{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T03:13:10Z","timestamp":1790392390624,"version":"4.1.0"},"reference-count":177,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2024,11,8]],"date-time":"2024-11-08T00:00:00Z","timestamp":1731024000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Cardiac channelopathies are inherited diseases that increase the risk of sudden cardiac death. While different genes have been associated with inherited channelopathies, there are still subtypes, e.g., catecholaminergic polymorphic ventricular tachycardia and Brugada syndrome, where the genetic cause remains unknown. Various models, including animal models, heterologous expression systems, and the human-induced pluripotent stem-cell-derived cardiomyocytes (hiPSCs-CMs) model, have been used to study the pathophysiological mechanisms of channelopathies. Recently, researchers have focused on using hiPSCs-CMs to understand the genotype\u2013phenotype correlation and screen drugs. By combining innovative techniques such as Clustered Regularly Interspaced Short Palindromic Repeats\/Clustered Regularly Interspaced Short Palindromic Repeats associated protein 9 (CRISPR\/Cas9)-mediated genome editing, and three-dimensional (3D) engineered heart tissues, we can gain new insights into the pathophysiological mechanisms of channelopathies. This approach holds promise for improving personalized drug treatment. This review highlights the role of hiPSCs-CMs in understanding the pathomechanism of Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia and how these models can be utilized for drug screening.<\/jats:p>","DOI":"10.3390\/ijms252212034","type":"journal-article","created":{"date-parts":[[2024,11,11]],"date-time":"2024-11-11T03:52:07Z","timestamp":1731297127000},"page":"12034","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["The Role of Human-Induced Pluripotent Stem Cells in Studying Cardiac Channelopathies"],"prefix":"10.3390","volume":"25","author":[{"given":"Merima","family":"Begovic","sequence":"first","affiliation":[{"name":"Institute of Physiology, Department of Cellular and Translational Physiology, Ruhr-University Bochum, 44801 Bochum, Germany"},{"name":"Institut f\u00fcr Forschung und Lehre (IFL), Molecular and Experimental Cardiology, St. Josef Hospital, Ruhr-University Bochum, 44791 Bochum, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Schneider","sequence":"additional","affiliation":[{"name":"Institute of Physiology, Department of Cellular and Translational Physiology, Ruhr-University Bochum, 44801 Bochum, Germany"},{"name":"Institut f\u00fcr Forschung und Lehre (IFL), Molecular and Experimental Cardiology, St. Josef Hospital, Ruhr-University Bochum, 44791 Bochum, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5895-0127","authenticated-orcid":false,"given":"Xiaobo","family":"Zhou","sequence":"additional","affiliation":[{"name":"Cardiology, Angiology, Haemostaseology, and Medical Intensive Care, Medical Centre Mannheim, Medical Faculty Mannheim, Heidelberg University, 68167 Mannheim, Germany"},{"name":"Key Laboratory of Medical Electrophysiology of Ministry of Education and Medical Electrophysiological Key Laboratory of Sichuan Province, Institute of Cardiovascular Research, Southwest Medical University, Luzhou 646000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3053-0008","authenticated-orcid":false,"given":"Nazha","family":"Hamdani","sequence":"additional","affiliation":[{"name":"Institute of Physiology, Department of Cellular and Translational Physiology, Ruhr-University Bochum, 44801 Bochum, Germany"},{"name":"Institut f\u00fcr Forschung und Lehre (IFL), Molecular and Experimental Cardiology, St. Josef Hospital, Ruhr-University Bochum, 44791 Bochum, Germany"},{"name":"Department of Physiology, Cardiovascular Research Institute, University Maastricht, 6229HX Maastricht, The Netherlands"},{"name":"HCEMM-SU Cardiovascular Comorbidities Research Group, Center for Pharmacology and Drug Research & Development, Department of Pharmacology and Pharmacotherapy, Int\u00e9zet c\u00edme Semmelweis University, 1089 Budapest, Hungary"},{"name":"Department of Cardiology and Rhythmology, St. Josef Hospital, Ruhr University, 44791 Bochum, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim","family":"Akin","sequence":"additional","affiliation":[{"name":"Cardiology, Angiology, Haemostaseology, and Medical Intensive Care, Medical Centre Mannheim, Medical Faculty Mannheim, Heidelberg University, 68167 Mannheim, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim","family":"El-Battrawy","sequence":"additional","affiliation":[{"name":"Institute of Physiology, Department of Cellular and Translational Physiology, Ruhr-University Bochum, 44801 Bochum, Germany"},{"name":"Institut f\u00fcr Forschung und Lehre (IFL), Molecular and Experimental Cardiology, St. Josef Hospital, Ruhr-University Bochum, 44791 Bochum, Germany"},{"name":"Department of Cardiology and Rhythmology, St. Josef Hospital, Ruhr University, 44791 Bochum, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,11,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e007394","DOI":"10.1161\/JAHA.117.007394","article-title":"Modeling Short QT Syndrome Using Human-Induced Pluripotent Stem Cell\u2013Derived Cardiomyocytes","volume":"7","author":"Lan","year":"2018","journal-title":"J. Am. Heart Assoc."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1002\/cpt.1449","article-title":"Drug Testing in Human-Induced Pluripotent Stem Cell\u2013Derived Cardiomyocytes from a Patient With Short QT Syndrome Type 1","volume":"106","author":"Zhao","year":"2019","journal-title":"Clin. Pharmacol. Ther."},{"key":"ref_3","first-page":"481","article-title":"Preclinical Short QT Syndrome Models: Studying the Phenotype and Drug-Screening","volume":"24","author":"Fan","year":"2022","journal-title":"EP Eur."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1161\/CIRCULATIONAHA.108.804617","article-title":"Sudden Deaths in Young Competitive Athletes: Analysis of 1866 Deaths in the United States, 1980\u20132006","volume":"119","author":"Maron","year":"2009","journal-title":"Circulation"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e001893","DOI":"10.1161\/CIRCGEN.117.001893","article-title":"Ion Channel Dysfunctions in Dilated Cardiomyopathy in Limb-Girdle Muscular Dystrophy","volume":"11","author":"Zhao","year":"2018","journal-title":"Circ. Genom. Precis. Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"f46","DOI":"10.1093\/europace\/euy042","article-title":"Electrical Dysfunctions in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes from a Patient with an Arrhythmogenic Right Ventricular Cardiomyopathy","volume":"20","author":"Zhao","year":"2018","journal-title":"Europace"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1161\/CIRCULATIONAHA.119.044794","article-title":"Intronic CRISPR Repair in a Preclinical Model of Noonan Syndrome-Associated Cardiomyopathy","volume":"142","author":"Hanses","year":"2020","journal-title":"Circulation"},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Sedaghat-Hamedani, F., Rebs, S., El-Battrawy, I., Chasan, S., Krause, T., Haas, J., Zhong, R., Liao, Z., Xu, Q., and Zhou, X. (2021). Identification of SCN5a p.C335R Variant in a Large Family with Dilated Cardiomyopathy and Conduction Disease. