Hypertrophic cardiomyopathy-linked mutation in troponin T causes myofibrillar disarray and pro-arrhythmic action potential changes in human iPSC cardiomyocytes
Wang L, Kim K, Parikh S, Cadar AG, Bersell KR, He H, Pinto JR, Kryshtal DO, Knollmann BC. Hypertrophic cardiomyopathy-linked mutation in troponin T causes myofibrillar disarray and pro-arrhythmic action potential changes in human iPSC cardiomyocytes. Journal Of Molecular And Cellular Cardiology 2017, 114: 320-327. PMID: 29217433, PMCID: PMC5800960, DOI: 10.1016/j.yjmcc.2017.12.002.Peer-Reviewed Original ResearchConceptsMyofilament Ca sensitivityHiPSC-CMsControl hiPSC-CMsCa handlingTroponin TAction potentialsAction potential triangulationPro-arrhythmic changesCa sensitivityHuman ventricular action potentialCardiac troponin TAction potential changesIntracellular Ca transientsVentricular action potentialHuman iPSC-cardiomyocytesRod-shaped cardiomyocytesCardiac action potentialDiastolic dysfunctionImpaired relaxationSystolic functionVentricular arrhythmiasHypertrophic cardiomyopathySudden deathArrhythmia riskCardiac hypertrophy