{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T15:02:51Z","timestamp":1790521371646,"version":"4.1.0"},"reference-count":59,"publisher":"The Company of Biologists","issue":"2","license":[{"start":{"date-parts":[[2021,3,23]],"date-time":"2021-03-23T00:00:00Z","timestamp":1616457600000},"content-version":"vor","delay-in-days":50,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Austrian Science Fund","award":["P30234-B27"],"award-info":[{"award-number":["P30234-B27"]}]},{"DOI":"10.13039\/501100016168","name":"Ludwig Boltzmann Society","doi-asserted-by":"crossref","award":["REM 2017-20"],"award-info":[{"award-number":["REM 2017-20"]}],"id":[{"id":"10.13039\/501100016168","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,2,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Besides skeletal muscle abnormalities, Duchenne muscular dystrophy (DMD) patients present with dilated cardiomyopathy development, which considerably contributes to morbidity and mortality. Because the mechanisms responsible for the cardiac complications in the context of DMD are largely unknown, evidence-based therapy approaches are still lacking. This has increased the need for basic research efforts into animal models for DMD. Here, we characterized in detail the cardiovascular abnormalities of Dmdmdx rats, with the aim of determining the suitability of this recently established dystrophin-deficient small animal as a model for DMD.<\/jats:p>\n               <jats:p>Various methods were applied to compare cardiovascular properties between wild-type and Dmdmdx rats, and to characterize the Dmdmdx cardiomyopathy. These methods comprised echocardiography, invasive assessment of left ventricular hemodynamics, examination of adverse remodeling and endothelial cell inflammation, and evaluation of vascular function, employing wire myography. Finally, intracellular Ca2+ transient measurements, and recordings of currents through L-type Ca2+ channels were performed in isolated single ventricular cardiomyocytes. We found that, similar to respective observations in DMD patients, the hearts of Dmdmdx rats show significantly impaired cardiac function, fibrosis and inflammation, consistent with the development of a dilated cardiomyopathy. Moreover, in Dmdmdx rats, vascular endothelial function is impaired, which may relate to inflammation and oxidative stress, and Ca2+ handling in Dmdmdx cardiomyocytes is abnormal.<\/jats:p>\n               <jats:p>These findings indicate that Dmdmdx rats represent a promising small-animal model to elucidate mechanisms of cardiomyopathy development in the dystrophic heart, and to test mechanism-based therapies aiming to combat cardiovascular complications in DMD.<\/jats:p>","DOI":"10.1242\/dmm.047704","type":"journal-article","created":{"date-parts":[[2021,2,22]],"date-time":"2021-02-22T13:10:25Z","timestamp":1613999425000},"source":"Crossref","is-referenced-by-count":37,"title":["Cardiovascular phenotype of the <i>Dmdmdx<\/i> rat \u2013 a suitable animal model for Duchenne muscular dystrophy"],"prefix":"10.1242","volume":"14","author":[{"given":"Petra Lujza","family":"Szab\u00f3","sequence":"first","affiliation":[{"name":"Ludwig Boltzmann Institute for Cardiovascular Research at Center for Biomedical Research, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janine","family":"Ebner","sequence":"additional","affiliation":[{"name":"Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xaver","family":"Koenig","sequence":"additional","affiliation":[{"name":"Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ouafa","family":"Hamza","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Cardiovascular Research at Center for Biomedical Research, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Watzinger","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Cardiovascular Research at Center for Biomedical Research, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Trojanek","sequence":"additional","affiliation":[{"name":"Center for Anatomy and Cell Biology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dietmar","family":"Abraham","sequence":"additional","affiliation":[{"name":"Center for Anatomy and Cell