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Connexin-43 Restoration Alleviates Desmosomal Arrhythmogenic Cardiomyopathy

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Zhang, Jing, Zanella, Fabian, Ellis, Matthew W., Bradford, William H., Gutierrez-Lara, Erika Joana, Wang, Tsui-Min, Fujita, Kyohei, Duron, Charlize +11 more·2026·Circulation: Heart Failure
DOI 10.1161/circheartfailure.125.013801PMC PMC12885159
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Abstract

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is a fatal genetic heart disease primarily caused by mutations in desmosomal genes, leading to impaired cell-cell adhesion, ventricular arrhythmias, and progressive heart failure. Although gene therapy for specific ACM populations shows promise, it remains unclear whether mutation-agnostic pathways dysregulated across desmosomal mutations could be exploited for therapeutic intervention in this genetically broad and severe population. The reduction in expression of the ventricular gap junction protein Cx43 (connexin-43) is a common molecular alteration underlying desmosomal junctional deficits and arrhythmias, suggesting a potential common underlying mechanism and a therapeutic target for ACM. We hypothesized that restoration of Cx43 expression could be a mutation-agnostic intervention for ACM. METHODS: We exploited adeno-associated-viral-mediated gene therapy to restore the gap junction protein, Cx43, in genetic mouse models and human stem cell models of ACM, harboring loss or mutations in desmosomal genes, including Dsp (desmoplakin), PKP2 (plakophilin-2), and DSG2 (desmoglein-2). RESULTS: Administration of AAV-Cx43 (adeno-associated-viral-mediated connexin-43) gene therapy alleviated the severe biventricular dilatation, contractile dysfunction, and arrhythmias, while prolonging lifespan in 2 severe desmosomal ACM mouse models, either harboring Dsp loss and a prevalent human PKP2 mutation. Viral-mediated restoration of Cx43 could also alleviate physiological deficits in ACM human induced pluripotent stem cell-derived cardiomyocytes harboring PKP2 and DSG2 mutations. Mechanistically, Cx43 targets desmosomal protein expression and relocalization to the cell junction to support their mechanical stabilization and coupling. CONCLUSIONS: By using mouse and human models of desmosomal ACM harboring different mutational backgrounds, we show the sufficiency of Cx43 gene therapy and its restoration to modify and alleviate ACM deficits. These data suggest that noncanonical functions of Cx43, including mechanical modulation and reassembly of the desmosome, are a therapeutic target with the potential to treat diverse ACM populations.

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  • Experimental approaches to enhance gap junction integrity, such as delivery of the truncated Cx43 isoform GJA1-20k, have improved electrical coupling and reduced arrhythmias in ACM models.

    “Experimental approaches to enhance gap junction integrity, such as delivery of the truncated Cx43 isoform, GJA1-20k, and other strategies, have improved electrical coupling and reduced arrhythmias in ACM models [96,121].”

