{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:49:16Z","timestamp":1787021356413,"version":"build-2736575974"},"reference-count":73,"publisher":"The Company of Biologists","issue":"8","license":[{"start":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T00:00:00Z","timestamp":1724716800000},"content-version":"vor","delay-in-days":26,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"crossref","award":["APP1143321"],"award-info":[{"award-number":["APP1143321"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"crossref","award":["APP2016822"],"award-info":[{"award-number":["APP2016822"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100021137","name":"Woodside Energy","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100021137","id-type":"DOI","asserted-by":"crossref"}]},{"name":"NSW Health"},{"DOI":"10.13039\/501100001773","name":"UNSW Sydney","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001773","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,8,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Hypertrophic cardiomyopathy (HCM) is an inherited heart muscle disease that is characterised by left ventricular wall thickening, cardiomyocyte disarray and fibrosis, and is associated with arrhythmias, heart failure and sudden death. However, it is unclear to what extent the electrophysiological disturbances that lead to sudden death occur secondary to structural changes in the myocardium or as a result of HCM cardiomyocyte electrophysiology. In this study, we used an induced pluripotent stem cell model of the R403Q variant in myosin heavy chain 7 (MYH7) to study the electrophysiology of HCM cardiomyocytes in electrically coupled syncytia, revealing significant conduction slowing and increased spatial dispersion of repolarisation \u2013 both well-established substrates for arrhythmia. Analysis of rhythmonome protein expression in MYH7 R403Q cardiomyocytes showed reduced expression of connexin-43 (also known as GJA1), sodium channels and inward rectifier potassium channels \u2013 a three-way hit that reduces electrotonic coupling and slows cardiac conduction. Our data represent a previously unreported, biophysical basis for arrhythmia in HCM that is intrinsic to cardiomyocyte electrophysiology. Later in the progression of the disease, these proarrhythmic phenotypes may be accentuated by myocyte disarray and fibrosis to contribute to sudden death.<\/jats:p>","DOI":"10.1242\/dmm.050407","type":"journal-article","created":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T03:40:52Z","timestamp":1724730052000},"source":"Crossref","is-referenced-by-count":7,"title":["Reduced connexin-43 expression, slow conduction and repolarisation dispersion in a model of hypertrophic cardiomyopathy"],"prefix":"10.1242","volume":"17","author":[{"given":"Seakcheng","family":"Lim","sequence":"first","affiliation":[{"name":"Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney 1 , Sydney 2050 , Australia"},{"name":"University of Sydney 2 Faculty of Medicine and Health , , Sydney 2050 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa M.","family":"Mangala","sequence":"additional","affiliation":[{"name":"Victor Chang Cardiac Research Institute 3 , Sydney, 2010 , Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 4 , Sydney 2052 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mira","family":"Holliday","sequence":"additional","affiliation":[{"name":"Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney 1 , Sydney 2050 , Australia"},{"name":"University of Sydney 2 Faculty of Medicine and Health , , Sydney 2050 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrietta","family":"Cserne Szappanos","sequence":"additional","affiliation":[{"name":"School of Human Sciences, The University of Western Australia 6 , Crawley 6009 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Barratt-Ross","sequence":"additional","affiliation":[{"name":"Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney 