Modeling of lamin A/C mutation premature cardiac aging using patient-specific induced pluripotent stem cells

Abstract

LMNA‐related DCM was modeled in‐vitro using patient‐specific iPSC‐CMs. Our results demonstrated that haploinsufficiency due to R225X LMNA non‐sense mutation was associated with accelerated nuclear senescence and apoptosis of iPSC‐ CMs under electrical stimulation, which can be significantly attenuated by therapeutic blockade of stress‐related ERK1/2 pathway.