“…Disopyramide was subsequently tested in a pilot study on two SQT1 patients and was found to exert beneficial effects on QT interval, rate dependence and ventricular ERP [89]. Moreover, disopyramide has also been reported to be beneficial in a patient with SQTS of unknown genotype [90]. By comparing the potency of a series of agents against WT-hERG, N588K-hERG, another attenuated-inactivation mutant S631A and a N588K-S631A double mutant, we recently demonstrated a strong correlation between the extent of attenuation of inactivation and the reduction in drug inhibitory potency [91], with an independent study providing evidence that Note that symbols do not denote plotted data-points; rather the concentration-response relations have been plotted as continuous lines, and some line types incorporate symbols to help differentiate between the different plots.…”