Variant c.238G > C (p.Val80Leu) of the KCNE1 gene has been associated with long QT syndrome.[12,13] KCNE1 regulates the potassium voltage-gated channel subfamily Q member 1 (KCNQ1) channel in a variety of ways[14]: by slowing voltage-stimulated channel activation, increasing conductance, and eliminating channel inactivation.[15] KCNQ1, a voltage-gated potassium channel composed of 676 amino acids, is essential for cardiac action potential repolarization. The gene discussed is KCNE1; the disease is Prolonged QT interval.