1 , Sydney 2050 , Australia"},{"name":"University of Sydney 2 Faculty of Medicine and Health , , Sydney 2050 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serena","family":"Li","sequence":"additional","affiliation":[{"name":"Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney 1 , Sydney 2050 , Australia"},{"name":"University of Sydney 2 Faculty of Medicine and Health , , Sydney 2050 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordan","family":"Thorpe","sequence":"additional","affiliation":[{"name":"Victor Chang Cardiac Research Institute 3 , Sydney, 2010 , Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 4 , Sydney 2052 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Whitney","family":"Liang","sequence":"additional","affiliation":[{"name":"Victor Chang Cardiac Research Institute 3 , Sydney, 2010 , Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 4 , Sydney 2052 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ginell N.","family":"Ranpura","sequence":"additional","affiliation":[{"name":"Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney 1 , Sydney 2050 , Australia"},{"name":"University of Sydney 2 Faculty of Medicine and Health , , Sydney 2050 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3859-3716","authenticated-orcid":false,"given":"Jamie I.","family":"Vandenberg","sequence":"additional","affiliation":[{"name":"Victor Chang Cardiac Research Institute 3 , Sydney, 2010 , Australia"},{"name":"School of Clinical Medicine, Faculty of Medicine and Health, UNSW Sydney 4 , Sydney 2052 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Semsarian","sequence":"additional","affiliation":[{"name":"Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney 1 , Sydney 2050 , Australia"},{"name":"University of Sydney 2 Faculty of Medicine and Health , , Sydney 2050 , Australia"},{"name":"Royal Prince Alfred Hospital 5 Department of Cardiology , , Sydney 2050 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6403-1282","authenticated-orcid":false,"given":"Adam P.","family":"Hill","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Livia C.","family":"Hool","sequence":"additional","affiliation":[{"name":"Victor Chang Cardiac Research Institute 3 , Sydney, 2010 , Australia"},{"name":"School of Human Sciences, The University of Western Australia 6 , Crawley 6009 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2024,8,27]]},"reference":[{"key":"2024082707403505700_DMM050407C1","doi-asserted-by":"publisher","first-page":"H2024","DOI":"10.1152\/ajpheart.00355.2007","article-title":"Role of spatial dispersion of repolarization in inherited and acquired sudden cardiac death syndromes","volume":"293","author":"Antzelevitch","year":"2007","journal-title":"Am. J. Physiol. Hear. Circ. Physiol."},{"key":"2024082707403505700_DMM050407C2","doi-asserted-by":"publisher","first-page":"1191","DOI":"10.1161\/CIRCULATIONAHA.106.672162","article-title":"Remote magnetic navigation to guide endocardial and epicardial catheter mapping of scar-related ventricular tachycardia","volume":"115","author":"Aryana","year":"2007","journal-title":"Circulation"},{"key":"2024082707403505700_DMM050407C3","doi-asserted-by":"publisher","first-page":"491","DOI":"10.1093\/biomet\/93.3.491","article-title":"Adaptive linear step-up procedures that control the false discovery rate","volume":"93","author":"Benjamini","year":"2006","journal-title":"Biometrika"},{"key":"2024082707403505700_DMM050407C4","doi-asserted-by":"publisher","first-page":"3053","DOI":"10.1161\/01.CIR.0000019407.35848.AF","article-title":"Polymorphic reentrant ventricular tachycardia in the isolated rabbit heart studied by high-density mapping","volume":"105","author":"Boersma","year":"2002","journal-title":"Circulation"},{"key":"2024082707403505700_DMM050407C5","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1161\/CIRCRESAHA.108.171835","article-title":"Perinatal loss of Nkx2-5 results in rapid conduction and contraction defects","volume":"103","author":"Briggs","year":"2008","journal-title":"Circ. Res."