    From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [121]

    The Results report that full-length Cx43 therapy reduced arrhythmias in ACM mouse models. The Discussion reports electrophysiological benefits from GJA1-20k delivery in an earlier study, but says this paper's own GJA1-20k experiment showed no physiological rescue in human ACM cells. The cited paper therefore supports benefits from Cx43 restoration, not an unqualified claim that its GJA1-20k delivery improved electrical coupling and reduced arrhythmias. (LLM, conf=0.87)

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  1. [1]Sen-Chowdhry S, Morgan RD, Chambers JC, McKenna WJ. Arrhythmogenic cardiomyopathy: etiology, diagnosis, and treatment. Annu Rev Med. 2010; 61: 233– 253. doi: 10.1146/annurev.med.052208.130419 20059337 · DOI
  2. [2]Thiene G, Corrado D, Basso C. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Orphanet J Rare Dis. 2007; 2: 45. doi: 10.1186/1750-1172-2-45 18001465 PMC2222049 · DOI
  3. [3]Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MG, Daubert JP, Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010; 121: 1533– 1541. doi: 10.1161/CIRCULATIONAHA.108.840827 20172911 PMC2860804 · DOI · Paper page
  4. [4]Marcus FI, Abidov A. Arrhythmogenic right ventricular cardiomyopathy 2012: diagnostic challenges and treatment. Journal of cardiovascular electrophysiology. 2012; 23: 1149– 1153. doi: 10.1111/j.1540-8167.2012.02412.x 22909229 · DOI
  5. [5]Mast TP, James CA, Calkins H, Teske AJ, Tichnell C, Murray B, Loh P, Russell SD, Velthuis BK, Judge DP, Evaluation of Structural Progression in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. JAMA Cardiol. 2017; 2: 293– 302. doi: 10.1001/jamacardio.2016.5034 28097316 · DOI
  6. [6]Basso C, Czarnowska E, Della Barbera M, Bauce B, Beffagna G, Wlodarska EK, Pilichou K, Ramondo A, Lorenzon A, Wozniek O, Ultrastructural evidence of intercalated disc remodelling in arrhythmogenic right ventricular cardiomyopathy: an electron microscopy investigation on endomyocardial biopsies. Eur Heart J. 2006; 27: 1847– 1854. doi: 10.1093/eurheartj/ehl095 16774985 · DOI
  7. [7]Bradford WH, Zhang J, Gutierrez-Lara EJ, Liang Y, Do A, Wang TM, Nguyen L, Mataraarachchi N, Wang J, Gu Y, Plakophilin 2 gene therapy prevents and rescues arrhythmogenic right ventricular cardiomyopathy in a mouse model harboring patient genetics. Nat Cardiovasc Res. 2023; 2: 1246– 1261. doi: 10.1038/s44161-023-00370-3 39196150 PMC11357983 · DOI
  8. [8]Kyriakopoulou E, Versteeg D, de Ruiter H, Perini I, Seibertz F, Doring Y, Zentilin L, Tsui H, van Kampen SJ, Tiburcy M, Therapeutic efficacy of AAV-mediated restoration of PKP2 in arrhythmogenic cardiomyopathy. Nat Cardiovasc Res. 2023; 2: 1262– 1276. doi: 10.1038/s44161-023-00378-9 38665939 PMC11041734 · DOI
  9. [9]van Opbergen CJM, Narayanan B, Sacramento CB, Stiles KM, Mishra V, Frenk E, Ricks D, Chen G, Zhang M, Yarabe P, AAV-Mediated Delivery of Plakophilin-2a Arrests Progression of Arrhythmogenic Right Ventricular Cardiomyopathy in Murine Hearts: Preclinical Evidence Supporting Gene Therapy in Humans. Circ Genom Precis Med. 2024; 17: e004305. doi: 10.1161/CIRCGEN.123.004305 38288614 PMC10923105 · DOI