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms222312990"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"6663","DOI":"10.1038\/s41467-022-34366-7","article-title":"Lamin A\/C-Dependent Chromatin Architecture Safeguards Na\u00efve Pluripotency to Prevent Aberrant Cardiovascular Cell Fate and Function","volume":"13","author":"Wang","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Brodehl, A., Ebbinghaus, H., Deutsch, M.-A., Gummert, J., G\u00e4rtner, A., Ratnavadivel, S., and Milting, H. (2019). Human Induced Pluripotent Stem-Cell-Derived Cardiomyocytes as Models for Genetic Cardiomyopathies. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20184381"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1391","DOI":"10.1016\/0735-1097(92)90253-J","article-title":"Right Bundle Branch Block, Persistent ST Segment Elevation and Sudden Cardiac Death: A Distinct Clinical and Electrocardiographic Syndrome: A Multicenter Report","volume":"20","author":"Brugada","year":"1992","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_12","first-page":"267","article-title":"Worldwide Prevalence of Brugada Syndrome: A Systematic Review and Meta-Analysis","volume":"34","author":"Vutthikraivit","year":"2018","journal-title":"Acta Cardiol. Sin."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/j.joa.2016.07.002","article-title":"J-Wave Syndromes Expert Consensus Conference Report: Emerging Concepts and Gaps in Knowledge","volume":"32","author":"Antzelevitch","year":"2016","journal-title":"J. Arrhythmia"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1016\/j.hrthm.2019.11.027","article-title":"Age at Diagnosis of Brugada Syndrome: Influence on Clinical Characteristics and Risk of Arrhythmia","volume":"17 Pt A","author":"Minier","year":"2020","journal-title":"Heart Rhythm"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1161\/CIRCULATIONAHA.109.887026","article-title":"Long-Term Prognosis of Patients Diagnosed with Brugada Syndrome","volume":"121","author":"Probst","year":"2010","journal-title":"Circulation"},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Pannone, L., Osei, R., Gauthey, A., Sorgente, A., Della Rocca, D.G., Overeinder, I., Bala, G., Almorad, A., Stroker, E., and Sieira, J. (2023). Age and Genetics Are Associated with Ventricular Fibrillation but Not with Monomorphic Ventricular Tachycardia in Brugada Syndrome. Europace, 25.","DOI":"10.1093\/europace\/euad122.268"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1111\/jce.14848","article-title":"Risk Stratification in Asymptomatic Patients with Brugada Syndrome: Utility of Multiple Risk Factor Combination Rather than Programmed Electrical Stimulation","volume":"32","author":"Shinohara","year":"2021","journal-title":"J. Cardiovasc. Electrophysiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.hrthm.2009.09.069","article-title":"An International Compendium of Mutations in the SCN5A-Encoded Cardiac Sodium Channel in Patients Referred for Brugada Syndrome Genetic Testing","volume":"7","author":"Kapplinger","year":"2010","journal-title":"Heart Rhythm"},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Nielsen, M.W., Holst, A.G., Olesen, S.-P., and Olesen, M.S. (2013). The Genetic Component of Brugada Syndrome. Front. Physiol., 4.","DOI":"10.3389\/fphys.2013.00179"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1016\/S0735-1097(02)01962-9","article-title":"Genotype-Phenotype Relationship in Brugada Syndrome: Electrocardiographic Features Differentiate SCN5A-Related Patients from Non\u2013SCN5A-Related Patients","volume":"40","author":"Smits","year":"2002","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1161\/CIRCGENETICS.109.853374","article-title":"SCN5A Mutations and the Role of Genetic Background in the Pathophysiology of Brugada Syndrome","volume":"2","author":"Probst","year":"2009","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1092","DOI":"10.1161\/CIRCULATIONAHA.113.003077","article-title":"Missense Mutations in Plakophilin-2 Cause Sodium Current Deficit and Associate with a Brugada Syndrome Phenotype","volume":"129","author":"Cerrone","year":"2014","journal-title":"Circulation"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1038\/32675","article-title":"Genetic Basis and Molecular Mechanism for Idiopathic Ventricular Fibrillation","volume":"392","author":"Chen","year":"1998","journal-title":"Nature"},{"key":"ref_24","first-page":"S395","article-title":"Genetic Variants in SCN10A Associated with Brugada Syndrome, Right Bundle Branch Block and Atrioventricular Block [Abstract]","volume":"9","author":"Hu","year":"2012","journal-title":"Heart Rhythm"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1161\/CIRCGENETICS.110.959130","article-title":"Mog1","volume":"4","author":"Kattygnarath","year":"2011","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_26","first-page":"2260","article-title":"Sodium Channel \u03921 Subunit Mutations Associated with Brugada Syndrome and Cardiac Conduction Disease in Humans","volume":"118","author":"Watanabe","year":"2008","journal-title":"J. Clin. Investig."},{"key":"ref_27","unstructured":"(2024, October 03). A Missense Mutation in the Sodium Channel \u03b22 Subunit Reveals SCN2B as a New Candidate Gene for Brugada Syndrome\u2014Riur\u00f3\u20142013\u2014Human Mutation\u2014Wiley Online Library. Available online: https:\/\/onlinelibrary.wiley.com\/doi\/abs\/10.1002\/humu.22328."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1161\/CIRCGENETICS.108.829192","article-title":"A Mutation in the \u03923 Subunit of the Cardiac Sodium Channel Associated with Brugada ECG Phenotype","volume":"2","author":"Hu","year":"2009","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_29","unstructured":"(2024, October 03). Molecular and Functional Characterization of Novel Glycerol-3-Phosphate Dehydrogenase 1\u2013Like Gene (GPD1-L) Mutations in Sudden Infant Death Syndrome | Circulation. Available online: https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCULATIONAHA.107.704627."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1161\/CIRCEP.111.969972","article-title":"A Novel Disease Gene for Brugada Syndrome","volume":"5","author":"Ishikawa","year":"2012","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1038\/ng1461","article-title":"Mutations in the Desmosomal Protein Plakophilin-2 Are Common in Arrhythmogenic Right Ventricular Cardiomyopathy","volume":"36","author":"Gerull","year":"2004","journal-title":"Nat. Genet."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1161\/01.CIR.103.5.710","article-title":"Right Bundle Branch Block, Right Precordial St-Segment Elevation, and Sudden Death in Young People","volume":"103","author":"Corrado","year":"2001","journal-title":"Circulation"},{"key":"ref_33","unstructured":"(2024, September 30). Epicardial Ventricular Tachycardia Ablation in a Patient with Brugada ECG Pattern and Mutation of PKP2 and DSP Genes | Circulation: Arrhythmia and Electrophysiology. Available online: https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCEP.114.002342."