},{"key":"2024082707403505700_DMM050407C6","doi-asserted-by":"publisher","first-page":"926","DOI":"10.1016\/S0735-1097(00)01195-5","article-title":"Alterations in potassium channel gene expression in atria of patients with persistent and paroxysmal atrial fibrillation: differential regulation of protein and mRNA levels for K+ channels","volume":"37","author":"Brundel","year":"2001","journal-title":"J. Am. Coll. Cardiol."},{"key":"2024082707403505700_DMM050407C7","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1016\/j.pcad.2023.04.009","article-title":"Evolution of genetic testing and gene therapy in hypertrophic cardiomyopathy","volume":"80","author":"Chiswell","year":"2023","journal-title":"Prog. Cardiovasc. Dis"},{"key":"2024082707403505700_DMM050407C8","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1186\/1475-925X-4-11","article-title":"Dispersion of cardiac action potential duration and the initiation of re-entry: A computational study","volume":"4","author":"Clayton","year":"2005","journal-title":"Biomed. Eng. Online"},{"key":"2024082707403505700_DMM050407C9","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1016\/j.stemcr.2018.11.015","article-title":"A contraction stress model of hypertrophic cardiomyopathy due to sarcomere mutations","volume":"12","author":"Cohn","year":"2019","journal-title":"Stem Cell Rep."},{"key":"2024082707403505700_DMM050407C10","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1016\/j.jelectrocard.2016.10.001","article-title":"ECG-derived spatial QRS-T angle is strongly associated with hypertrophic cardiomyopathy","volume":"50","author":"Cortez","year":"2017","journal-title":"J. Electrocardiol."},{"key":"2024082707403505700_DMM050407C61","doi-asserted-by":"publisher","first-page":"11296","DOI":"10.1038\/s41598-023-38296-2","article-title":"Cytoskeletal disarray increases arrhythmogenic vulnerability during sympathetic stimulation in a model of hypertrophic cardiomyopathy","volume":"13","author":"Cserne Szappanos","year":"2023","journal-title":"Sci. Rep."},{"key":"2024082707403505700_DMM050407C11","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1152\/physiolgenomics.00021.2020","article-title":"Silencing of MYH7 ameliorates disease phenotypes in human iPSC-cardiomyocytes","volume":"52","author":"Dainis","year":"2020","journal-title":"Physiol. Genomics"},{"key":"2024082707403505700_DMM050407C12","doi-asserted-by":"publisher","first-page":"1000","DOI":"10.1161\/CIRCRESAHA.117.310355","article-title":"Calcium signaling and transcriptional regulation in cardiomyocytes","volume":"121","author":"Dewenter","year":"2017","journal-title":"Circ. Res."},{"key":"2024082707403505700_DMM050407C13","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1177\/107424840601100103","article-title":"Subcellular remodeling as a viable target for the treatment of congestive heart failure","volume":"11","author":"Dhalla","year":"2006","journal-title":"J. Cardiovasc. Pharmacol. Ther."},{"key":"2024082707403505700_DMM050407C14","doi-asserted-by":"publisher","first-page":"858","DOI":"10.1093\/europace\/euw087","article-title":"Torsade de pointes arrhythmias arise at the site of maximal heterogeneity of repolarization in the chronic complete atrioventricular block dog","volume":"19","author":"Dunnink","year":"2015","journal-title":"Europace"},{"key":"2024082707403505700_DMM050407C15","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1111\/chd.12317","article-title":"Familial atrial septal defect and sudden cardiac death: identification of a novel NKX2-5 mutation and a review of the literature","volume":"11","author":"Elles\u00f8e","year":"2016","journal-title":"Congenit. Hear. Dis."