2023
Chronic diastolic stretch unmasks conduction defects in an in vitro model of arrhythmogenic cardiomyopathy
Ng R, Gokhan I, Stankey P, Akar F, Campbell S. Chronic diastolic stretch unmasks conduction defects in an in vitro model of arrhythmogenic cardiomyopathy. AJP Heart And Circulatory Physiology 2023, 325: h1373-h1385. PMID: 37830983, PMCID: PMC10977872, DOI: 10.1152/ajpheart.00709.2022.Peer-Reviewed Original ResearchArrhythmogenic cardiomyopathyDiastolic stretchConduction deficitsConduction velocityIntensity of exerciseAction potential parametersDifferent time pointsDisease progressionHigh afterloadContractile functionHemodynamic parametersProgressive disorderUnderlying pathomechanismsArrhythmia vulnerabilityConduction defectsPhysiological shorteningHeart tissueConnexin 43Time pointsERK signalingCardiomyopathyLysosome activityCardiac cycleDesmosomal proteinsExercise
2022
Humanized Dsp ACM Mouse Model Displays Stress-Induced Cardiac Electrical and Structural Phenotypes
Stevens TL, Manring HR, Wallace MJ, Argall A, Dew T, Papaioannou P, Antwi-Boasiako S, Xu X, Campbell SG, Akar FG, Borzok MA, Hund TJ, Mohler PJ, Koenig SN, El Refaey M. Humanized Dsp ACM Mouse Model Displays Stress-Induced Cardiac Electrical and Structural Phenotypes. Cells 2022, 11: 3049. PMID: 36231013, PMCID: PMC9562631, DOI: 10.3390/cells11193049.Peer-Reviewed Original ResearchConceptsArrhythmogenic cardiomyopathyMouse modelStructural phenotypesFibro-fatty infiltrationFirst mouse modelHeart failureChamber dilationVentricular arrhythmiasPressure overloadArrhythmic eventsCardiac performanceCardiac stressSudden deathCardiovascular stressInherited disorderG variantConnexin 43MiceDesmosomal genesReduced expressionExternal stressorsACM familyDisease developmentMurine equivalentIncomplete penetrance