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1161\/CIRCULATIONAHA.118.035070","article-title":"Reappraisal of Reported Genes for Sudden Arrhythmic Death: Evidence-Based Evaluation of Gene Validity for Brugada Syndrome","volume":"138","author":"Hosseini","year":"2018","journal-title":"Circulation"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1161\/CIRCULATIONAHA.106.668392","article-title":"Loss-of-Function Mutations in the Cardiac Calcium Channel Underlie a New Clinical Entity Characterized by ST-Segment Elevation, Short QT Intervals, and Sudden Cardiac Death","volume":"115","author":"Antzelevitch","year":"2007","journal-title":"Circulation"},{"key":"ref_36","doi-asserted-by":"crossref","unstructured":"Liu, H., Chatel, S., Simard, C., Syam, N., Salle, L., Probst, V., Morel, J., Millat, G., Lopez, M., and Abriel, H. (2013). Molecular Genetics and Functional Anomalies in a Series of 248 Brugada Cases with 11 Mutations in the TRPM4 Channel. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0054131"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1161\/CIRCEP.110.959619","article-title":"KCNE5 (KCNE1L) Variants Are Novel Modulators of Brugada Syndrome and Idiopathic Ventricular Fibrillation","volume":"4","author":"Ohno","year":"2011","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1016\/j.cardiores.2005.06.027","article-title":"Role of Sequence Variations in the Human Ether-a-Go-Go-Related Gene (HERG, KCNH2) in the Brugada Syndrome","volume":"68","author":"Verkerk","year":"2005","journal-title":"Cardiovasc. Res."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1161\/CIRCRESAHA.115.303657","article-title":"Characterization of SEMA3A-Encoded Semaphorin as a Naturally Occurring Kv4.3 Protein Inhibitor and Its Contribution to Brugada Syndrome","volume":"115","author":"Boczek","year":"2014","journal-title":"Circ. Res."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1161\/CIRCEP.107.748103","article-title":"Functional Effects of KCNE3 Mutation and Its Role in the Development of Brugada Syndrome","volume":"1","author":"Cordeiro","year":"2008","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1016\/j.hrthm.2011.10.035","article-title":"Molecular Genetic and Functional Association of Brugada and Early Repolarization Syndromes with S422L Missense Mutation in KCNJ8","volume":"9","author":"Hu","year":"2012","journal-title":"Heart Rhythm"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1024","DOI":"10.1016\/j.hrthm.2011.02.021","article-title":"Transient Outward Current (Ito) Gain-of-Function Mutations in the KCND3-Encoded Kv4.3 Potassium Channel and Brugada Syndrome","volume":"8","author":"Giudicessi","year":"2011","journal-title":"Heart Rhythm"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1007\/s00424-016-1870-1","article-title":"Mutation in S6 Domain of HCN4 Channel in Patient with Suspected Brugada Syndrome Modifies Channel Function","volume":"468","author":"Biel","year":"2016","journal-title":"Pfl\u00fcg. Arch. Eur. J. Physiol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1016\/j.ijcard.2013.12.084","article-title":"ABCC9 Is a Novel Brugada and Early Repolarization Syndrome Susceptibility Gene","volume":"171","author":"Hu","year":"2014","journal-title":"Int. J. Cardiol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/s00399-023-00937-4","article-title":"[Asymptomatic channelopathies: Risk stratification and primary prophylaxis]","volume":"34","author":"Aweimer","year":"2023","journal-title":"Herzschrittmacherther. Elektrophysiol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1002\/clc.23247","article-title":"Implantable Cardioverter-Defibrillator in Brugada Syndrome: Long-Term Follow-Up","volume":"42","author":"Roterberg","year":"2019","journal-title":"Clin. Cardiol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"e004185","DOI":"10.1161\/CIRCEP.116.004185","article-title":"Management of Brugada Syndrome 2016: Should All High Risk Patients Receive an ICD?","volume":"9","author":"Belhassen","year":"2016","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_48","unstructured":"(2023, September 05). ESC Guidelines on Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death. Available online: https:\/\/www.escardio.org\/Guidelines\/Clinical-Practice-Guidelines\/Ventricular-Arrhythmias-and-the-Prevention-of-Sudden-Cardiac-Death."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1660","DOI":"10.1161\/01.CIR.100.15.1660","article-title":"Cellular Basis for the Brugada Syndrome and Other Mechanisms of Arrhythmogenesis Associated With ST-Segment Elevation","volume":"100","author":"Yan","year":"1999","journal-title":"Circulation"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"H548","DOI":"10.1152\/ajpheart.1996.271.2.H548","article-title":"I(to) and Action Potential Notch Are Smaller in Left vs. Right Canine Ventricular Epicardium","volume":"271","author":"Sun","year":"1996","journal-title":"Am. J. Physiol.-Heart Circ. Physiol."},{"key":"ref_51","unstructured":"(2023, September 25). Transient Outward Current Prominent in Canine Ventricular Epicardium but Not Endocardium | Circulation Research. Available online: https:\/\/www.ahajournals.org\/doi\/abs\/10.1161\/01.RES.62.1.116."},{"key":"ref_52","unstructured":"(2023, September 25). Ionic and Cellular Basis for the Predominance of the Brugada Syndrome Phenotype in Males | Circulation. Available online: https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/01.CIR.0000032002.22105.7A."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1161\/CIRCULATIONAHA.110.972612","article-title":"Prevention of Ventricular Fibrillation Episodes in Brugada Syndrome by Catheter Ablation Over the Anterior Right Ventricular Outflow Tract Epicardium","volume":"123","author":"Nademanee","year":"2011","journal-title":"Circulation"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1016\/j.cardiores.2005.03.005","article-title":"Pathophysiological Mechanisms of Brugada Syndrome: Depolarization Disorder, Repolarization Disorder, or More?","volume":"67","author":"Meregalli","year":"2005","journal-title":"Cardiovasc. Res."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/S0008-6363(02)00242-0","article-title":"Body Surface Potential Mapping in Patients with Brugada Syndrome: Right Precordial ST Segment Variations and Reverse Changes in Left Precordial Leads","volume":"54","author":"Bruns","year":"2002","journal-title":"Cardiovasc. Res."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.cardiores.2004.02.016","article-title":"Intercellular Coupling through Gap Junctions Masks M Cells in the Human Heart","volume":"62","author":"Conrath","year":"2004","journal-title":"Cardiovasc. Res."},{"key":"ref_57","first-page":"74","article-title":"A 35-Year Effective Treatment of Catecholaminergic Polymorphic Ventricular Tachycardia with Propafenone","volume":"5","author":"Marx","year":"2018","journal-title":"Heart Rhythm Case Rep."},{"key":"ref_58","doi-asserted-by":"crossref","unstructured":"Kallas, D., Lamba, A., Roston, T.M., Arslanova, A., Franciosi, S., Tibbits, G.F., and Sanatani, S. (2021). Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: A Translational Perspective for the Clinician-Scientist. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22179293"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1038\/s10038-020-0738-6","article-title":"Classification and Correlation of RYR2 Missense Variants in Individuals with Catecholaminergic Polymorphic Ventricular Tachycardia Reveals Phenotypic Relationships","volume":"65","author":"Olubando","year":"2020","journal-title":"J. Hum. Genet."