Structural interplay of KV7.1 and KCNE1 is essential for normal repolarization and is compromised in short QT syndrome 2 (KV7.1-A287T)

. 2016 Sep 12;2(6):521–529. doi: 10.1016/j.hrcr.2016.08.015

KEY TEACHING POINTS

•
Kv7.1 and KCNE1 form the cardiac I_Ks channel complex. This channel current is a main component of atrial and ventricular repolarization.
•
The Kv7.1–KCNE1 structural interplay can be altered by mutations in patients to result in abnormal cardiac repolarization.
•
Aberrant function of I_Ks can lead to long QT syndrome (I_Ks loss of function) or, rarely, to short QT syndrome (I_Ks gain of function).