},{"key":"2024082707403505700_DMM050407C16","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1016\/B978-0-12-381332-9.00026-8","article-title":"Chapter 9.1\u2005-\u2005NK-2 Class Homeodomain Proteins: Conserved Regulators of Cardiogenesis","author":"Elliott","year":"2010","journal-title":"Heart development and regeneration"},{"key":"2024082707403505700_DMM050407C17","doi-asserted-by":"publisher","first-page":"2733","DOI":"10.1093\/eurheartj\/ehu284","article-title":"2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)","volume":"35","author":"Elliott","year":"2014","journal-title":"Eur. Hear. J."},{"key":"2024082707403505700_DMM050407C18","doi-asserted-by":"publisher","first-page":"1208","DOI":"10.1093\/bioinformatics\/btx752","article-title":"Reactome diagram viewer: data structures and strategies to boost performance","volume":"34","author":"Fabregat","year":"2018","journal-title":"Bioinformatics"},{"key":"2024082707403505700_DMM050407C19","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1016\/j.yjmcc.2021.04.009","article-title":"Translational investigation of electrophysiology in hypertrophic cardiomyopathy","volume":"157","author":"Flenner","year":"2021","journal-title":"J. Mol. Cell. Cardiol."},{"key":"2024082707403505700_DMM050407C20","doi-asserted-by":"publisher","first-page":"999","DOI":"10.1016\/0092-8674(90)90274-I","article-title":"A molecular basis for familial hypertrophic cardiomyopathy: A \u03b2 cardiac myosin heavy chain gene missense mutation","volume":"62","author":"Geisterfer-Lowrance","year":"1990","journal-title":"Cell"},{"key":"2024082707403505700_DMM050407C21","doi-asserted-by":"publisher","first-page":"731","DOI":"10.1126\/science.272.5262.731","article-title":"A mouse model of familial hypertrophic cardiomyopathy","volume":"272","author":"Geisterfer-Lowrance","year":"1996","journal-title":"Science"},{"key":"2024082707403505700_DMM050407C22","doi-asserted-by":"publisher","first-page":"432","DOI":"10.1161\/01.RES.57.3.432","article-title":"Reentrant ventricular arrhythmias in the late myocardial infarction period in the dog. 13. Correlation of activation and refractory maps","volume":"57","author":"Gough","year":"2018","journal-title":"Circ. Res."},{"key":"2024082707403505700_DMM050407C23","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1161\/01.RES.14.1.44","article-title":"Nonuniform recovery of excitability in ventricular muscle","volume":"14","author":"Han","year":"1964","journal-title":"Circ. Res."},{"key":"2024082707403505700_DMM050407C24","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1093\/cvr\/cvu205","article-title":"Study familial hypertrophic cardiomyopathy using patient-specific induced pluripotent stem cells","volume":"104","author":"Han","year":"2014","journal-title":"Cardiovasc. Res."},{"key":"2024082707403505700_DMM050407C25","doi-asserted-by":"publisher","first-page":"1738","DOI":"10.1161\/CIRCULATIONAHA.115.020086","article-title":"Genotype-dependent and -independent calcium signaling dysregulation in human hypertrophic cardiomyopathy","volume":"134","author":"Helms","year":"2016","journal-title":"Circulation"},{"key":"2024082707403505700_DMM050407C26","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1016\/j.scr.2018.11.011","article-title":"Development of induced pluripotent stem cells from a patient with hypertrophic cardiomyopathy who carries the pathogenic myosin heavy chain 7 mutation p.Arg403Gln","volume":"33","author":"Holliday","year":"2018","journal-title":"Stem Cell Res."},{"key":"2024082707403505700_DMM050407C27","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1016\/j.yjmcc.2017.01.006","article-title":"Early remodeling of repolarizing K+ currents in the \u03b1MHC403\/+ mouse model of familial hypertrophic cardiomyopathy","volume":"103","author":"Hueneke","year":"2017","journal-title":"J. Mol. Cell. Cardiol."},{"key":"2024082707403505700_DMM050407C28","doi-asserted-by":"publisher","first-page":"R102","DOI":"10.1152\/ajpregu.00233.2012","article-title":"Differential expression of the pro-natriuretic peptide convertases corin and furin in experimental heart failure and atrial fibrosis","volume":"304","author":"Ichiki","year":"2013","journal-title":"Am. J. Physiol. Regul. Integr. Comp. Physiol."