Intracellular calcium current disorder and disease phenotype in <i>OBSCN</i> mutant iPSC-based cardiomyocytes in arrhythmogenic right ventricular cardiomyopathy
Peipei Chen, Ying Xiao, Yuanpin Wang, Zhifa Zheng, Lianfeng Chen, Xufei Yang, Jingyi Li, Wei Wu, Shuyang Zhang
Abstract
caused phenotypic alteration accompanied by disrupted localization and decreased expression of its anchoring protein Ank1.5. Furthermore, there was an accumulation of lipids with an increase in fatty fibrosis area and myocardial structural disorder, possibly leading to dysrhythmia in calcium channel-related myocardial contraction. These observations suggested the possibility of attenuating ARVC progression by therapeutic modulation of OBSCN expression.
Topics & Concepts
PhenotypeCardiomyopathyIntracellularCalcium in biologyCardiologyInternal medicineMutantDiseaseCalciumMedicineClinical phenotypeCell biologyBiologyHeart failureGeneticsGeneCardiovascular Effects of ExerciseCardiac electrophysiology and arrhythmiasCardiomyopathy and Myosin Studies