  10. [12]Lyon RC, Mezzano V, Wright AT, Pfeiffer E, Chuang J, Banares K, Castaneda A, Ouyang K, Cui L, Contu R, Connexin defects underlie arrhythmogenic right ventricular cardiomyopathy in a novel mouse model. Hum Mol Genet. 2014; 23: 1134– 1150. doi: 10.1093/hmg/ddt508 24108106 PMC3919010 · DOI
  11. [13]Liang Y, Lyon RC, Pellman J, Bradford WH, Lange S, Bogomolovas J, Dalton ND, Gu Y, Bobar M, Lee MH, Desmosomal COP9 regulates proteome degradation in arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Clin Invest. 2021; 131. doi: 10.1172/JCI137689 PMC8159691 33857019 · DOI
  12. [15]Asimaki A, Tandri H, Huang H, Halushka MK, Gautam S, Basso C, Thiene G, Tsatsopoulou A, Protonotarios N, McKenna WJ, A new diagnostic test for arrhythmogenic right ventricular cardiomyopathy. N Engl J Med. 2009; 360: 1075– 1084. doi: 10.1056/NEJMoa0808138 19279339 · DOI · Paper page
  13. [16]Zhang X, Guo L, Zeng H, White SL, Furniss M, Balasubramanian B, Lis E, Lagrutta A, Sannajust F, Zhao LL, Multi-parametric assessment of cardiomyocyte excitation-contraction coupling using impedance and field potential recording: A tool for cardiac safety assessment. Journal of pharmacological and toxicological methods. 2016; 81: 201– 216. doi: 10.1016/j.vascn.2016.06.004 27282640 · DOI
  14. [17]Prasad KM, Xu Y, Yang Z, Acton ST, French BA. Robust cardiomyocyte-specific gene expression following systemic injection of AAV: in vivo gene delivery follows a Poisson distribution. Gene therapy. 2011; 18: 43– 52. doi: 10.1038/gt.2010.105 20703310 PMC2988989 · DOI
  15. [18]Burridge PW, Matsa E, Shukla P, Lin ZC, Churko JM, Ebert AD, Lan F, Diecke S, Huber B, Mordwinkin NM, Chemically defined generation of human cardiomyocytes. Nature methods. 2014; 11: 855– 860. doi: 10.1038/nmeth.2999 24930130 PMC4169698 · DOI · Paper page
  16. [19]Lian X, Zhang J, Azarin SM, Zhu K, Hazeltine LB, Bao X, Hsiao C, Kamp TJ, Palecek SP. Directed cardiomyocyte differentiation from human pluripotent stem cells by modulating Wnt/beta-catenin signaling under fully defined conditions. Nature protocols. 2013; 8: 162– 175. doi: 10.1038/nprot.2012.150 23257984 PMC3612968 · DOI · Paper page
  17. [20]Chen G, Gulbranson DR, Hou Z, Bolin JM, Ruotti V, Probasco MD, Smuga-Otto K, Howden SE, Diol NR, Propson NE, Chemically defined conditions for human iPSC derivation and culture. Nature methods. 2011; 8: 424– 429. doi: 10.1038/nmeth.1593 21478862 PMC3084903 · DOI · Paper page
  18. [21]Zanella F, Sheikh F. Patient-Specific Induced Pluripotent Stem Cell Models: Generation and Characterization of Cardiac Cells. Methods Mol Biol. 2016; 1353: 147– 162. doi: 10.1007/7651_2014_172 25520292 PMC5357616 · DOI
  19. [22]Percie du Sert N, Hurst V, Ahluwalia A, Alam S, Avey MT, Baker M, Browne WJ, Clark A, Cuthill IC, Dirnagl U, The ARRIVE guidelines 2.0: updated guidelines for reporting animal research. BMJ Open Sci. 2020; 4: e100115. doi: 10.1136/bmjos-2020-100115 PMC7610906 34095516 · DOI
  20. [23]Denis A, Sacher F, Derval N, Martin R, Lim HS, Pambrun T, Massoullie G, Duchateau J, Cochet H, Pillois X, Arrhythmogenic response to isoproterenol testing vs. exercise testing in arrhythmogenic right ventricular cardiomyopathy patients. Europace. 2018; 20: f30– f36. doi: 10.1093/europace/euy007 29401235 · DOI
  21. [24]Dalal D, Molin LH, Piccini J, Tichnell C, James C, Bomma C, Prakasa K, Towbin JA, Marcus FI, Spevak PJ, Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in plakophilin-2. Circulation. 2006; 113: 1641– 1649. doi: 10.1161/CIRCULATIONAHA.105.568642 16549640 · DOI