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1136\/jmg.2004.028993","article-title":"Catecholaminergic Polymorphic Ventricular Tachycardia: RYR2 Mutations, Bradycardia, and Follow up of the Patients","volume":"42","author":"Postma","year":"2005","journal-title":"J. Med. Genet."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"e20","DOI":"10.15420\/aer.2022.09","article-title":"Catecholaminergic Polymorphic Ventricular Tachycardia","volume":"11","author":"Abbas","year":"2022","journal-title":"Arrhythmia Electrophysiol. Rev."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/j.pcad.2019.05.006","article-title":"Sudden Cardiac Death in Long QT Syndrome (LQTS), Brugada Syndrome, and Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)","volume":"62","author":"Singh","year":"2019","journal-title":"Prog. Cardiovasc. Dis."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/j.ccep.2023.04.002","article-title":"Catecholaminergic Polymorphic Ventricular Tachycardia: A Review of Therapeutic Strategies","volume":"15","author":"Bergeman","year":"2023","journal-title":"Card. Electrophysiol. Clin."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1186\/2044-5040-1-18","article-title":"Ryanodine Receptors","volume":"1","author":"Capes","year":"2011","journal-title":"Skelet. Muscle"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"2592","DOI":"10.1016\/j.jacc.2020.12.073","article-title":"Precision Medicine in Catecholaminergic Polymorphic Ventricular Tachycardia: JACC Focus Seminar 5\/5","volume":"77","author":"Priori","year":"2021","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"100439","DOI":"10.1016\/j.genrep.2019.100439","article-title":"A de Novo Heterozygous Cardiac Ryanodine Receptor Gene (RYR2) Mutation in a Catecholaminergic Polymorphic Ventricular Tachycardia Patient","volume":"16","author":"Hou","year":"2019","journal-title":"Gene Rep."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1016\/j.yjmcc.2016.08.020","article-title":"Regulation of Sarcoplasmic Reticulum Ca2+ Release by Serine-Threonine Phosphatases in the Heart","volume":"101","author":"Terentyev","year":"2016","journal-title":"J. Mol. Cell Cardiol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"E1669","DOI":"10.1073\/pnas.1419795112","article-title":"Arrhythmogenesis in a Catecholaminergic Polymorphic Ventricular Tachycardia Mutation That Depresses Ryanodine Receptor Function","volume":"112","author":"Zhao","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1500","DOI":"10.1093\/eurheartj\/ehab687","article-title":"Evaluation of Gene Validity for CPVT and Short QT Syndrome in Sudden Arrhythmic Death","volume":"43","author":"Walsh","year":"2022","journal-title":"Eur. Heart J."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1390","DOI":"10.15252\/emmm.201505719","article-title":"TECRL, a New Life-Threatening Inherited Arrhythmia Gene Associated with Overlapping Clinical Features of Both LQTS and CPVT","volume":"8","author":"Devalla","year":"2016","journal-title":"EMBO Mol. Med."},{"key":"ref_71","doi-asserted-by":"crossref","unstructured":"Deb, A., Tow, B.D., Qing, Y., Walker, M., Hodges, E.R., Stewart, J.A., Knollmann, B.C., Zheng, Y., Wang, Y., and Liu, B. (2023). Genetic Inhibition of Mitochondrial Permeability Transition Pore Exacerbates Ryanodine Receptor 2 Dysfunction in Arrhythmic Disease. Cells, 12.","DOI":"10.3390\/cells12020204"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1080\/19336950.2017.1344799","article-title":"FK506-Binding Proteins 12 and 12.6 (FKBPs) as Regulators of Cardiac Ryanodine Receptors: Insights from New Functional and Structural Knowledge","volume":"11","author":"Gonano","year":"2017","journal-title":"Channels"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"13062","DOI":"10.1073\/pnas.0402388101","article-title":"RyR2 Mutations Linked to Ventricular Tachycardia and Sudden Death Reduce the Threshold for Store-Overload-Induced Ca2+ Release (SOICR)","volume":"101","author":"Jiang","year":"2004","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1016\/j.joa.2015.09.008","article-title":"Current Topics in Catecholaminergic Polymorphic Ventricular Tachycardia","volume":"32","author":"Sumitomo","year":"2016","journal-title":"J. Arrhythm."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"E112","DOI":"10.1093\/eurheartj\/suab104","article-title":"Ventricular Fibrillation Ablation in Cardiomyopathies and Arrhythmic Storm","volume":"23","author":"Pappone","year":"2021","journal-title":"Eur. Heart J. Suppl."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/j.tcm.2020.04.011","article-title":"The Role of the Autonomic Nervous System in Cardiac Arrhythmias: The Neuro-Cardiac Axis, More Foe than Friend?","volume":"31","author":"Manolis","year":"2021","journal-title":"Trends Cardiovasc. Med."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1483","DOI":"10.1161\/CIRCRESAHA.110.227744","article-title":"In the RyR2(R4496C) Mouse Model of CPVT, \u03b2-Adrenergic Stimulation Induces Ca Waves by Increasing SR Ca Content and Not by Decreasing the Threshold for Ca Waves","volume":"107","author":"Kashimura","year":"2010","journal-title":"Circ. Res."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1661","DOI":"10.1161\/CIRCRESAHA.111.243956","article-title":"Calmodulin-Dependent Protein Kinase II: Linking Heart Failure and Arrhythmias","volume":"110","author":"Swaminathan","year":"2012","journal-title":"Circ. Res."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"e004161","DOI":"10.1161\/CIRCEP.116.004161","article-title":"Novel CPVT-Associated Calmodulin Mutation in CALM3 (CALM3-A103V) Activates Arrhythmogenic Ca Waves and Sparks","volume":"9","author":"Boczek","year":"2016","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1161\/01.CIR.103.2.196","article-title":"Mutations in the Cardiac Ryanodine Receptor Gene (hRyR2) Underlie Catecholaminergic Polymorphic Ventricular Tachycardia","volume":"103","author":"Priori","year":"2001","journal-title":"Circulation"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"9137","DOI":"10.1073\/pnas.0402546101","article-title":"A Cardiac Arrhythmia Syndrome Caused by Loss of Ankyrin-B Function","volume":"101","author":"Mohler","year":"2004","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_82","first-page":"A14699","article-title":"Abstract 14699: Spectrum and Prevalence of CALM1, CALM2, and CALM3 Mutations in Long QT Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia, Idiopathic Ventricular Fibrillation, and Sudden Unexplained Death in the Young","volume":"128","author":"Boczek","year":"2013","journal-title":"Circulation"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"7636","DOI":"10.1073\/pnas.0902919106","article-title":"Ablation of triadin causes loss of cardiac Ca2+ release units, impaired excitation\u2013contraction coupling, and cardiac arrhythmias","volume":"106","author":"Chopra","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"1378","DOI":"10.1086\/324565","article-title":"A Missense Mutation in a Highly Conserved Region of CASQ2 Is Associated with Autosomal Recessive Catecholamine-Induced Polymorphic Ventricular Tachycardia in Bedouin Families from Israel","volume":"69","author":"Lahat","year":"2001","journal-title":"Am. J. Hum. Genet."},{"key":"ref_85","first-page":"66","article-title":"Involvement of the Kir2 Gene Family in Catecholaminergic Polymorphic Ventricular Tachycardia; Analysis for Mutations and Identification of Numerous Pseudogenes","volume":"25","author":"Postma","year":"2004","journal-title":"Eur. Heart J."