Action Potential Clamp and Pharmacology of the Variant 1 Short QT Syndrome T618I hERG K+ Channel

Abstract

The T618I mutation produces a more modest effect on repolarising I(hERG) than reported previously for the N588K-hERG variant 1 SQTS mutation. All drugs studied here appear substantially to retain their ability to inhibit I(hERG) in the setting of the SQTS-linked T618I mutation.