Human induce pluripotent stem cell-derived cardiomyocyte model to study PUFA analogs as a potential treatment of long QT syndrome

Abstract

Long QT syndrome (LQTS) is an inheritable ventricular arrhythmia that predisposes patients to torsades de pointes and sudden cardiac death. Mutations in voltage-gated ion channels and their regulatory β subunits involved in the cardiac action potential (AP) generate LQTS by prolonging the QT interval of the electrocardiogram. 15 types of LQTS (LQT1-15) have been described depending on the underlying gene mutated. The majority of the cases correlate to LQT1-2 which are mutations in voltage-gated potassium channels involved in cardiac AP repolarization.