Department of Physiology and Biophysics, Miller School of Medicine, University of Miami, Miami, FL 33136, USA.
Int J Mol Sci. 2020 Dec 11;21(24):9440. doi: 10.3390/ijms21249440.
The delayed rectifier potassium IKs channel is an important regulator of the duration of the ventricular action potential. Hundreds of mutations in the genes ( and ) encoding the IKs channel cause long QT syndrome (LQTS). LQTS is a heart disorder that can lead to severe cardiac arrhythmias and sudden cardiac death. A better understanding of the IKs channel (here called the KCNQ1/KCNE1 channel) properties and activities is of great importance to find the causes of LQTS and thus potentially treat LQTS. The KCNQ1/KCNE1 channel belongs to the superfamily of voltage-gated potassium channels. The KCNQ1/KCNE1 channel consists of both the pore-forming subunit KCNQ1 and the modulatory subunit KCNE1. KCNE1 regulates the function of the KCNQ1 channel in several ways. This review aims to describe the current structural and functional knowledge about the cardiac KCNQ1/KCNE1 channel. In addition, we focus on the modulation of the KCNQ1/KCNE1 channel and its potential as a target therapeutic of LQTS.
延迟整流钾通道 IKs 是心室动作电位时程的重要调节因子。编码 IKs 通道的基因（ 和 ）中有数百种突变会导致长 QT 综合征（LQTS）。LQTS 是一种心脏疾病，可导致严重的心律失常和心脏性猝死。更好地了解 IKs 通道（此处称为 KCNQ1/KCNE1 通道）的特性和活动对于寻找 LQTS 的原因并潜在地治疗 LQTS 非常重要。KCNQ1/KCNE1 通道属于电压门控钾通道超家族。KCNQ1/KCNE1 通道由形成孔的亚基 KCNQ1 和调节亚基 KCNE1 组成。KCNE1 通过多种方式调节 KCNQ1 通道的功能。本综述旨在描述目前关于心脏 KCNQ1/KCNE1 通道的结构和功能知识。此外，我们还重点介绍了 KCNQ1/KCNE1 通道的调节及其作为 LQTS 治疗靶点的潜力。
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