Abstract: Objective: To establish the induced pluripotent stem cells (iPSCs) from patients with arrhythmogenic right ventricular cardiomyopathy (ARVC).Methods: The fibroblasts were isolated from ARVC patient and DNA mutation sites were confirmed. We reprogrammed the patient fibroblasts to pluripotency by sendaiviral transduction with defined pluripotency factors. Then, we evaluated the pluripotency of ARVC-iPSCs by performing the immunofluorescence analysis, real-time PCR and 3 germ layer formation assay. We also induced the ARVC-iPSCs into cardiac specific differentiation by regulating Wnt signaling pathway.Results: Apart from alkaline phosphatase positive staining, iPSCs derived from ARVC patients expressed pluripotent related genes and embryonic stem cell marker OCT4, SSEA4 and TRA-1-81. Embryonic body formation in vitro and teratoma test in vivo demonstrated that ARVC-iPSCs could differentiate into all three germ layers. After in vitro cardiac differentiation, ARVC-iPSC could be successfully induced to spontaneously beating mass with cTNT staining positive.Conclusion: Induced pluripotent stem cell was successfully established by integration free sendai virus from dermal fibroblast of patients with ARVC. It would provide the valuable experimental model to study the molecular mechanism of ARVC.

Key words: arrhythmogenic right ventricular cardiomyopathy, induced pluripotent stem cells, cardiac differentiation