Ontology highlight
ABSTRACT:
SUBMITTER: Lee J
PROVIDER: S-EPMC6779479 | biostudies-literature | 2019 Aug
REPOSITORIES: biostudies-literature
Nature 20190717 7769
Lamin A/C (LMNA) is one of the most frequently mutated genes associated with dilated cardiomyopathy (DCM). DCM related to mutations in LMNA is a common inherited cardiomyopathy that is associated with systolic dysfunction and cardiac arrhythmias. Here we modelled the LMNA-related DCM in vitro using patient-specific induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs). Electrophysiological studies showed that the mutant iPSC-CMs displayed aberrant calcium homeostasis that led to arrhyt ...[more]