},{"key":"2024082707403505700_DMM050407C29","doi-asserted-by":"publisher","first-page":"1049","DOI":"10.1152\/physrev.1989.69.4.1049","article-title":"Electrophysiological mechanisms of ventricular arrhythmias resulting from myocardial ischemia and infarction","volume":"69","author":"Janse","year":"1989","journal-title":"Physiol. Rev."},{"key":"2024082707403505700_DMM050407C30","first-page":"167","article-title":"Modulation of repolarization by electrotonic interactions","volume":"27","author":"Joyner","year":"1986","journal-title":"Jpn. Heart J."},{"key":"2024082707403505700_DMM050407C31","doi-asserted-by":"publisher","first-page":"1333","DOI":"10.1091\/mbc.E21-03-0130","article-title":"Violin SuperPlots: visualizing replicate heterogeneity in large data sets","volume":"32","author":"Kenny","year":"2021","journal-title":"Mol. Biol. Cell"},{"key":"2024082707403505700_DMM050407C32","doi-asserted-by":"publisher","first-page":"673497","DOI":"10.3389\/fphys.2021.673497","article-title":"Distribution of cardiac and renal corin and proprotein convertase subtilisin\/kexin-6 in the experimental model of cardio-renal syndrome of various severities","volume":"12","author":"Khoury","year":"2021","journal-title":"Front. Physiol."},{"key":"2024082707403505700_DMM050407C33","doi-asserted-by":"publisher","first-page":"154","DOI":"10.3389\/fphys.2013.00154","article-title":"Determinants of myocardial conduction velocity: implications for arrhythmogenesis","volume":"4","author":"King","year":"2013","journal-title":"Front. Physiol."},{"key":"2024082707403505700_DMM050407C34","doi-asserted-by":"publisher","first-page":"426","DOI":"10.1016\/j.cardiores.2003.12.010","article-title":"Connexin 43 expression and distribution in compensated and decompensated cardiac hypertrophy in patients with aortic stenosis","volume":"62","author":"Kostin","year":"2004","journal-title":"Cardiovasc. Res."},{"key":"2024082707403505700_DMM050407C35","doi-asserted-by":"publisher","first-page":"3112","DOI":"10.1093\/cvr\/cvab375","article-title":"Electrophysiological heterogeneity in large populations of rabbit ventricular cardiomyocytes","volume":"118","author":"Lachaud","year":"2022","journal-title":"Cardiovasc. Res."},{"key":"2024082707403505700_DMM050407C36","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/j.stem.2012.10.010","article-title":"Abnormal calcium handling properties underlie familial hypertrophic cardiomyopathy pathology in patient-specific induced pluripotent stem cells","volume":"12","author":"Lan","year":"2013","journal-title":"Cell Stem Cell"},{"key":"2024082707403505700_DMM050407C37","doi-asserted-by":"publisher","first-page":"H1516","DOI":"10.1152\/ajpheart.00947.2003","article-title":"Rat corin gene: molecular cloning and reduced expression in experimental heart failure","volume":"287","author":"Langenickel","year":"2004","journal-title":"Am. J. Physiol. Hear. Circ. Physiol."},{"key":"2024082707403505700_DMM050407C38","doi-asserted-by":"publisher","first-page":"1426","DOI":"10.1161\/01.RES.65.5.1426","article-title":"Cellular uncoupling can unmask dispersion of action potential duration in ventricular myocardium. A computer modeling study","volume":"65","author":"Lesh","year":"2018","journal-title":"Circ. Res."},{"key":"2024082707403505700_DMM050407C39","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1016\/j.cell.2016.03.014","article-title":"On the dependency of cellular protein levels on mRNA abundance","volume":"165","author":"Liu","year":"2016","journal-title":"Cell"},{"key":"2024082707403505700_DMM050407C40","doi-asserted-by":"publisher","first-page":"iii102","DOI":"10.1093\/europace\/euy226","article-title":"Electrocardiogram phenotypes in hypertrophic cardiomyopathy caused by distinct mechanisms: apico-basal repolarization gradients vs. Purkinje-myocardial coupling