What is the role of the LQT1 gene in the etiology of long QT syndrome (LQTS)?

Updated: Nov 29, 2017
  • Author: Ali A Sovari, MD, FACP, FACC; Chief Editor: Mikhael F El-Chami, MD  more...
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Answer

The LQT1 gene (KVLQT1 or KCNQ1) encodes for part of the IKs slowly deactivating, delayed rectifier potassium channel. [6] More than 170 mutations (most missense) of this gene have been reported. Their net effect is a decreased outward potassium current. Therefore, the channels remain open longer than usual, with a delay in ventricular repolarization and with QT prolongation.


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