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 …

Gating and regulation of KCNQ1 and KCNQ1+ KCNE1 channel complexes

Y Wang, J Eldstrom, D Fedida - Frontiers in Physiology, 2020 - frontiersin.org
The IKs channel complex is formed by the co-assembly of Kv7. 1 (KCNQ1), a voltage-gated
potassium channel, with its β-subunit, KCNE1 and the association of numerous accessory …

Inactivation of KCNQ1 potassium channels reveals dynamic coupling between voltage sensing and pore opening

P Hou, J Eldstrom, J Shi, L Zhong, K McFarland… - Nature …, 2017 - nature.com
In voltage-activated ion channels, voltage sensor (VSD) activation induces pore opening via
VSD-pore coupling. Previous studies show that the pore in KCNQ1 channels opens when …

A new KCNQ1 mutation at the S5 segment that impairs its association with KCNE1 is responsible for short QT syndrome

C Moreno, A Oliveras, A De La Cruz… - Cardiovascular …, 2015 - academic.oup.com
Aims KCNQ1 and KCNE1 encode Kv7. 1 and KCNE1, respectively, the pore-forming and the
accessory subunits of the slow delayed rectifier potassium current, I Ks. KCNQ1 mutations …

ML277 specifically enhances the fully activated open state of KCNQ1 by modulating VSD-pore coupling

P Hou, J Shi, KMF White, Y Gao, J Cui - Elife, 2019 - elifesciences.org
Upon membrane depolarization, the KCNQ1 potassium channel opens at the intermediate
(IO) and activated (AO) states of the stepwise voltage-sensing domain (VSD) activation. In …

Mechanisms underlying the dual effect of polyunsaturated fatty acid analogs on Kv7. 1

SI Liin, S Yazdi, R Ramentol, R Barro-Soria… - Cell reports, 2018 - cell.com
Polyunsaturated fatty acid (PUFA) analogs represent a new class of potential anti-arrhythmic
KV 7.1 and KV 7.1+ KCNE1 channel activators. In this study, we describe dual independent …

Molecular basis of decreased Kir4. 1 function in SeSAME/EAST syndrome

DM Williams, CMB Lopes… - Journal of the …, 2010 - journals.lww.com
SeSAME/EAST syndrome is a channelopathy consisting of a hypokalemic,
hypomagnesemic, metabolic alkalosis associated with seizures, sensorineural deafness …

ML277 regulates KCNQ1 single-channel amplitudes and kinetics, modified by voltage sensor state

J Eldstrom, DA McAfee, Y Dou, Y Wang… - Journal of General …, 2021 - rupress.org
KCNQ1 is a pore-forming K+ channel subunit critically important to cardiac repolarization at
high heart rates.(2R)-N-[4-(4-methoxyphenyl)-2-thiazolyl]-1-[(4-methylphenyl) sulfonyl]-2 …

A benzodiazepine activator locks Kv7.1 channels open by electro-mechanical uncoupling

JA Schreiber, M Möller, M Zaydman, L Zhao… - Communications …, 2022 - nature.com
Loss-of-function mutations in Kv7. 1 often lead to long QT syndrome (LQTS), a cardiac
repolarization disorder associated with arrhythmia and subsequent sudden cardiac death …

The S4-S5 linker of KCNQ1 channels forms a structural scaffold with the S6 segment controlling gate closure

AJ Labro, IR Boulet, FS Choveau, E Mayeur… - Journal of biological …, 2011 - ASBMB
In vivo, KCNQ1 α-subunits associate with the β-subunit KCNE1 to generate the slowly
activating cardiac potassium current (I Ks). Structurally, they share their topology with other …