Biology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannes","family":"Todt","sequence":"additional","affiliation":[{"name":"Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helmut","family":"Kubista","sequence":"additional","affiliation":[{"name":"Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Schicker","sequence":"additional","affiliation":[{"name":"Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9verine","family":"Remy","sequence":"additional","affiliation":[{"name":"INSERM, Center for Research in Transplantation and Immunology, UMR 1064, Nantes Universit\u00e9, F-44000 Nantes, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ignacio","family":"Anegon","sequence":"additional","affiliation":[{"name":"INSERM, Center for Research in Transplantation and Immunology, UMR 1064, Nantes Universit\u00e9, F-44000 Nantes, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4652-1998","authenticated-orcid":false,"given":"Attila","family":"Kiss","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Cardiovascular Research at Center for Biomedical Research, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno K.","family":"Podesser","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Cardiovascular Research at Center for Biomedical Research, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karlheinz","family":"Hilber","sequence":"additional","affiliation":[{"name":"Department of Neurophysiology and Pharmacology, Center for Physiology and Pharmacology, Medical University of Vienna, Vienna 1090, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2021,2,22]]},"reference":[{"key":"2021042701072720000_DMM047704C1","doi-asserted-by":"publisher","first-page":"290","DOI":"10.1016\/S0008-6363(97)00272-1","article-title":"Alterations in calcium handling in cardiac hypertrophy and heart failure","volume":"37","author":"Balke","year":"1998","journal-title":"Cardiovasc. Res."},{"key":"2021042701072720000_DMM047704C2","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1023\/A:1025473606484","article-title":"New perspectives on the key role of calcium in the progression of heart disease","volume":"24","author":"Benitah","year":"2003","journal-title":"J. Muscle Res. Cell Motil."},{"key":"2021042701072720000_DMM047704C3","doi-asserted-by":"publisher","first-page":"1046","DOI":"10.1161\/01.CIR.85.3.1046","article-title":"Intracellular calcium handling in isolated ventricular myocytes from patients with terminal heart failure","volume":"85","author":"Beuckelmann","year":"1992","journal-title":"Circulation"},{"key":"2021042701072720000_DMM047704C4","doi-asserted-by":"publisher","first-page":"684","DOI":"10.1016\/0002-8703(95)90316-X","article-title":"Altered diastolic [Ca2+]i handling in human ventricular myocytes from patients with terminal heart failure","volume":"129","author":"Beuckelmann","year":"1995","journal-title":"Am. Heart J."},{"key":"2021042701072720000_DMM047704C5","doi-asserted-by":"publisher","first-page":"1345","DOI":"10.1161\/CIRCULATIONAHA.108.846402","article-title":"Neuronal nitric oxide synthase protects against myocardial infarction-induced ventricular arrhythmia and mortality in mice","volume":"120","author":"Burger","year":"2009","journal-title":"Circulation"},{"key":"2021042701072720000_DMM047704C6","doi-asserted-by":"publisher","first-page":"1971","DOI":"10.1038\/nprot.2006.306","article-title":"A continuous fluorescence resonance energy transfer angiotensin I-converting enzyme assay","volume":"1","author":"Carmona","year":"2006","journal-title":"Nat. Protoc."},{"key":"2021042701072720000_DMM047704C7","first-page":"S2","article-title":"ACE inhibition and vascular remodeling of resistance vessels: vascular compliance and cardiovascular implications","volume":"2","author":"Cohn","year":"2000","journal-title":"Heart Dis."},{"key":"2021042701072720000_DMM047704C8","doi-asserted-by":"publisher","first-page":"1006","DOI":"10.1016\/j.nmd.2012.05.002","article-title":"Cardiomyopathy in the dystrophin\/utrophin-deficient mouse model of severe muscular dystrophy is characterized by dysregulation of matrix metalloproteinases","volume":"22","author":"Delfin","year":"2012","journal-title":"Neuromuscul. Disord."