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1016\/j.cell.2007.11.019","article-title":"Induction of Pluripotent Stem Cells from Adult Human Fibroblasts by Defined Factors","volume":"131","author":"Takahashi","year":"2007","journal-title":"Cell"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1513","DOI":"10.1161\/CIRCULATIONAHA.109.868885","article-title":"Cardiomyocyte Differentiation of Human Induced Pluripotent Stem Cells","volume":"120","author":"Zwi","year":"2009","journal-title":"Circulation"},{"key":"ref_88","doi-asserted-by":"crossref","unstructured":"(2023, September 11). Generation of Integration-Free Induced Pluripotent Stem Cells from Urine-Derived Cells Isolated from Individuals with Down Syndrome | Stem Cells Translational Medicine | Oxford Academic. Available online: https:\/\/academic.oup.com\/stcltm\/article\/6\/6\/1465\/6398814.","DOI":"10.1002\/sctm.16-0128"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"558","DOI":"10.5966\/sctm.2013-0006","article-title":"Blood Cell-Derived Induced Pluripotent Stem Cells Free of Reprogramming Factors Generated by Sendai Viral Vectors","volume":"2","author":"Ye","year":"2013","journal-title":"Stem Cells Transl. Med."},{"key":"ref_90","doi-asserted-by":"crossref","unstructured":"Zhong, R., Schimanski, T., Zhang, F., Lan, H., Hohn, A., Xu, Q., Huang, M., Liao, Z., Qiao, L., and Yang, Z. (2022). A Preclinical Study on Brugada Syndrome with a CACNB2 Variant Using Human Cardiomyocytes from Induced Pluripotent Stem Cells. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23158313"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1111\/pcmr.12369","article-title":"NF1 Loss Induces Senescence during Human Melanocyte Differentiation in an iPSC-Based Model","volume":"28","author":"Larribere","year":"2015","journal-title":"Pigment. Cell Melanoma Res."},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Y\u00fccel, G., Zhao, Z., El-Battrawy, I., Lan, H., Lang, S., Li, X., Buljubasic, F., Zimmermann, W.-H., Cyganek, L., and Utikal, J. (2017). Lipopolysaccharides Induced Inflammatory Responses and Electrophysiological Dysfunctions in Human-Induced Pluripotent Stem Cell Derived Cardiomyocytes. Sci. Rep., 7.","DOI":"10.1038\/s41598-017-03147-4"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"6067096","DOI":"10.1155\/2018\/6067096","article-title":"Ion Channel Expression and Characterization in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes","volume":"2018","author":"Zhao","year":"2018","journal-title":"Stem Cells Int."},{"key":"ref_94","unstructured":"(2023, September 11). Viral Vectors: A Look Back and Ahead on Gene Transfer Technology. Available online: https:\/\/arpi.unipi.it\/handle\/11568\/1029349."},{"key":"ref_95","doi-asserted-by":"crossref","unstructured":"Hubbard, J.J., Sullivan, S.K., Mills, J.A., Hayes, B.J., Torok-Storb, B.J., and Ramakrishnan, A. (2014). Efficient iPS Cell Generation from Blood Using Episomes and HDAC Inhibitors. J. Vis. Exp., 52009.","DOI":"10.3791\/52009"},{"key":"ref_96","unstructured":"(2023, September 30). Generation of Human Induced Pluripotent Stem Cells (hiPSCs) from Fibroblasts Using Episomal Vectors\u2014DE. Available online: https:\/\/www.thermofisher.com\/de\/de\/home\/references\/protocols\/cell-culture\/stem-cell-protocols\/ipsc-protocols\/generation-human-induced-pluripotent-stem-cells-fibroblasts.html."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"348","DOI":"10.2183\/pjab.85.348","article-title":"Efficient Induction of Transgene-Free Human Pluripotent Stem Cells Using a Vector Based on Sendai Virus, an RNA Virus That Does Not Integrate into the Host Genome","volume":"85","author":"Fusaki","year":"2009","journal-title":"Proc. Jpn. Acad. Ser. B"},{"key":"ref_98","doi-asserted-by":"crossref","unstructured":"Yang, W. (2014). iPSC Reprogramming from Human Peripheral Blood Using Sendai Virus Mediated Gene Transfer. StemBook, Harvard Stem Cell Institute.","DOI":"10.3824\/stembook.1.73.1"},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"101926","DOI":"10.1016\/j.scr.2020.101926","article-title":"Establishment and Characterization of Two iPSC Lines Derived from Healthy Controls","volume":"47","author":"Patel","year":"2020","journal-title":"Stem Cell Res."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"1410","DOI":"10.1093\/europace\/euz122","article-title":"A Cellular Model of Brugada Syndrome with SCN10A Variants Using Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes","volume":"21","author":"Albers","year":"2019","journal-title":"Europace"},{"key":"ref_101","doi-asserted-by":"crossref","unstructured":"Buljubasic, F., El-Battrawy, I., Lan, H., Lomada, S.K., Chatterjee, A., Zhao, Z., Li, X., Zhong, R., Xu, Q., and Huang, M. (2020). Nucleoside Diphosphate Kinase B Contributes to Arrhythmogenesis in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes from a Patient with Arrhythmogenic Right Ventricular Cardiomyopathy. J. Clin. Med., 9.","DOI":"10.3390\/jcm9020486"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1016\/j.ymthe.2018.12.009","article-title":"mRNA-Based Genetic Reprogramming","volume":"27","author":"Warren","year":"2019","journal-title":"Mol. Ther."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1161\/CIRCRESAHA.114.300558","article-title":"Engineering Adolescence: Maturation of Human Pluripotent Stem Cell-Derived Cardiomyocytes","volume":"114","author":"Yang","year":"2014","journal-title":"Circ. Res."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"990","DOI":"10.1016\/j.jacc.2012.02.066","article-title":"Modeling of Catecholaminergic Polymorphic Ventricular Tachycardia with Patient-Specific Human-Induced Pluripotent Stem Cells","volume":"60","author":"Itzhaki","year":"2012","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1704","DOI":"10.1089\/scd.2013.0509","article-title":"A Simple, Cost-Effective but Highly Efficient System for Deriving Ventricular Cardiomyocytes from Human Pluripotent Stem Cells","volume":"23","author":"Weng","year":"2014","journal-title":"Stem Cells Dev."},{"key":"ref_106","doi-asserted-by":"crossref","unstructured":"Zhao, M., Tang, Y., Zhou, Y., and Zhang, J. (2019). Deciphering Role of Wnt Signalling in Cardiac Mesoderm and Cardiomyocyte Differentiation from Human iPSCs: Four-Dimensional Control of Wnt Pathway for hiPSC-CMs Differentiation. Sci. Rep., 9.","DOI":"10.1038\/s41598-019-55620-x"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"e99941","DOI":"10.1172\/jci.insight.99941","article-title":"Deep Phenotyping of Human Induced Pluripotent Stem Cell\u2013Derived Atrial and Ventricular Cardiomyocytes","volume":"3","author":"Cyganek","year":"2018","journal-title":"JCI Insight"},{"key":"ref_108","doi-asserted-by":"crossref","unstructured":"Ma, D., Liu, Z., Loh, L.J., Zhao, Y., Li, G., Liew, R., Islam, O., Wu, J., Chung, Y.Y., and Teo, W.S. (2018). Identification of an INa-Dependent and Ito-Mediated Proarrhythmic Mechanism in Cardiomyocytes Derived from Pluripotent Stem Cells of a Brugada Syndrome Patient. Sci. Rep., 8.","DOI":"10.1038\/s41598-018-29574-5"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1161\/CIRCRESAHA.119.315378","article-title":"Workshop Report","volume":"125","author":"Pang","year":"2019","journal-title":"Circ. Res."