The EAG Voltage-Dependent K+ Channel Subfamily: Similarities and Differences in Structural Organization and Gating

F Barros, P de la Peña, P Domínguez… - Frontiers in …, 2020 - frontiersin.org
EAG (ether-à-go-go or KCNH) are a subfamily of the voltage-gated potassium (Kv) channels.
Like for all potassium channels, opening of EAG channels drives the membrane potential …

New structures and gating of voltage-dependent potassium (Kv) channels and their relatives: a multi-domain and dynamic question

F Barros, LA Pardo, P Domínguez, LM Sierra… - International Journal of …, 2019 - mdpi.com
Voltage-dependent potassium channels (Kv channels) are crucial regulators of cell
excitability that participate in a range of physiological and pathophysiological processes …

Large-scale mutational analysis of Kv11. 1 reveals molecular insights into type 2 long QT syndrome

CL Anderson, CE Kuzmicki, RR Childs, CJ Hintz… - Nature …, 2014 - nature.com
It has been suggested that deficient protein trafficking to the cell membrane is the dominant
mechanism associated with type 2 Long QT syndrome (LQT2) caused by Kv11. 1 potassium …

The structural mechanism of KCNH-channel regulation by the eag domain

Y Haitin, AE Carlson, WN Zagotta - Nature, 2013 - nature.com
The KCNH voltage-dependent potassium channels (ether-à-go-go, EAG; EAG-related gene,
ERG; EAG-like channels, ELK) are important regulators of cellular excitability,, and have key …

The ERG1 K+ Channel and Its Role in Neuronal Health and Disease

FG Sanchez-Conde, EN Jimenez-Vazquez… - Frontiers in Molecular …, 2022 - frontiersin.org
The ERG1 potassium channel, encoded by KCNH2, has long been associated with cardiac
electrical excitability. Yet, a growing body of work suggests that ERG1 mediates physiology …

Structure of the C-terminal region of an ERG channel and functional implications

TI Brelidze, EC Gianulis, F DiMaio… - Proceedings of the …, 2013 - National Acad Sciences
The human ether-à-go-go–related gene (hERG) encodes a K+ channel crucial for
repolarization of the cardiac action potential. EAG-related gene (ERG) channels contain a C …

An intracellular hydrophobic nexus critical for hERG1 channel slow deactivation

WA Stevens-Sostre, L Flores-Aldama, D Bustos, J Li… - Biophysical …, 2024 - cell.com
Slow deactivation is a critical property of voltage-gated K+ channels encoded by the human
Ether-à-go-go-Related Gene 1 (hERG). hERG1 channel deactivation is modulated by …

Electrophysiological characterization of the hERG R56Q LQTS variant and targeted rescue by the activator RPR260243

JM Kemp, DG Whittaker, R Venkateshappa… - Journal of General …, 2021 - rupress.org
Human Ether-à-go-go (hERG) channels contribute to cardiac repolarization, and inherited
variants or drug block are associated with long QT syndrome type 2 (LQTS2) and …

Multiple interactions between cytoplasmic domains regulate slow deactivation of Kv11. 1 channels

CA Ng, K Phan, AP Hill, JI Vandenberg… - Journal of Biological …, 2014 - ASBMB
The intracellular domains of many ion channels are important for fine-tuning their gating
kinetics. In Kv11. 1 channels, the slow kinetics of channel deactivation, which are critical for …

Modulation of hERG K+ Channel Deactivation by Voltage Sensor Relaxation

YP Shi, S Thouta, TW Claydon - Frontiers in pharmacology, 2020 - frontiersin.org
The hERG (human-ether-à-go-go-related gene) channel underlies the rapid delayed rectifier
current, Ikr, in the heart, which is essential for normal cardiac electrical activity and rhythm …