Abstract
Long QT syndrome (LQTS) is an inherited ventricular repolarization disorder. Patients with the QT interval prolonged might develop lethal ventricular arrhythmias such as Torsade des pointes and sudden cardiac death. There are two major currents involved in the ventricular repolarization, IKr and IKs currents. Loss-of-function mutations on any of the genes that encode the proteins involved in the generation of these two currents are directly related to LQTS. IKs channel is formed by four alpha subunits, coded by the KCNQ1 gene, co-assembled with regulatory beta subunits, encoded by the KCNE1 gene.
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