{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Josvai MB"],"funding":["NHLBI NIH HHS","National Institutes of Health","National Heart Lung and Blood Institute Division of Intramural Research"],"pagination":["2983-2994"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC12554002"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["22(11)"],"pubmed_abstract":["Human induced pluripotent stem cells (iPSCs) have emerged as a transformative platform for modeling inherited cardiac arrhythmia syndromes and uncovering human-specific disease mechanisms. However, the promise of iPSC-derived cardiomyocytes lies beyond the recapitulation of arrhythmogenic phenotypes and channelopathies. In this review, we explore recent works which have enabled mechanistic interrogation and therapeutic insight for inherited arrhythmia syndromes, beyond the capabilities of traditional animal models. Such studies have leveraged iPSCs to elucidate the role of splice variants, transcriptional regulation, and mitochondrial stress in arrhythmogenesis. Further, iPSC systems have proven important for reclassifying variants of uncertain significance and in modeling idiopathic arrhy"],"journal":["Heart rhythm"],"pubmed_title":["The role of iPSC research for insight into inherited arrhythmia conditions."],"pmcid":["PMC12554002"],"funding_grant_id":["T32 HL007936","R01 HL170521","R01 HL163987","T32 HL00793621"],"pubmed_authors":["Eckhardt LL","Josvai MB","Anderson CL"],"additional_accession":[]},"is_claimable":false,"name":"The role of iPSC research for insight into inherited arrhythmia conditions.","description":"Human induced pluripotent stem cells (iPSCs) have emerged as a transformative platform for modeling inherited cardiac arrhythmia syndromes and uncovering human-specific disease mechanisms. However, the promise of iPSC-derived cardiomyocytes lies beyond the recapitulation of arrhythmogenic phenotypes and channelopathies. In this review, we explore recent works which have enabled mechanistic interrogation and therapeutic insight for inherited arrhythmia syndromes, beyond the capabilities of traditional animal models. Such studies have leveraged iPSCs to elucidate the role of splice variants, transcriptional regulation, and mitochondrial stress in arrhythmogenesis. Further, iPSC systems have proven important for reclassifying variants of uncertain significance and in modeling idiopathic arrhy","dates":{"release":"2025-01-01T00:00:00Z","publication":"2025 Nov","modification":"2026-06-10T05:20:01.162Z","creation":"2026-06-10T03:07:12.973Z"},"accession":"S-EPMC12554002","cross_references":{"pubmed":["40456302"],"doi":["10.1016/j.hrthm.2025.03.2005"]}}