  22. [25]den Haan AD, Tan BY, Zikusoka MN, Llado LI, Jain R, Daly A, Tichnell C, James C, Amat-Alarcon N, Abraham T, Comprehensive desmosome mutation analysis in north americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation Cardiovascular genetics. 2009; 2: 428– 435. doi: 10.1161/CIRCGENETICS.109.858217 20031617 PMC2801867 · DOI
  23. [26]Hamidi H, Lilja J, Ivaska J. Using xCELLigence RTCA Instrument to Measure Cell Adhesion. Bio Protoc. 2017; 7. doi: 10.21769/BioProtoc.2646 PMC5777632 29367941 · DOI
  24. [27]Xi B, Wang T, Li N, Ouyang W, Zhang W, Wu J, Xu X, Wang X, Abassi YA. Functional cardiotoxicity profiling and screening using the xCELLigence RTCA Cardio System. J Lab Autom. 2011; 16: 415– 421. doi: 10.1016/j.jala.2011.09.002 22093298 · DOI
  25. [29]Almenar-Queralt A, Merkurjev D, Kim HS, Navarro M, Ma Q, Chaves RS, Allegue C, Driscoll SP, Chen AG, Kohlnhofer B, Chromatin establishes an immature version of neuronal protocadherin selection during the naive-to-primed conversion of pluripotent stem cells. Nat Genet. 2019; 51: 1691– 1701. doi: 10.1038/s41588-019-0526-4 31740836 PMC7061033 · DOI
  26. [30]Kotini M, Barriga EH, Leslie J, Gentzel M, Rauschenberger V, Schambony A, Mayor R. Gap junction protein Connexin-43 is a direct transcriptional regulator of N-cadherin in vivo. Nat Commun. 2018; 9: 3846. doi: 10.1038/s41467-018-06368-x 30242148 PMC6155008 · DOI
  27. [31]Smyth JW, Shaw RM. Autoregulation of connexin43 gap junction formation by internally translated isoforms. Cell Rep. 2013; 5: 611– 618. doi: 10.1016/j.celrep.2013.10.009 24210816 PMC3898934 · DOI
  28. [32]Palatinus JA, Valdez S, Taylor L, Whisenant C, Selzman CH, Drakos SG, Ranjan R, Hong T, Saffitz JE, Shaw RM. GJA1-20k Rescues Cx43 Localization and Arrhythmias in Arrhythmogenic Cardiomyopathy. Circ Res. 2023; 132: 744– 746. doi: 10.1161/CIRCRESAHA.122.322294 36927183 PMC10314823 · DOI · Paper page
  29. [33]Lo CW. Role of gap junctions in cardiac conduction and development: insights from the connexin knockout mice. Circ Res. 2000; 87: 346– 348. doi: 10.1161/01.res.87.5.346 10969030 · DOI
  30. [34]Fidler LM, Wilson GJ, Liu F, Cui X, Scherer SW, Taylor GP, Hamilton RM. Abnormal connexin43 in arrhythmogenic right ventricular cardiomyopathy caused by plakophilin-2 mutations. J Cell Mol Med. 2009; 13: 4219– 4228. doi: 10.1111/j.1582-4934.2008.00438.x 18662195 PMC4496128 · DOI
  31. [35]Fontes MS, van Veen TA, de Bakker JM, van Rijen HV. Functional consequences of abnormal Cx43 expression in the heart. Biochim Biophys Acta. 2012; 1818: 2020– 2029. doi: 10.1016/j.bbamem.2011.07.039 21839722 · DOI
  32. [36]Gutstein DE, Morley GE, Tamaddon H, Vaidya D, Schneider MD, Chen J, Chien KR, Stuhlmann H, Fishman GI. Conduction slowing and sudden arrhythmic death in mice with cardiac-restricted inactivation of connexin43. Circ Res. 2001; 88: 333– 339. doi: 10.1161/01.res.88.3.333 11179202 PMC3630465 · DOI
  33. [37]Eckardt D, Theis M, Degen J, Ott T, van Rijen HV, Kirchhoff S, Kim JS, de Bakker JM, Willecke K. Functional role of connexin43 gap junction channels in adult mouse heart assessed by inducible gene deletion. J Mol Cell Cardiol. 2004; 36: 101– 110. doi: 10.1016/j.yjmcc.2003.10.006 14734052 · DOI
