INS and long QT syndrome 1: ,6,7,8 While inherited mutations of KCNQ1 are primarily associated with cardiovascular pathologies (e.g., long QT syndrome 1 (LQT1), familial atrial fibrillation)4,9 and hearing loss,10 there are several lines of evidence suggesting that KCNQ1 is also involved in the regulation of insulin secretion.11