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1038\/nbt.3749","article-title":"Influence of Donor Age on Induced Pluripotent Stem Cells","volume":"35","author":"Ferguson","year":"2017","journal-title":"Nat. Biotechnol."},{"key":"ref_111","doi-asserted-by":"crossref","unstructured":"Ahmed, R.E., Anzai, T., Chanthra, N., and Uosaki, H. (2020). A Brief Review of Current Maturation Methods for Human Induced Pluripotent Stem Cells-Derived Cardiomyocytes. Front. Cell Dev. Biol., 8.","DOI":"10.3389\/fcell.2020.00178"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1038\/s41586-020-2797-4","article-title":"Cells of the Adult Human Heart","volume":"588","author":"Maatz","year":"2020","journal-title":"Nature"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1161\/CIRCRESAHA.115.307778","article-title":"Revisiting Cardiac Cellular Composition","volume":"118","author":"Pinto","year":"2016","journal-title":"Circ. Res."},{"key":"ref_114","doi-asserted-by":"crossref","unstructured":"Sharma, P., and Gentile, C. (2021). Cardiac Spheroids as in Vitro Bioengineered Heart Tissues to Study Human Heart Pathophysiology. J. Vis. Exp., e61962.","DOI":"10.3791\/61962-v"},{"key":"ref_115","doi-asserted-by":"crossref","unstructured":"Ergir, E., Oliver-De La Cruz, J., Fernandes, S., Cassani, M., Niro, F., Pereira-Sousa, D., Vrbsk\u00fd, J., Vinarsk\u00fd, V., Perestrelo, A.R., and Debellis, D. (2022). Generation and Maturation of Human iPSC-Derived 3D Organotypic Cardiac Microtissues in Long-Term Culture. Sci. Rep., 12.","DOI":"10.1038\/s41598-022-22225-w"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1089\/ten.tec.2014.0376","article-title":"Development and Characterization of a Scaffold-Free 3D Spheroid Model of Induced Pluripotent Stem Cell-Derived Human Cardiomyocytes","volume":"21","author":"Beauchamp","year":"2015","journal-title":"Tissue Eng. Part C Methods"},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"862","DOI":"10.1016\/j.stem.2020.05.004","article-title":"Human-iPSC-Derived Cardiac Stromal Cells Enhance Maturation in 3D Cardiac Microtissues and Reveal Non-Cardiomyocyte Contributions to Heart Disease","volume":"26","author":"Giacomelli","year":"2020","journal-title":"Cell Stem Cell"},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.1089\/ten.tea.2016.0027","article-title":"Three-Dimensional Adult Cardiac Extracellular Matrix Promotes Maturation of Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes","volume":"22","author":"Fong","year":"2016","journal-title":"Tissue Eng. Part A"},{"key":"ref_119","doi-asserted-by":"crossref","unstructured":"Zhang, M., Xu, Y., Chen, Y., Yan, Q., Li, X., Ding, L., Wei, T., and Zeng, D. (2022). Three-Dimensional Poly-(\u03b5-Caprolactone) Nanofibrous Scaffolds Promote the Maturation of Human Pluripotent Stem Cells-Induced Cardiomyocytes. Front. Cell Dev. Biol., 10.","DOI":"10.3389\/fcell.2022.875278"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"1932","DOI":"10.1002\/jbm.a.37428","article-title":"Compatibility and Function of Human Induced Pluripotent Stem Cell Derived Cardiomyocytes on an Electrospun Nanofibrous Scaffold, Generated from an Ionomeric Polyurethane Composite","volume":"110","author":"Chen","year":"2022","journal-title":"J. Biomed. Mater. Res. Part A"},{"key":"ref_121","doi-asserted-by":"crossref","unstructured":"Hecker, M., and Duncker, D.J. (2023). Biophysical Stretch Induced Differentiation and Maturation of Induced Pluripotent Stem Cell-Derived Cardiomyocytes. Cardiac Mechanobiology in Physiology and Disease, Springer International Publishing. Cardiac and Vascular Biology.","DOI":"10.1007\/978-3-031-23965-6"},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Hong, Y., Zhao, Y., Li, H., Yang, Y., Chen, M., Wang, X., Luo, M., and Wang, K. (2023). Engineering the Maturation of Stem Cell-Derived Cardiomyocytes. Front. Bioeng. Biotechnol., 11.","DOI":"10.3389\/fbioe.2023.1155052"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"e96291","DOI":"10.1172\/jci.insight.96291","article-title":"Brugada Syndrome Trafficking\u2013Defective Nav1.5 Channels Can Trap Cardiac Kir2.1\/2.2 Channels","volume":"3","author":"Matamoros","year":"2018","journal-title":"JCI Insight"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"3079","DOI":"10.1161\/CIRCULATIONAHA.111.066092","article-title":"Cardiomyocytes Derived from Pluripotent Stem Cells Recapitulate Electrophysiological Characteristics of an Overlap Syndrome of Cardiac Sodium Channel Disease","volume":"125","author":"Davis","year":"2012","journal-title":"Circulation"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"e004227","DOI":"10.1161\/CIRCEP.116.004227","article-title":"Readthrough-Promoting Drugs Gentamicin and PTC124 Fail to Rescue Nav1.5 Function of Human-Induced Pluripotent Stem Cell\u2013Derived Cardiomyocytes Carrying Nonsense Mutations in the Sodium Channel Gene SCN5A","volume":"9","author":"Kosmidis","year":"2016","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"2086","DOI":"10.1016\/j.jacc.2016.07.779","article-title":"Patient-Specific and Genome-Edited Induced Pluripotent Stem Cell\u2013Derived Cardiomyocytes Elucidate Single-Cell Phenotype of Brugada Syndrome","volume":"68","author":"Liang","year":"2016","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.yjmcc.2017.10.002","article-title":"Sodium Channel Current Loss of Function in Induced Pluripotent Stem Cell-Derived Cardiomyocytes from a Brugada Syndrome Patient","volume":"114","author":"Selga","year":"2018","journal-title":"J. Mol. Cell. Cardiol."},{"key":"ref_128","doi-asserted-by":"crossref","unstructured":"de la Roche, J., Angsutararux, P., Kempf, H., Janan, M., Bolesani, E., Thiemann, S., Wojciechowski, D., Coffee, M., Franke, A., and Schwanke, K. (2019). Comparing Human iPSC-Cardiomyocytes versus HEK293T Cells Unveils Disease-Causing Effects of Brugada Mutation A735V of NaV1.5 Sodium Channels. Sci. Rep., 9.","DOI":"10.1038\/s41598-019-47632-4"},{"key":"ref_129","unstructured":"(2024, October 08). Embryonic Type Na+ Channel \u03b2-Subunit, SCN3B Masks the Disease Phenotype of BRUGADA Syndrome | Scientific Reports. Available online: https:\/\/www.nature.com\/articles\/srep34198."},{"key":"ref_130","doi-asserted-by":"crossref","unstructured":"El-Battrawy, I., M\u00fcller, J., Zhao, Z., Cyganek, L., Zhong, R., Zhang, F., Kleinsorge, M., Lan, H., Li, X., and Xu, Q. (2019). Studying Brugada Syndrome with an SCN1B Variants in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes. Front. Cell Dev. Biol., 7.","DOI":"10.3389\/fcell.2019.00261"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"2028","DOI":"10.1093\/europace\/euac091","article-title":"Epigenetic Mechanism of L-Type Calcium Channel \u03b2-Subunit Downregulation in Short QT Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes with CACNB2 Mutation","volume":"24","author":"Zhong","year":"2022","journal-title":"Europace"},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"3081","DOI":"10.1093\/eurheartj\/ehz308","article-title":"RRAD Mutation Causes Electrical and Cytoskeletal Defects in Cardiomyocytes Derived from a Familial Case of Brugada Syndrome","volume":"40","author":"Belbachir","year":"2019","journal-title":"Eur. Heart J."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/j.scr.2017.11.003","article-title":"Ajmaline Blocks INa and IKr without Eliciting Differences between Brugada Syndrome Patient and Control Human Pluripotent Stem Cell-Derived Cardiac Clusters","volume":"25","author":"Miller","year":"2017","journal-title":"Stem Cell Res."