},{"key":"2021042701072720000_DMM047704C9","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1186\/2044-5040-3-9","article-title":"Vascular-targeted therapies for Duchenne muscular dystrophy","volume":"3","author":"Ennen","year":"2013","journal-title":"Skelet Muscle"},{"key":"2021042701072720000_DMM047704C10","doi-asserted-by":"publisher","first-page":"e87844","DOI":"10.1371\/journal.pone.0087844","article-title":"New perspectives in the renin-angiotensin-aldosterone system (RAAS) II: albumin suppresses angiotensin converting enzyme (ACE) activity in human","volume":"9","author":"Fagyas","year":"2014","journal-title":"PLoS ONE"},{"key":"2021042701072720000_DMM047704C11","doi-asserted-by":"publisher","first-page":"1559","DOI":"10.1073\/pnas.0908540107","article-title":"Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy","volume":"107","author":"Fauconnier","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2021042701072720000_DMM047704C12","doi-asserted-by":"publisher","first-page":"538","DOI":"10.1542\/peds.2010-0208","article-title":"Cardiac findings in congenital muscular dystrophies","volume":"126","author":"Finsterer","year":"2010","journal-title":"Pediatrics"},{"key":"2021042701072720000_DMM047704C13","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1007\/5584_2017_106","article-title":"New insights in cardiac calcium handling and excitation-contraction coupling","volume":"1067","author":"Gambardella","year":"2018","journal-title":"Adv. Exp. Med. Biol."},{"key":"2021042701072720000_DMM047704C14","doi-asserted-by":"publisher","first-page":"H710","DOI":"10.1152\/ajpheart.00890.2013","article-title":"NADPH oxidase-2 inhibition restores contractility and intracellular calcium handling and reduces arrhythmogenicity in dystrophic cardiomyopathy","volume":"307","author":"Gonzalez","year":"2014","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"2021042701072720000_DMM047704C15","doi-asserted-by":"publisher","first-page":"729","DOI":"10.1016\/S0092-8674(00)80533-4","article-title":"Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy","volume":"90","author":"Grady","year":"1997","journal-title":"Cell"},{"key":"2021042701072720000_DMM047704C16","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1016\/j.scr.2013.12.004","article-title":"Dystrophin-deficient cardiomyocytes derived from human urine: new biologic reagents for drug discovery","volume":"12","author":"Guan","year":"2014","journal-title":"Stem Cell Res"},{"key":"2021042701072720000_DMM047704C17","doi-asserted-by":"publisher","first-page":"e0117306","DOI":"10.1371\/journal.pone.0117306","article-title":"Skeletal muscle fibrosis in the mdx\/utrn+\/- mouse validates its suitability as a murine model of Duchenne muscular dystrophy","volume":"10","author":"Gutpell","year":"2015","journal-title":"PLoS ONE"},{"key":"2021042701072720000_DMM047704C18","doi-asserted-by":"publisher","first-page":"A15","DOI":"10.1016\/j.jacc.2006.06.070","article-title":"Reversal of calcium cycling defects in advanced heart failure toward molecular therapy","volume":"48","author":"Hoshijima","year":"2006","journal-title":"J. Am. Coll. Cardiol."},{"key":"2021042701072720000_DMM047704C19","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1017\/S1047951119002610","article-title":"Left ventricular dysfunction in Duchenne muscular dystrophy","volume":"30","author":"James","year":"2020","journal-title":"Cardiol. Young"},{"key":"2021042701072720000_DMM047704C20","doi-asserted-by":"publisher","first-page":"H2373","DOI":"10.1152\/ajpheart.00448.2005","article-title":"Utrophin deficiency worsens cardiac contractile dysfunction present in dystrophin-deficient mdx mice","volume":"289","author":"Janssen","year":"2005","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"2021042701072720000_DMM047704C21","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1111\/j.1745-7599.2009.00404.x","article-title":"Current understanding and management of dilated cardiomyopathy in Duchenne and Becker muscular dystrophy","volume":"21","author":"Kaspar","year":"2009","journal-title":"J. Am. Acad. Nurse Pract."