2022
Muscle LIM Protein Force-Sensing Mediates Sarcomeric Biomechanical Signaling in Human Familial Hypertrophic Cardiomyopathy
Riaz M, Park J, Sewanan LR, Ren Y, Schwan J, Das SK, Pomianowski PT, Huang Y, Ellis MW, Luo J, Liu J, Song L, Chen IP, Qiu C, Yazawa M, Tellides G, Hwa J, Young LH, Yang L, Marboe CC, Jacoby DL, Campbell SG, Qyang Y. Muscle LIM Protein Force-Sensing Mediates Sarcomeric Biomechanical Signaling in Human Familial Hypertrophic Cardiomyopathy. Circulation 2022, 145: 1238-1253. PMID: 35384713, PMCID: PMC9109819, DOI: 10.1161/circulationaha.121.056265.Peer-Reviewed Original ResearchConceptsHypertrophic cardiomyopathySarcomeric mutationsFamilial hypertrophic cardiomyopathySudden cardiac deathCardiac myosin heavy chainMechanism-based treatmentsDevelopment of hypertrophyActivated T cellsCalcineurin-nuclear factorForce productionPhenotypic expressionPluripotent stem cell-derived cardiomyocytesStem cell-derived cardiomyocytesHeart failureCardiac deathVentricular hypertrophyCell-derived cardiomyocytesCardiac contractilityPharmacological interventionsT cellsCardiac diseaseCardiac hypertrophyPatient-specific induced pluripotent stem cellsPharmacological meansTwitch relaxation
2019
Extracellular Matrix From Hypertrophic Myocardium Provokes Impaired Twitch Dynamics in Healthy Cardiomyocytes
Sewanan LR, Schwan J, Kluger J, Park J, Jacoby DL, Qyang Y, Campbell SG. Extracellular Matrix From Hypertrophic Myocardium Provokes Impaired Twitch Dynamics in Healthy Cardiomyocytes. JACC Basic To Translational Science 2019, 4: 495-505. PMID: 31468004, PMCID: PMC6712054, DOI: 10.1016/j.jacbts.2019.03.004.Peer-Reviewed Original ResearchHypertrophic cardiomyopathySarcomeric gene mutationsMiniature swine modelPluripotent stem cell-derived cardiomyocytesExtracellular matrix abnormalitiesStem cell-derived cardiomyocytesPoor relaxationCell-derived cardiomyocytesHealthy cardiomyocytesExtracellular matrixMouse modelPharmacological correctionProlonged contractionSwine modelHealthy extracellular matrixMuscle contractionGene mutationsMatrix abnormalitiesTwitch dynamicsCardiomyocytesContractionCardiomyopathyTherapyMyocardiumAbnormalities