},{"key":"ref_134","doi-asserted-by":"crossref","unstructured":"Veerman, C.C., Mengarelli, I., Guan, K., Stauske, M., Barc, J., Tan, H.L., Wilde, A.A.M., Verkerk, A.O., and Bezzina, C.R. (2016). hiPSC-Derived Cardiomyocytes from Brugada Syndrome Patients without Identified Mutations Do Not Exhibit Clear Cellular Electrophysiological Abnormalities. Sci. Rep., 6.","DOI":"10.1038\/srep30967"},{"key":"ref_135","doi-asserted-by":"crossref","unstructured":"Li, W., Stauske, M., Luo, X., Wagner, S., Vollrath, M., Mehnert, C.S., Schubert, M., Cyganek, L., Chen, S., and Hasheminasab, S.-M. (2020). Disease Phenotypes and Mechanisms of iPSC-Derived Cardiomyocytes from Brugada Syndrome Patients with a Loss-of-Function SCN5A Mutation. Front. Cell Dev. Biol., 8.","DOI":"10.3389\/fcell.2020.592893"},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"102719","DOI":"10.1016\/j.scr.2022.102719","article-title":"Generation of Two Induced Pluripotent Stem Cell (iPSC) Lines (BBANTWi006-A, BBANTWi007-A) from Brugada Syndrome Patients Carrying an SCN5A Mutation","volume":"60","author":"Simons","year":"2022","journal-title":"Stem Cell Res."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.hrthm.2022.08.021","article-title":"Disrupted CaV1.2 Selectivity Causes Overlapping Long QT and Brugada Syndrome Phenotypes in the CACNA1C-E1115K iPS Cell Model","volume":"20","author":"Kashiwa","year":"2023","journal-title":"Heart Rhythm"},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"e1130","DOI":"10.1002\/ctm2.1130","article-title":"Novel Insights in the Pathomechanism of Brugada Syndrome and Fever-Related Type 1 ECG Changes in a Preclinical Study Using Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes","volume":"13","author":"Li","year":"2023","journal-title":"Clin. Transl. Med."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1186\/s13287-023-03477-3","article-title":"Patient-Specific iPSC-Derived Cardiomyocytes Reveal Aberrant Activation of Wnt\/\u03b2-Catenin Signaling in SCN5A-Related Brugada Syndrome","volume":"14","author":"Cai","year":"2023","journal-title":"Stem Cell Res. Ther."},{"key":"ref_140","doi-asserted-by":"crossref","unstructured":"Kamga, M.V.K., Reppel, M., Hescheler, J., and Nguemo, F. (2021). Modeling Genetic Cardiac Channelopathies Using Induced Pluripotent Stem Cells\u2014Status Quo from an Electrophysiological Perspective. Biochem. Pharmacol., 192.","DOI":"10.1016\/j.bcp.2021.114746"},{"key":"ref_141","first-page":"8842398","article-title":"Susceptibility to Ventricular Arrhythmias Resulting from Mutations in FKBP1B, PXDNL, and SCN9A Evaluated in hiPSC Cardiomyocytes","volume":"2020","author":"Smith","year":"2020","journal-title":"Stem Cells Int."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"1677","DOI":"10.1161\/CIRCULATIONAHA.113.001883","article-title":"Drug Screening Using a Library of Human Induced Pluripotent Stem Cell\u2013Derived Cardiomyocytes Reveals Disease-Specific Patterns of Cardiotoxicity","volume":"127","author":"Liang","year":"2013","journal-title":"Circulation"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"395","DOI":"10.7555\/JBR.35.20210045","article-title":"Pathogenesis and Drug Response of iPSC-Derived Cardiomyocytes from Two Brugada Syndrome Patients with Different Nav1.5-Subunit Mutations","volume":"35","author":"Zhu","year":"2021","journal-title":"J. Biomed. Res."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1093\/cvr\/cvab045","article-title":"Tbx5 Variants Disrupt Nav1.5 Function Differently in Patients Diagnosed with Brugada or Long QT Syndrome","volume":"118","author":"Tinaquero","year":"2022","journal-title":"Cardiovasc. Res."},{"key":"ref_145","doi-asserted-by":"crossref","unstructured":"Koivum\u00e4ki, J.T., Naumenko, N., Tuomainen, T., Takalo, J., Oksanen, M., Puttonen, K.A., Lehtonen, \u0160., Kuusisto, J., Laakso, M., and Koistinaho, J. (2018). Structural Immaturity of Human iPSC-Derived Cardiomyocytes: In Silico Investigation of Effects on Function and Disease Modeling. Front. Physiol., 9.","DOI":"10.3389\/fphys.2018.00080"},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"102259","DOI":"10.1016\/j.scr.2021.102259","article-title":"Generation of iPSC Lines from CPVT Patient Carrying Heterozygous Mutation p.A2254V in the Ryanodine Receptor 2 Gene","volume":"53","author":"Li","year":"2021","journal-title":"Stem Cell Res."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1002\/emmm.201100194","article-title":"Dantrolene Rescues Arrhythmogenic RYR2 Defect in a Patient-Specific Stem Cell Model of Catecholaminergic Polymorphic Ventricular Tachycardia","volume":"4","author":"Jung","year":"2012","journal-title":"EMBO Mol. Med."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1007\/s11033-019-05201-y","article-title":"Mutation-Specific Differences in Arrhythmias and Drug Responses in CPVT Patients: Simultaneous Patch Clamp and Video Imaging of iPSC Derived Cardiomyocytes","volume":"47","author":"Swan","year":"2020","journal-title":"Mol. Biol. Rep."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1242\/dmm.026823","article-title":"A Human Pluripotent Stem Cell Model of Catecholaminergic Polymorphic Ventricular Tachycardia Recapitulates Patient-Specific Drug Responses","volume":"9","author":"Preininger","year":"2016","journal-title":"Dis. Models Mech."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"2006","DOI":"10.1111\/jcmm.12581","article-title":"Functional Abnormalities in iPSC-Derived Cardiomyocytes Generated from CPVT1 and CPVT2 Patients Carrying Ryanodine or Calsequestrin Mutations","volume":"19","author":"Novak","year":"2015","journal-title":"J. Cell. Mol. Med."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"e011387","DOI":"10.1161\/CIRCEP.122.011387","article-title":"Novel Calmodulin Variant p.E46K Associated with Severe Catecholaminergic Polymorphic Ventricular Tachycardia Produces Robust Arrhythmogenicity in Human Induced Pluripotent Stem Cell\u2013Derived Cardiomyocytes","volume":"16","author":"Gao","year":"2023","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"e004725","DOI":"10.1161\/CIRCEP.116.004725","article-title":"Patient-Specific Drug Screening Using a Human Induced Pluripotent Stem Cell Model of Catecholaminergic Polymorphic Ventricular Tachycardia Type 2","volume":"10","author":"Maizels","year":"2017","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_153","doi-asserted-by":"crossref","unstructured":"Nijak, A., Saenen, J., Labro, A.J., Schepers, D., Loeys, B.L., and Alaerts, M. (2021). iPSC-Cardiomyocyte Models of Brugada Syndrome\u2014Achievements, Challenges and Future Perspectives. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22062825"},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"12686","DOI":"10.3390\/ijms160612686","article-title":"Single Cell Electrical Characterization Techniques","volume":"16","author":"Mansor","year":"2015","journal-title":"Int. J. Mol. Sci."},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"101662","DOI":"10.1016\/j.scr.2019.101662","article-title":"Establishment of an Automated Patch-Clamp Platform for Electrophysiological and Pharmacological Evaluation of hiPSC-CMs","volume":"41","author":"Li","year":"2019","journal-title":"Stem Cell Res."