Molecular pathophysiology of congenital long QT syndrome

MS Bohnen, G Peng, SH Robey… - Physiological …, 2017 - journals.physiology.org
Ion channels represent the molecular entities that give rise to the cardiac action potential,
the fundamental cellular electrical event in the heart. The concerted function of these …

Ancillary Subunits Associated With Voltage-Dependent K+ Channels

O Pongs, JR Schwarz - Physiological reviews, 2010 - journals.physiology.org
Since the first discovery of Kvβ-subunits more than 15 years ago, many more ancillary Kv
channel subunits were characterized, for example, KChIPs, KCNEs, and BKβ-subunits. The …

Stoichiometry of the KCNQ1-KCNE1 ion channel complex

K Nakajo, MH Ulbrich, Y Kubo… - Proceedings of the …, 2010 - National Acad Sciences
The KCNQ1 voltage-gated potassium channel and its auxiliary subunit KCNE1 play a crucial
role in the regulation of the heartbeat. The stoichiometry of KCNQ1 and KCNE1 complex has …

KCNQ-encoded potassium channels as therapeutic targets

V Barrese, JB Stott, IA Greenwood - Annual review of …, 2018 - annualreviews.org
Kv7 channels are voltage-gated potassium channels encoded by KCNQ genes that have a
considerable physiological impact in many cell types. This reliance upon Kv7 channels for …

Structure, function, and modification of the voltage sensor in voltage-gated ion channels

SI Börjesson, F Elinder - Cell biochemistry and biophysics, 2008 - Springer
Voltage-gated ion channels are crucial for both neuronal and cardiac excitability. Decades
of research have begun to unravel the intriguing machinery behind voltage sensitivity …

Polyunsaturated fatty acid analogs act antiarrhythmically on the cardiac IKs channel

SI Liin, M Silverå Ejneby… - Proceedings of the …, 2015 - National Acad Sciences
Polyunsaturated fatty acids (PUFAs) affect cardiac excitability. Kv7. 1 and the β-subunit
KCNE1 form the cardiac IKs channel that is central for cardiac repolarization. In this study …

Domain–domain interactions determine the gating, permeation, pharmacology, and subunit modulation of the IKs ion channel

MA Zaydman, MA Kasimova, K McFarland, Z Beller… - Elife, 2014 - elifesciences.org
Voltage-gated ion channels generate electrical currents that control muscle contraction,
encode neuronal information, and trigger hormonal release. Tissue-specific expression of …

Voltage-dependent gating: novel insights from KCNQ1 channels

J Cui - Biophysical journal, 2016 - cell.com
Gating of voltage-dependent cation channels involves three general molecular processes:
voltage sensor activation, sensor-pore coupling, and pore opening. KCNQ1 is a voltage …

The KCNQ1 channel–remarkable flexibility in gating allows for functional versatility

SI Liin, R Barro‐Soria, HP Larsson - The Journal of physiology, 2015 - Wiley Online Library
The KCNQ1 channel (also called Kv7. 1 or KvLQT1) belongs to the superfamily of voltage‐
gated K+ (Kv) channels. KCNQ1 shares several general features with other Kv channels but …

Location of KCNE1 relative to KCNQ1 in the IKS potassium channel by disulfide cross-linking of substituted cysteines

DY Chung, PJ Chan, JR Bankston… - Proceedings of the …, 2009 - National Acad Sciences
The cardiac-delayed rectifier K+ current (IKS) is carried by a complex of KCNQ1 (Q1)
subunits, containing the voltage-sensor domains and the pore, and auxiliary KCNE1 (E1) …