Within the context of other channelopathies, pore helix missense variants in many voltage-gated channels are associated with arrhythmogenic Long QT syndrome 1 (Kv11.1, KCNH2 gene); Long QT syndrome 2 (Kv 7.1, KCNQ1 gene); Brugada syndrome (NaV1.5, SCN5A gene) several types of epilepsy (NaV1.1, SCN1A gene; NaV1.3, SCN3A gene); and other conditions (27, , , –31). The gene discussed is KCNQ1; the disease is channelopathy.