},{"key":"ref_156","unstructured":"(2024, September 30). Forced Aggregation and Defined Factors Allow Highly Uniform-Sized Embryoid Bodies and Functional Cardiomyocytes from Human Embryonic and Induced Pluripotent Stem Cells | Heart and Vessels. Available online: https:\/\/link.springer.com\/article\/10.1007\/s00380-013-0436-9."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1016\/j.bios.2016.05.073","article-title":"Atomic Force Microscopy Combined with Human Pluripotent Stem Cell Derived Cardiomyocytes for Biomechanical Sensing","volume":"85","author":"Pesl","year":"2016","journal-title":"Biosens. Bioelectron."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"5427","DOI":"10.1038\/s41467-024-49755-3","article-title":"Tracking Single hiPSC-Derived Cardiomyocyte Contractile Function Using CONTRAX an Efficient Pipeline for Traction Force Measurement","volume":"15","author":"Pardon","year":"2024","journal-title":"Nat. Commun."},{"key":"ref_159","doi-asserted-by":"crossref","unstructured":"Miccoli, B., Lopez, C.M., Goikoetxea, E., Putzeys, J., Sekeri, M., Krylychkina, O., Chang, S.-W., Firrincieli, A., Andrei, A., and Reumers, V. (2019). High-Density Electrical Recording and Impedance Imaging with a Multi-Modal CMOS Multi-Electrode Array Chip. Front. Neurosci., 13.","DOI":"10.3389\/fnins.2019.00641"},{"key":"ref_160","doi-asserted-by":"crossref","unstructured":"Kussauer, S., David, R., and Lemcke, H. (2019). hiPSCs Derived Cardiac Cells for Drug and Toxicity Screening and Disease Modeling: What Micro- Electrode-Array Analyses Can Tell Us. Cells, 8.","DOI":"10.3390\/cells8111331"},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"4267","DOI":"10.1016\/j.biomaterials.2010.12.022","article-title":"Patterned Cardiomyocytes on Microelectrode Arrays as a Functional, High Information Content Drug Screening Platform","volume":"32","author":"Natarajan","year":"2011","journal-title":"Biomaterials"},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.yjmcc.2019.09.007","article-title":"Cardiotoxicity Screening of Illicit Drugs and New Psychoactive Substances (NPS) in Human iPSC-Derived Cardiomyocytes Using Microelectrode Array (MEA) Recordings","volume":"136","author":"Zwartsen","year":"2019","journal-title":"J. Mol. Cell. Cardiol."},{"key":"ref_163","doi-asserted-by":"crossref","unstructured":"(2023, September 11). Detection of Drug-Induced Torsades de Pointes Arrhythmia Mechanisms Using hiPSC-CM Syncytial Monolayers in a High-Throughput Screening Voltage Sensitive Dye Assay | Toxicological Sciences | Oxford Academic. Available online: https:\/\/academic.oup.com\/toxsci\/article\/173\/2\/402\/5640507?login=false.","DOI":"10.1093\/toxsci\/kfz235"},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1093\/toxsci\/kfy274","article-title":"Arrhythmogenicity Test Based on a Human-Induced Pluripotent Stem Cell (iPSC)-Derived Cardiomyocyte Layer","volume":"168","author":"Slotvitsky","year":"2019","journal-title":"Toxicol. Sci."},{"key":"ref_165","doi-asserted-by":"crossref","unstructured":"Juang, J.-M.J., Binda, A., Lee, S.-J., Hwang, J.-J., Chen, W.-J., Liu, Y.-B., Lin, L.-Y., Yu, C.-C., Ho, L.-T., and Huang, H.-C. (2020). GSTM3 Variant Is a Novel Genetic Modifier in Brugada Syndrome, a Disease with Risk of Sudden Cardiac Death. eBioMedicine, 57.","DOI":"10.1016\/j.ebiom.2020.102843"},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/j.yjmcc.2023.08.003","article-title":"Utilizing Human Induced Pluripotent Stem Cells to Study Atrial Arrhythmias in the Short QT Syndrome","volume":"183","author":"Shiti","year":"2023","journal-title":"J. Mol. Cell. Cardiol."},{"key":"ref_167","unstructured":"Lamore, S.D., Scott, C.W., and Peters, M.F. (2004). Cardiomyocyte Impedance Assay, Eli Lilly & Company and the National Center for Advancing Translational Sciences."},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1016\/j.bbrc.2018.03.198","article-title":"Biophysical Study of Human Induced Pluripotent Stem Cell-Derived Cardiomyocyte Structural Maturation during Long-Term Culture","volume":"499","author":"Dias","year":"2018","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"3014","DOI":"10.1016\/j.celrep.2017.09.005","article-title":"Comparative Gene Expression Analyses Reveal Distinct Molecular Signatures between Differentially Reprogrammed Cardiomyocytes","volume":"20","author":"Zhou","year":"2017","journal-title":"Cell Rep."},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"1306","DOI":"10.1016\/j.stemcr.2018.11.011","article-title":"Perspectives on the Use of Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes in Biomedical Research","volume":"11","author":"Mummery","year":"2018","journal-title":"Stem Cell Rep."},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1038\/nature09342","article-title":"Epigenetic Memory in Induced Pluripotent Stem Cells","volume":"467","author":"Kim","year":"2010","journal-title":"Nature"},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1089\/scd.2014.0533","article-title":"Immaturity of Human Stem-Cell-Derived Cardiomyocytes in Culture: Fatal Flaw or Soluble Problem?","volume":"24","author":"Veerman","year":"2015","journal-title":"Stem Cells Dev."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1096\/fj.13-228007","article-title":"Advancing Functional Engineered Cardiac Tissues toward a Preclinical Model of Human Myocardium","volume":"28","author":"Turnbull","year":"2014","journal-title":"FASEB J."},{"key":"ref_174","unstructured":"(2023, September 10). Shaheen: Human Induced Pluripotent Stem Cell-Derived...\u2014Google Scholar. Available online: https:\/\/scholar.google.com\/scholar_lookup?title=human%20induced%20pluripotent%20stem%20cell-derived%20cardiac%20cell%20sheets%20expressing%20genetically%20encoded%20voltage%20indicator%20for%20pharmacological%20and%20arrhythmia%20studies&author=N%20Shaheen&author=A%20Shiti&author=I%20Huber&author=R%20Shinnawi&author=G%20Arbel&author=A%20Gepstein&author=N%20Setter&author=I%20Goldfracht&author=A%20Gruber&author=SV%20Chorna&author=L.%20Gepstein&publication_year=2018&journal=Stem%20Cell%20Rep&volume=10&pages=1879-1894."},{"key":"ref_175","unstructured":"(2023, September 10). Personalised Organs-on-Chips: Functional Testing for Precision Medicine\u2014Lab on a Chip (RSC Publishing). Available online: https:\/\/pubs.rsc.org\/en\/content\/articlehtml\/2019\/lc\/c8lc00827b."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1016\/j.addr.2015.05.010","article-title":"Human Engineered Heart Tissue as a Model System for Drug Testing","volume":"96","author":"Eder","year":"2016","journal-title":"Adv. Drug Deliv. Rev."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"2405","DOI":"10.1093\/eurheartj\/ehz779","article-title":"Living Myocardial Slices: A Novel Multicellular Model for Cardiac Translational Research","volume":"41","author":"Perbellini","year":"2020","journal-title":"Eur. Heart J."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/25\/22\/12034\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:29:12Z","timestamp":1760113752000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/25\/22\/12034"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,8]]},"references-count":177,"journal-issue":{"issue":"22","published-online":{"date-parts":[[2024,11]]}},"alternative-id":["ijms252212034"],"URL":"https:\/\/doi.org\/10.3390\/ijms252212034","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,11,8]]}}}