abnormalities","volume":"20","author":"Lyon","year":"2018","journal-title":"Europace"},{"key":"2024082707403505700_DMM050407C41","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1016\/j.jjcc.2017.01.006","article-title":"QT spatial dispersion and sudden cardiac death in hypertrophic cardiomyopathy: Time for reappraisal","volume":"70","author":"Magr\u00ec","year":"2017","journal-title":"J. Cardiol"},{"key":"2024082707403505700_DMM050407C42","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1016\/S0008-6363(99)00360-0","article-title":"Heterogeneous changes in action potential and intracellular Ca2+ in left ventricular myocyte sub-types from rabbits with heart failure","volume":"45","author":"McIntosh","year":"2000","journal-title":"Cardiovasc. Res."},{"key":"2024082707403505700_DMM050407C43","doi-asserted-by":"publisher","first-page":"E8372","DOI":"10.1073\/pnas.1703109114","article-title":"Functional screening in human cardiac organoids reveals a metabolic mechanism for cardiomyocyte cell cycle arrest","volume":"114","author":"Mills","year":"2017","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2024082707403505700_DMM050407C44","doi-asserted-by":"publisher","first-page":"349","DOI":"10.1113\/jphysiol.1913.sp001596","article-title":"On dynamic equilibrium in the heart","volume":"46","author":"Mines","year":"1913","journal-title":"J. Physiol."},{"key":"2024082707403505700_DMM050407C45","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1016\/j.molmed.2019.06.005","article-title":"Modeling hypertrophic cardiomyopathy: mechanistic insights and pharmacological intervention","volume":"25","author":"Mosqueira","year":"2019","journal-title":"Trends Mol. Med."},{"key":"2024082707403505700_DMM050407C46","doi-asserted-by":"publisher","first-page":"821","DOI":"10.1016\/j.bbrc.2009.10.056","article-title":"The search for Nkx2\u20135-regulated genes using purified embryonic stem cell-derived cardiomyocytes with Nkx2\u20135 gene targeting","volume":"390","author":"Nakashima","year":"2009","journal-title":"Biochem. Bioph. Res. Co."},{"key":"2024082707403505700_DMM050407C47","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1161\/CIRCEP.111.965434","article-title":"Crescendo in depolarization and repolarization heterogeneity heralds development of ventricular tachycardia in hospitalized patients with decompensated heart failure","volume":"5","author":"Nearing","year":"2012","journal-title":"Circ. Arrhythm. Electrophysiol."},{"key":"2024082707403505700_DMM050407C48","doi-asserted-by":"publisher","first-page":"2102","DOI":"10.1016\/S0140-6736(21)01205-8","article-title":"Hypertrophic cardiomyopathy: a practical approach to guideline directed management","volume":"398","author":"Ommen","year":"2021","journal-title":"Lancet"},{"key":"2024082707403505700_DMM050407C49","doi-asserted-by":"publisher","first-page":"3022","DOI":"10.1016\/j.jacc.2020.08.044","article-title":"2020 AHA\/ACC Guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: executive summary a report of the american college of cardiology\/american heart association joint committee on clinical practice guidelines","volume":"76","author":"Ommen","year":"2020","journal-title":"J. Am. Coll. Cardiol"},{"key":"2024082707403505700_DMM050407C50","doi-asserted-by":"publisher","first-page":"38390","DOI":"10.1074\/jbc.M205686200","article-title":"Genomic structures of the human and murine corin genes and functional GATA elements in their promoters*","volume":"277","author":"Pan","year":"2002","journal-title":"J. Biol. Chem."},{"key":"2024082707403505700_DMM050407C51","doi-asserted-by":"publisher","first-page":"549","DOI":"10.1161\/CIRCRESAHA.116.310396","article-title":"Transient notch activation induces long-term gene expression changes leading to sick sinus syndrome in Mice","volume":"121","author":"Qiao","year":"2017","journal-title":"Circ. Res."