  34. [38]Patel DM, Dubash AD, Kreitzer G, Green KJ. Disease mutations in desmoplakin inhibit Cx43 membrane targeting mediated by desmoplakin-EB1 interactions. J Cell Biol. 2014; 206: 779– 797. doi: 10.1083/jcb.201312110 25225338 PMC4164953 · DOI
  35. [39]Wang W, James CA, Calkins H. Diagnostic and therapeutic strategies for arrhythmogenic right ventricular dysplasia/cardiomyopathy patient. Europace. 2019; 21: 9– 21. doi: 10.1093/europace/euy063 29688316 PMC6321962 · DOI
  36. [40]Oxford EM, Musa H, Maass K, Coombs W, Taffet SM, Delmar M. Connexin43 remodeling caused by inhibition of plakophilin-2 expression in cardiac cells. Circ Res. 2007; 101: 703– 711. doi: 10.1161/CIRCRESAHA.107.154252 17673670 · DOI
  37. [41]Kam CY, Dubash AD, Magistrati E, Polo S, Satchell KJF, Sheikh F, Lampe PD, Green KJ. Desmoplakin maintains gap junctions by inhibiting Ras/MAPK and lysosomal degradation of connexin-43. J Cell Biol. 2018; 217: 3219– 3235. doi: 10.1083/jcb.201710161 29959233 PMC6123000 · DOI
  38. [42]Yin T, Green KJ. Regulation of desmosome assembly and adhesion. Semin Cell Dev Biol. 2004; 15: 665– 677. doi: 10.1016/j.semcdb.2004.09.005 15561586 · DOI
  39. [43]Nekrasova O, Green KJ. Desmosome assembly and dynamics. Trends Cell Biol. 2013; 23: 537– 546. doi: 10.1016/j.tcb.2013.06.004 23891292 PMC3913269 · DOI
  40. [44]Kostetskii I, Li J, Xiong Y, Zhou R, Ferrari VA, Patel VV, Molkentin JD, Radice GL. Induced deletion of the N-cadherin gene in the heart leads to dissolution of the intercalated disc structure. Circulation research. 2005; 96: 346– 354. doi: 10.1161/01.RES.0000156274.72390.2c 15662031 · DOI
  41. [45]Shafraz O, Rubsam M, Stahley SN, Caldara AL, Kowalczyk AP, Niessen CM, Sivasankar S. E-cadherin binds to desmoglein to facilitate desmosome assembly. Elife. 2018; 7. doi: 10.7554/eLife.37629 PMC6066328 29999492 · DOI
  42. [46]Martins-Marques T, Witschas K, Ribeiro I, Zuzarte M, Catarino S, Ribeiro-Rodrigues T, Caramelo F, Aasen T, Carreira IM, Goncalves L, Cx43 can form functional channels at the nuclear envelope and modulate gene expression in cardiac cells. Open Biol. 2023; 13: 230258. doi: 10.1098/rsob.230258 37907090 PMC10645070 · DOI
  43. [47]Ai Z, Fischer A, Spray DC, Brown AM, Fishman GI. Wnt-1 regulation of connexin43 in cardiac myocytes. J Clin Invest. 2000; 105: 161– 171. doi: 10.1172/JCI7798 10642594 PMC377428 · DOI
  44. [48]Rinaldi F, Hartfield EM, Crompton LA, Badger JL, Glover CP, Kelly CM, Rosser AE, Uney JB, Caldwell MA. Cross-regulation of Connexin43 and beta-catenin influences differentiation of human neural progenitor cells. Cell Death Dis. 2014; 5: e1017. doi: 10.1038/cddis.2013.546 24457961 PMC4040652 · DOI
  45. [50]Vasquez C, Mohandas P, Louie KL, Benamer N, Bapat AC, Morley GE. Enhanced fibroblast-myocyte interactions in response to cardiac injury. Circ Res. 2010; 107: 1011– 1020. doi: 10.1161/CIRCRESAHA.110.227421 20705922 PMC2993566 · DOI
  46. [51]Roell W, Klein AM, Breitbach M, Becker TS, Parikh A, Lee J, Zimmermann K, Reining S, Gabris B, Ottersbach A, Overexpression of Cx43 in cells of the myocardial scar: Correction of post-infarct arrhythmias through heterotypic cell-cell coupling. Scientific reports. 2018; 8: 7145. doi: 10.1038/s41598-018-25147-8 29739982 PMC5940892 · DOI