},{"key":"2021042701072720000_DMM047704C22","doi-asserted-by":"publisher","first-page":"e20300","DOI":"10.1371\/journal.pone.0020300","article-title":"Voltage-gated ion channel dysfunction precedes cardiomyopathy development in the dystrophic heart","volume":"6","author":"Koenig","year":"2011","journal-title":"PLoS ONE"},{"key":"2021042701072720000_DMM047704C23","doi-asserted-by":"publisher","first-page":"15565","DOI":"10.1073\/pnas.1002178107","article-title":"NADPH oxidase 4 (Nox4) is a major source of oxidative stress in the failing heart","volume":"107","author":"Kuroda","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2021042701072720000_DMM047704C24","doi-asserted-by":"publisher","first-page":"e110371","DOI":"10.1371\/journal.pone.0110371","article-title":"Characterization of dystrophin deficient rats: a new model for Duchenne muscular dystrophy","volume":"9","author":"Larcher","year":"2014","journal-title":"PLoS ONE"},{"key":"2021042701072720000_DMM047704C25","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1093\/cvr\/cvu119","article-title":"Blunted cardiac beta-adrenergic response as an early indication of cardiac dysfunction in Duchenne muscular dystrophy","volume":"103","author":"Li","year":"2014","journal-title":"Cardiovasc. Res."},{"key":"2021042701072720000_DMM047704C26","doi-asserted-by":"publisher","first-page":"743","DOI":"10.1006\/jmcc.1997.0626","article-title":"Calcium content of the sarcoplasmic reticulum in isolated ventricular myocytes from patients with terminal heart failure","volume":"30","author":"Lindner","year":"1998","journal-title":"J. Mol. Cell. Cardiol."},{"key":"2021042701072720000_DMM047704C27","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1242\/dmm.018424","article-title":"Animal models of Duchenne muscular dystrophy: from basic mechanisms to gene therapy","volume":"8","author":"McGreevy","year":"2015","journal-title":"Dis. Model. Mech."},{"key":"2021042701072720000_DMM047704C28","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1016\/0022-510X(87)90007-4","article-title":"Vascular endothelial cell injury and platelet embolism in Duchenne muscular dystrophy at the preclinical stage","volume":"82","author":"Miike","year":"1987","journal-title":"J. Neurol. Sci."},{"key":"2021042701072720000_DMM047704C29","doi-asserted-by":"publisher","first-page":"2085","DOI":"10.1212\/WNL.0000000000000498","article-title":"PDE5 inhibition alleviates functional muscle ischemia in boys with Duchenne muscular dystrophy","volume":"82","author":"Nelson","year":"2014","journal-title":"Neurology"},{"key":"2021042701072720000_DMM047704C30","doi-asserted-by":"publisher","first-page":"1533","DOI":"10.1016\/j.cca.2011.04.033","article-title":"Circulating tenascin-C levels in patients with dilated cardiomyopathy in the course of Emery-Dreifuss muscular dystrophy","volume":"412","author":"Niebroj-Dobosz","year":"2011","journal-title":"Clin. Chim. Acta"},{"key":"2021042701072720000_DMM047704C31","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1016\/0167-5273(90)90082-G","article-title":"The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy","volume":"26","author":"Nigro","year":"1990","journal-title":"Int. J. Cardiol."},{"key":"2021042701072720000_DMM047704C32","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1186\/s12968-016-0292-8","article-title":"Native T1 values identify myocardial changes and stratify disease severity in patients with Duchenne muscular dystrophy","volume":"18","author":"Olivieri","year":"2016","journal-title":"J. Cardiovasc. Magn. Reson."},{"key":"2021042701072720000_DMM047704C33","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1172\/JCI121297","article-title":"Endothelial progerin expression causes cardiovascular pathology through an impaired mechanoresponse","volume":"129","author":"Osmanagic-Myers","year":"2019","journal-title":"J. Clin. Invest."},{"key":"2021042701072720000_DMM047704C34","doi-asserted-by":"publisher","first-page":"2131","DOI":"10.3389\/fimmu.2019.02131","article-title":"Immunophenotype of a rat model of Duchenne's disease and demonstration of improved muscle strength after anti-CD45RC antibody treatment","volume":"10","author":"Ouisse","year":"2019","journal-title":"Front. Immunol."},{"key":"2021042701072720000_DMM047704C35","doi-asserted-by":"publisher","first-page":"1169","DOI":"10.1161\/01.CIR.92.5.1169","article-title":"Alterations in intracellular calcium handling associated with the inverse force-frequency relation in human dilated cardiomyopathy","volume":"92","author":"Pieske","year":"1995","journal-title":"Circulation"},{"key":"2021042701072720000_DMM047704C36","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1016\/j.ijcard.2019.03.003","article-title":"Remote ischemic perconditioning attenuates adverse cardiac remodeling and preserves left ventricular function in a rat model of reperfused myocardial infarction","volume":"285","author":"Pilz","year":"2019","journal-title":"Int. J. Cardiol."