},{"key":"2024082707403505700_DMM050407C52","doi-asserted-by":"publisher","first-page":"1831","DOI":"10.1126\/science.7892609","article-title":"Cardiac malformation in neonatal mice lacking connexin43","volume":"267","author":"Reaume","year":"1995","journal-title":"Science"},{"key":"2024082707403505700_DMM050407C53","doi-asserted-by":"publisher","first-page":"10487","DOI":"10.1074\/jbc.RA118.002081","article-title":"Hypertrophic cardiomyopathy mutations increase myofilament Ca2+ buffering, alter intracellular Ca2+ handling, and stimulate Ca2+-dependent signaling","volume":"293","author":"Robinson","year":"2018","journal-title":"J Biological Chem"},{"key":"2024082707403505700_DMM050407C54","doi-asserted-by":"publisher","first-page":"S19","DOI":"10.1136\/hrt.72.6_Suppl.S19","article-title":"Electrophysiological investigation of patients with hypertrophic cardiomyopathy: Evidence that slowed intraventricular conduction is associated with an increased risk of sudden death","volume":"72","author":"Saumarez","year":"1994","journal-title":"Heart"},{"key":"2024082707403505700_DMM050407C55","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1161\/01.CIR.86.2.467","article-title":"Ventricular fibrillation in hypertrophic cardiomyopathy is associated with increased fractionation of paced right ventricular electrograms","volume":"86","author":"Saumarez","year":"2018","journal-title":"Circulation"},{"key":"2024082707403505700_DMM050407C56","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1038\/nmeth.2089","article-title":"NIH Image to ImageJ: 25 years of image analysis","volume":"9","author":"Schneider","year":"2012","journal-title":"Nat. Methods"},{"key":"2024082707403505700_DMM050407C57","doi-asserted-by":"publisher","first-page":"2096","DOI":"10.1161\/CIRCULATIONAHA.104.515643","article-title":"Electrophysiological characteristics of septal hypertrophy in patients with hypertrophic obstructive cardiomyopathy and moderate to severe symptoms","volume":"112","author":"Schumacher","year":"2005","journal-title":"Circulation"},{"key":"2024082707403505700_DMM050407C58","doi-asserted-by":"publisher","first-page":"2055","DOI":"10.1006\/jmcc.2001.1466","article-title":"A polymorphic modifier gene alters the hypertrophic response in a murine model of familial hypertrophic cardiomyopathy","volume":"33","author":"Semsarian","year":"2001","journal-title":"J. Mol. Cell. Cardiol"},{"key":"2024082707403505700_DMM050407C59","doi-asserted-by":"publisher","first-page":"1013","DOI":"10.1172\/JCI200214677","article-title":"The L-type calcium channel inhibitor diltiazem prevents cardiomyopathy in a mouse model","volume":"109","author":"Semsarian","year":"2002","journal-title":"J. Clin. Investig."},{"key":"2024082707403505700_DMM050407C60","doi-asserted-by":"publisher","first-page":"1249","DOI":"10.1016\/j.jacc.2015.01.019","article-title":"New Perspectives on the Prevalence of Hypertrophic Cardiomyopathy","volume":"65","author":"Semsarian","year":"2015","journal-title":"J. Am. Coll. Cardiol."},{"key":"2024082707403505700_DMM050407C62","doi-asserted-by":"publisher","first-page":"1324","DOI":"10.1111\/jce.13288","article-title":"Interlead heterogeneity of R- and T-wave morphology in standard 12-lead ECGs predicts sustained ventricular tachycardia\/fibrillation and arrhythmic death in patients with cardiomyopathy","volume":"28","author":"Tan","year":"2017","journal-title":"J. Cardiovasc. Electrophysiol"},{"key":"2024082707403505700_DMM050407C63","doi-asserted-by":"publisher","first-page":"828","DOI":"10.1161\/CIRCULATIONAHA.119.042339","article-title":"Myosin sequestration regulates sarcomere function, cardiomyocyte energetics, and metabolism, informing the pathogenesis of hypertrophic cardiomyopathy","volume":"141","author":"Toepfer","year":"2019","journal-title":"Circulation"},{"key":"2024082707403505700_DMM050407C64","doi-asserted-by":"publisher","first-page":"H1625","DOI":"10.1152\/ajpheart.00298.2004","article-title":"Upregulation of corin gene expression in hypertrophic cardiomyocytes and failing myocardium","volume":"287","author":"Tran","year":"2004","journal-title":"Am. J. Physiol Heart