KCNQ1 and long QT syndrome 1: Because of the important role of the assembly of KCNE1 subunits in the response of KCNQ1/KCNE1 channels to these pharmacological agents, we sought to determine whether these compounds might have distinct potential in rescuing the activity of KCNQ1 channels assembled with KCNE1 subunits harboring specific LQT-1 and LQT-5 mutations that have previously been shown to affect the IKS complex assembly and thus altering modulation of KCNE1-bound channels: KCNE1-L51H (Bianchi et al., 1999), KCNE1-G52R (Ma et al., 2003) and KCNQ1 S546L (Dvir et al., 2014).