},{"key":"2021042701072720000_DMM047704C37","doi-asserted-by":"publisher","first-page":"847","DOI":"10.1097\/HJH.0000000000001628","article-title":"Tenascin-C promotes chronic pressure overload-induced cardiac dysfunction, hypertrophy and myocardial fibrosis","volume":"36","author":"Podesser","year":"2018","journal-title":"J. Hypertens."},{"key":"2021042701072720000_DMM047704C38","doi-asserted-by":"publisher","first-page":"491","DOI":"10.1016\/j.nmd.2004.04.007","article-title":"Evolution of the mdx mouse cardiomyopathy: physiological and morphological findings","volume":"14","author":"Quinlan","year":"2004","journal-title":"Neuromuscul. Disord."},{"key":"2021042701072720000_DMM047704C39","doi-asserted-by":"publisher","first-page":"e13567","DOI":"10.14814\/phy2.13567","article-title":"Calcium current properties in dystrophin-deficient ventricular cardiomyocytes from aged mdx mice","volume":"6","author":"Rubi","year":"2018","journal-title":"Physiol Rep"},{"key":"2021042701072720000_DMM047704C40","doi-asserted-by":"publisher","first-page":"330","DOI":"10.1016\/j.tcm.2017.12.006","article-title":"ACE inhibition to slow progression of myocardial fibrosis in muscular dystrophies","volume":"28","author":"Russo","year":"2018","journal-title":"Trends Cardiovasc. Med."},{"key":"2021042701072720000_DMM047704C41","doi-asserted-by":"publisher","first-page":"1269","DOI":"10.1007\/s00246-017-1657-y","article-title":"Central arterial function measured by non-invasive pulse wave analysis is abnormal in patients with duchenne muscular dystrophy","volume":"38","author":"Ryan","year":"2017","journal-title":"Pediatr. Cardiol."},{"key":"2021042701072720000_DMM047704C42","doi-asserted-by":"crossref","first-page":"2113","DOI":"10.1002\/ehf2.12794","article-title":"Tenascin-C aggravates ventricular dilatation and angiotensin-converting enzyme activity after myocardial infarction in mice","volume":"7","author":"Santer","year":"2020","journal-title":"ESC Heart Fail"},{"key":"2021042701072720000_DMM047704C43","doi-asserted-by":"publisher","first-page":"2319","DOI":"10.1016\/j.jacc.2006.03.033","article-title":"Serum tenascin-C might be a novel predictor of left ventricular remodeling and prognosis after acute myocardial infarction","volume":"47","author":"Sato","year":"2006","journal-title":"J. Am. Coll. Cardiol."},{"key":"2021042701072720000_DMM047704C44","doi-asserted-by":"publisher","first-page":"e52","DOI":"10.1161\/01.RES.0000064585.95749.6D","article-title":"Cardiac neuronal nitric oxide synthase isoform regulates myocardial contraction and calcium handling","volume":"92","author":"Sears","year":"2003","journal-title":"Circ. Res."},{"key":"2021042701072720000_DMM047704C45","doi-asserted-by":"publisher","first-page":"1263","DOI":"10.1139\/cjpp-2016-0634","article-title":"Transcriptional regulation of endothelial-to-mesenchymal transition in cardiac fibrosis: role of myocardin-related transcription factor A and activating transcription factor 3","volume":"95","author":"Sharma","year":"2017","journal-title":"Can. J. Physiol. Pharmacol."},{"key":"2021042701072720000_DMM047704C46","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1016\/j.yjmcc.2012.12.009","article-title":"Cardiac phenotype of duchenne muscular dystrophy: insights from cellular studies","volume":"58","author":"Shirokova","year":"2013","journal-title":"J. Mol. Cell. Cardiol."},{"key":"2021042701072720000_DMM047704C47","doi-asserted-by":"publisher","first-page":"1578","DOI":"10.1126\/science.2662404","article-title":"The molecular basis of muscular dystrophy in the mdx mouse: a point mutation","volume":"244","author":"Sicinski","year":"1989","journal-title":"Science"},{"key":"2021042701072720000_DMM047704C48","doi-asserted-by":"publisher","first-page":"190","DOI":"10.1001\/jamacardio.2016.4801","article-title":"Myocardial fibrosis progression in duchenne and becker muscular dystrophy: a randomized clinical trial","volume":"2","author":"Silva","year":"2017","journal-title":"JAMA Cardiol"},{"key":"2021042701072720000_DMM047704C49","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1002\/mus.22097","article-title":"Cardiomyopathy of Duchenne muscular dystrophy: current understanding and future directions","volume":"44","author":"Spurney","year":"2011","journal-title":"Muscle Nerve"},{"key":"2021042701072720000_DMM047704C50","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1016\/j.mce.2015.01.012","article-title":"Endocannabinoid-mediated modulation of Gq\/11 protein-coupled receptor signaling-induced vasoconstriction and hypertension","volume":"403","author":"Szekeres","year":"2015","journal-title":"Mol. Cell. Endocrinol."