6736(16)00082-9","article-title":"Calcium upregulation by percutaneous administration of gene therapy in patients with cardiac disease (CUPID 2): A randomised, multinational, double-blind, placebo-controlled, phase 2b trial","volume":"387","author":"Greenberg","year":"2016","journal-title":"Lancet"},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1038\/s41434-020-0171-7","article-title":"Investigation of the safety and feasibility of AAV1\/SERCA2a gene transfer in patients with chronic heart failure supported with a left ventricular assist device\u2014The SERCA-LVAD TRIAL","volume":"27","author":"Lyon","year":"2020","journal-title":"Gene Ther."},{"key":"ref_100","doi-asserted-by":"crossref","unstructured":"Park, J.H., and Kho, C. (2021). MicroRNAs and Calcium Signaling in Heart Disease. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms221910582"}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/14\/7785\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:50:29Z","timestamp":1760140229000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/14\/7785"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,14]]},"references-count":100,"journal-issue":{"issue":"14","published-online":{"date-parts":[[2022,7]]}},"alternative-id":["ijms23147785"],"URL":"https:\/\/doi.org\/10.3390\/ijms23147785","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,7,14]]}}}