  47. [52]Narmoneva DA, Vukmirovic R, Davis ME, Kamm RD, Lee RT. Endothelial cells promote cardiac myocyte survival and spatial reorganization: implications for cardiac regeneration. Circulation. 2004; 110: 962– 968. doi: 10.1161/01.CIR.0000140667.37070.07 15302801 PMC2754572 · DOI
  48. [53]Veerman CC, Kosmidis G, Mummery CL, Casini S, Verkerk AO, Bellin M. Immaturity of human stem-cell-derived cardiomyocytes in culture: fatal flaw or soluble problem? Stem Cells Dev. 2015; 24: 1035– 1052. doi: 10.1089/scd.2014.0533 25583389 · DOI
  49. [54]Bruce AF, Rothery S, Dupont E, Severs NJ. Gap junction remodelling in human heart failure is associated with increased interaction of connexin43 with ZO-1. Cardiovascular research. 2008; 77: 757– 765. doi: 10.1093/cvr/cvm083 18056766 PMC5436744 · DOI
  50. [55]Sarrouilhe D, Dejean C, Mesnil M. Connexin43- and Pannexin-Based Channels in Neuroinflammation and Cerebral Neuropathies. Frontiers in molecular neuroscience. 2017; 10: 320. doi: 10.3389/fnmol.2017.00320 29066951 PMC5641369 · DOI
  51. [56]Laird DW, Lampe PD. Therapeutic strategies targeting connexins. Nature reviews Drug discovery. 2018; 17: 905– 921. doi: 10.1038/nrd.2018.138 30310236 PMC6461534 · DOI

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Sorted by citations

Authors
Zhang, J., Zanella, F., Ellis, M. W., Bradford, W. H., Gutierrez-Lara, E. J., Wang, T.-M., Fujita, K., Duron, C., Karakikes, I., Lyon, R. C., Mezzano, V., Roberts, J. D., Carromeu, C., Gu, Y., Martin, J. L., Muotri, A. R., Scheinman, M. M., Peterson, K. L., & Sheikh, F.
Affiliations
  • University of California San Diego (US)
  • Stanford Cardiovascular Institute (US)
  • Population Health Research Institute (CA)
  • Department of Physiology and Biophysics, Center for Cardiovascular Research, University of Illinois, Chicago (J.L.M.).
  • University of California, San Francisco (US)
Corresponding author
  • Zhang, Jing
  • Zanella, Fabian
  • Ellis, Matthew W.
ORCID
Venue
Circulation: Heart Failure
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2026-04
Volume
19
Issue
4
Pages
e013801-e013801
ISSN
  • 1941-3289 (ISSN-L)
  • 1941-3297
DOI
10.1161/circheartfailure.125.013801
PMC
PMC12885159
OpenAlex
W7125734491
Cited by
5
References
4
Citations by year
2026: 5
FWCI
24.98
Type
journal article
Language
en
Open access
Locations
  • Circulation Heart Failure (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (publishedVersion)
Funders
  • National Institutes of Health (1f32hl172624-01, 1s10od023527-01)
  • National Heart, Lung, and Blood Institute (R01HL181001, R01HL162369)
  • National Cancer Institute (P30CA023100)
  • National Institute of General Medical Sciences (K12GM068524)
  • National Institute of Neurological Disorders and Stroke (P30NS047101)
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Connexins and lens biology
Keywords
arrhythmogenic cardiomyopathy, connexin 43, desmosomes, desmosomal gene mutations, gap junctions, adeno-associated virus gene therapy, desmoplakin, plakophilin-2, desmoglein 2, iPSC-derived cardiomyocytes, ventricular arrhythmias
MeSH terms
Gene Therapy Agents, Dependovirus, Animals, Desmosomes, Disease Models, Animal, Humans, Male, Mutation, Genetic Therapy, Connexin 43, Arrhythmogenic Right Ventricular Dysplasia, Myocytes, Cardiac, Desmoplakins, Plakophilins, Desmoglein 2, Mice, Induced Pluripotent Stem Cells

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