},{"key":"2021042701072720000_DMM047704C51","doi-asserted-by":"publisher","first-page":"399","DOI":"10.1111\/j.1365-2990.1997.tb01314.x","article-title":"The abnormal expression of utrophin in Duchenne and Becker muscular dystrophy is age related","volume":"23","author":"Taylor","year":"1997","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"2021042701072720000_DMM047704C52","doi-asserted-by":"publisher","first-page":"L401","DOI":"10.1152\/ajplung.00114.2010","article-title":"Validation of high-resolution echocardiography and magnetic resonance imaging vs. high-fidelity catheterization in experimental pulmonary hypertension","volume":"299","author":"Urboniene","year":"2010","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"2021042701072720000_DMM047704C53","doi-asserted-by":"publisher","first-page":"1068","DOI":"10.1038\/s41467-017-01146-7","article-title":"Reducing sarcolipin expression mitigates Duchenne muscular dystrophy and associated cardiomyopathy in mice","volume":"8","author":"Voit","year":"2017","journal-title":"Nat. Commun."},{"key":"2021042701072720000_DMM047704C54","doi-asserted-by":"publisher","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":"2021042701072720000_DMM047704C55","doi-asserted-by":"publisher","first-page":"1921","DOI":"10.1093\/hmg\/ddi197","article-title":"Cardiomyopathy in dystrophin-deficient hearts is prevented by expression of a neuronal nitric oxide synthase transgene in the myocardium","volume":"14","author":"Wehling-Henricks","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"2021042701072720000_DMM047704C56","doi-asserted-by":"publisher","first-page":"dmm035774","DOI":"10.1242\/dmm.035774","article-title":"Tracking progress: an update on animal models for Duchenne muscular dystrophy","volume":"11","author":"Wells","year":"2018","journal-title":"Dis. Model. Mech."},{"key":"2021042701072720000_DMM047704C57","doi-asserted-by":"publisher","first-page":"H846","DOI":"10.1152\/ajpheart.00688.2006","article-title":"Intracellular calcium handling in ventricular myocytes from mdx mice","volume":"292","author":"Williams","year":"2007","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"2021042701072720000_DMM047704C58","doi-asserted-by":"publisher","first-page":"375","DOI":"10.1002\/ejhf.464","article-title":"Significance of myocardial tenascin-C expression in left ventricular remodelling and long-term outcome in patients with dilated cardiomyopathy","volume":"18","author":"Yokokawa","year":"2016","journal-title":"Eur. J. Heart Fail"},{"key":"2021042701072720000_DMM047704C59","doi-asserted-by":"publisher","first-page":"1137","DOI":"10.1016\/0002-8703(91)90674-7","article-title":"Late potentials in progressive muscular dystrophy of the Duchenne type","volume":"121","author":"Yotsukura","year":"1991","journal-title":"Am. Heart J."}],"container-title":["Disease Models &amp; Mechanisms"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1242\/dmm.047704","content-type":"unspecified","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.047704\/1820765\/dmm047704.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.047704\/1820765\/dmm047704.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,27]],"date-time":"2021-04-27T02:48:41Z","timestamp":1619491721000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.biologists.com\/dmm\/article\/14\/2\/dmm047704\/237298\/Cardiovascular-phenotype-of-the-Dmdmdx-rat-a"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,1]]},"references-count":59,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,2,1]]}},"URL":"https:\/\/doi.org\/10.1242\/dmm.047704","relation":{},"ISSN":["1754-8403","1754-8411"],"issn-type":[{"value":"1754-8403","type":"print"},{"value":"1754-8411","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,1]]},"article-number":"dmm047704"}}