C"},{"key":"2024082707403505700_DMM050407C65","doi-asserted-by":"publisher","first-page":"1048","DOI":"10.1161\/01.CIR.0000117402.70689.75","article-title":"Slow conduction and enhanced anisotropy increase the propensity for ventricular tachyarrhythmias in adult mice with induced deletion of connexin43","volume":"109","author":"van Rijen","year":"2004","journal-title":"Circulation"},{"key":"2024082707403505700_DMM050407C66","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1016\/S0002-9149(01)01640-X","article-title":"Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy","volume":"88","author":"Varnava","year":"2001","journal-title":"Am. J. Cardiol."},{"key":"2024082707403505700_DMM050407C67","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1016\/j.abb.2019.03.006","article-title":"Impaired calcium handling and mitochondrial metabolic dysfunction as early markers of hypertrophic cardiomyopathy","volume":"665","author":"Viola","year":"2019","journal-title":"Arch. Biochem. Biophys."},{"key":"2024082707403505700_DMM050407C68","doi-asserted-by":"publisher","first-page":"18123","DOI":"10.1073\/pnas.0509145102","article-title":"Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia","volume":"102","author":"Wolf","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2024082707403505700_DMM050407C69","doi-asserted-by":"publisher","first-page":"e159","DOI":"10.1016\/j.jacc.2020.08.045","article-title":"2020 AHA\/ACC Guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy a report of the American college of cardiology\/American heart association joint committee on clinical practice guidelines","volume":"76","author":"Writing Committee Members","year":"2020","journal-title":"J. Am. Coll. Cardiol"},{"key":"2024082707403505700_DMM050407C70","doi-asserted-by":"publisher","first-page":"3685","DOI":"10.1093\/eurheartj\/ehz326","article-title":"Modelling diastolic dysfunction in induced pluripotent stem cell-derived cardiomyocytes from hypertrophic cardiomyopathy patients","volume":"40","author":"Wu","year":"2019","journal-title":"Eur. Hear. J."},{"key":"2024082707403505700_DMM050407C71","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1093\/cvr\/cvw073","article-title":"Patient-derived models link re-entrant driver localization in atrial fibrillation to fibrosis spatial pattern","volume":"110","author":"Zahid","year":"2016","journal-title":"Cardiovasc. Res."},{"key":"2024082707403505700_DMM050407C72","doi-asserted-by":"publisher","first-page":"H477","DOI":"10.1152\/ajpheart.00806.2009","article-title":"Reduced expression of Cx43 attenuates ventricular remodeling after myocardial infarction via impaired TGF-beta signaling","volume":"298","author":"Zhang","year":"2009","journal-title":"Am. J. Physiol. Hear. Circ. Physiol."},{"key":"2024082707403505700_DMM050407C73","doi-asserted-by":"publisher","first-page":"802","DOI":"10.1016\/j.stem.2019.02.015","article-title":"A human iPSC double-reporter system enables purification of cardiac lineage subpopulations with distinct function and drug response profiles","volume":"24","author":"Zhang","year":"2019","journal-title":"Cell Stem Cell"}],"container-title":["Disease Models &amp; Mechanisms"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.050407\/3561717\/dmm050407.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.050407\/3561717\/dmm050407.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T03:41:00Z","timestamp":1724730060000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.biologists.com\/dmm\/article\/17\/8\/dmm050407\/361760\/Reduced-connexin-43-expression-slow-conduction-and"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,1]]},"references-count":73,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2024,8,1]]}},"URL":"https:\/\/doi.org\/10.1242\/dmm.050407","relation":{},"ISSN":["1754-8403","1754-8411"],"issn-type":[{"value":"1754-8403","type":"print"},{"value":"1754-8411","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,8,1]]},"article-number":"dmm050407"}}