The voltage-dependent potassium channel Kv7.1 is a tetramer composed of Kcnq 1 a and Kcne 1 b subunits.It has been found functionally expressed in heart,inner ear,kidney and colons and mediating depolarization in the cardiac myocytes and potassium secretion into endolymph in the inner ear epithelial cells and assisting chloride homeostasis in epithelium of kidney and colons under physiological condition.Regulatory mechanisms of the channel function via adrenergic and purinergic receptors are well documented.Inherited mutations found in both kcnq1 and kcne1 gene have been directly linked to clinical phenotypes of dominant form of Long QT syndrome with hearing loss (Jervell-Lange-Neilson Syndrome) and recessive form of Long QT syndrome without hearing loss (Romano-Word Syndrome),which could be duplicated in experimental animals with gene engineering of knock-out and/or transgenic techniques.