Can KCNE1 influence the heart’s electrical activity in long-QT syndrome?

New pathogenic mechanisms in the long QT syndrome: KCNE1 modulation of hERG

Professor Jules Hancox and colleagues at the University of Bristol will investigate how a small protein called KCNE1 can influence the electrical activity of the heart. The KCNE1 protein interacts with potassium ion channels called hERGs which are heart cell wall proteins that allow positively charged potassium atoms (potassium ions) to pass into and out of the cell. Mutation of the hERG channel is thought to give rise to a genetic form of a condition called long-QT Syndrome (LQTS), which can lead to sudden death. This research will see whether variations of the KCNE1 protein can alter hERG electrical activity and sensitivity to drugs associated with LQTS. LQTS is the most common inherited heart rhythm disturbance. It occurs in about 1 in 2,000 people. LQTS may be genetically inherited, but it can also be caused by certain medications taken for other medical conditions. There are two types of LQTS. In most cases, the condition delays the flow of potassium ions out of heart muscle cells. However, for a small number of people, the sodium channels are affected and too many sodium ions are allowed into the cells. This causes a disturbance in the heart rhythm which can result in blackout and palpitations. This group’s research will help inform the development of drugs that target the hERG channel to help treat long QT syndrome.