The C-terminal D85N KCNE1 polymorphism has been reported to produce a dominant negative effect to reduce IhERG (Nishio et al. 2009; Nof et al. 2011) and a recent report has highlighted a significantly higher incidence of D85N KCNE1 in patients with drug-induced LQTS than in population-based controls (Kaab et al. 2012). Here, KCNE1 is linked to familial long QT syndrome.