[HTML][HTML] hERG K+ channels: structure, function, and clinical significance

JI Vandenberg, MD Perry, MJ Perrin… - Physiological …, 2012 - journals.physiology.org
The human ether-a-go-go related gene (hERG) encodes the pore-forming subunit of the
rapid component of the delayed rectifier K+ channel, Kv11. 1, which are expressed in the …

Pro-arrhythmic effects of gain-of-function potassium channel mutations in the short QT syndrome

JC Hancox, CY Du, A Butler… - … of the Royal …, 2023 - royalsocietypublishing.org
The congenital short QT syndrome (SQTS) is a rare condition characterized by abbreviated
rate-corrected QT (QTc) intervals on the electrocardiogram and by increased susceptibility to …

Murine electrophysiological models of cardiac arrhythmogenesis

CLH Huang - Physiological reviews, 2017 - journals.physiology.org
Cardiac arrhythmias can follow disruption of the normal cellular electrophysiological
processes underlying excitable activity and their tissue propagation as coherent wavefronts …

Emerging therapeutic targets in the short QT syndrome

JC Hancox, DG Whittaker, C Du, AG Stuart… - Expert opinion on …, 2018 - Taylor & Francis
ABSTRACT Introduction: Short QT Syndrome (SQTS) is a rare but dangerous condition
characterised by abbreviated repolarisation, atrial and ventricular arrhythmias and risk of …

Computational approaches to understand cardiac electrophysiology and arrhythmias

BN Roberts, PC Yang, SB Behrens… - American Journal …, 2012 - journals.physiology.org
Cardiac rhythms arise from electrical activity generated by precisely timed opening and
closing of ion channels in individual cardiac myocytes. These impulses spread throughout …

Genotype-dependent differences in age of manifestation and arrhythmia complications in short QT syndrome

DT Harrell, T Ashihara, T Ishikawa, I Tominaga… - International journal of …, 2015 - Elsevier
Abstract Background Short QT syndrome (SQTS) is a rare inheritable arrhythmia, associated
with atrial and ventricular fibrillations, caused by mutations in six cardiac ion channel genes …

Evaluating pro-arrhythmogenic effects of the T634S-hERG mutation: insights from a simulation study

W Hu, W Zhang, K Zhang, E Al-Moubarak… - Interface …, 2023 - royalsocietypublishing.org
A mutation to serine of a conserved threonine (T634S) in the hERG K+ channel S6 pore
region has been identified as a variant of uncertain significance, showing a loss-of-function …

In silico investigation of the short QT syndrome, using human ventricle models incorporating electromechanical coupling

I Adeniran, JC Hancox, H Zhang - Frontiers in Physiology, 2013 - frontiersin.org
Introduction: Genetic forms of the Short QT Syndrome (SQTS) arise due to cardiac ion
channel mutations leading to accelerated ventricular repolarization, arrhythmias and sudden …

The virtual heart as a platform for screening drug cardiotoxicity

Y Yuan, X Bai, C Luo, K Wang… - British journal of …, 2015 - Wiley Online Library
To predict the safety of a drug at an early stage in its development is a major challenge as
there is a lack of in vitro heart models that correlate data from preclinical toxicity screening …

Proarrhythmia in KCNJ2-linked short QT syndrome: insights from modelling

I Adeniran, A El Harchi, JC Hancox… - Cardiovascular …, 2012 - academic.oup.com
Aims One form of the short QT syndrome (SQT3) has been linked to the D172N gain-in-
function mutation to Kir2. 1, which preferentially increases outward current through channels …