Associated Papers
SPARC: a structural pathogenicity algorithm for risk classification of hERG variants.
Chatelain FC et al.
Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology, 2026
High-throughput screens identify genotype-specific therapeutics for channelopathies.
Egly CL et al.
Jci Insight, 2025
Knock-in Kcnh2 rabbit model of long QT syndrome type-2, epilepsy, and sudden death.
Singh V et al.
Journal of Translational Medicine, 2025
Molecular insights into the rescue mechanism of an HERG activator against severe LQT2 mutations.
Kumawat A et al.
Journal of Biomedical Science, 2025
Knock-in Kcnh2 Rabbit Model of Long QT Syndrome Type-2, Epilepsy, and Sudden Death.
Singh V et al.
Biorxiv : the Preprint Server for Biology, 2024
Potassium dependent structural changes in the selectivity filter of HERG potassium channels.
Lau CHY et al.
Nature Communications, 2024
Kcnh2 deletion is associated with rat embryonic development defects via destruction of KCNH2‑integrin β1 complex.
Hu S et al.
International Journal of Molecular Medicine, 2024
Differential Effects of Remdesivir and Lumacaftor on Homomeric and Heteromeric hERG Channels.
Campagna N et al.
Molecular Pharmacology, 2023
Evolutionary coupling analysis guides identification of mistrafficking-sensitive variants in cardiac K+ channels: Validation with hERG.
Zhang Y et al.
Frontiers in Pharmacology, 2022
K2P channel C-type gating involves asymmetric selectivity filter order-disorder transitions.
Lolicato M et al.
Science Advances, 2020
Selectivity filter modalities and rapid inactivation of the hERG1 channel.
Miranda WE et al.
Proceedings of the National Academy of Sciences of the United States of America, 2020
Structures Illuminate Cardiac Ion Channel Functions in Health and in Long QT Syndrome.
Brewer KR et al.
Frontiers in Pharmacology, 2020
Identification of a proton sensor that regulates conductance and open time of single hERG channels.
Wilson SL et al.
Scientific Reports, 2019
Human ether‑à‑go‑go‑related gene mutation L539fs/47‑hERG leads to cell apoptosis through the endoplasmic reticulum stress pathway.
Ma S et al.
International Journal of Molecular Medicine, 2019
Characterization of the σ-Pore in Mutant hKv1.3 Potassium Channels.
Tyutyaev P et al.
Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology, 2018
Visualizing Mutation-Specific Differences in the Trafficking-Deficient Phenotype of Kv11.1 Proteins Linked to Long QT Syndrome Type 2.
Hall AR et al.
Frontiers in Physiology, 2018
Role of mutation and pharmacologic block of human KCNH2 in vasculogenesis and fetal mortality: partial rescue by transforming growth factor-β.
Teng G et al.
Circulation. Arrhythmia and Electrophysiology, 2015
Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome.
Anderson CL et al.
Nature Communications, 2014
Altered and dynamic ion selectivity of K+ channels in cell development and excitability.
Chen H et al.
Trends in Pharmacological Sciences, 2014
Elevated heart rate triggers action potential alternans and sudden death. translational study of a homozygous KCNH2 mutation.
Schweigmann U et al.
Plos One, 2014
Pharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum.
Smith JL et al.
American Journal of Physiology. Cell Physiology, 2013
Structure-guided topographic mapping and mutagenesis to elucidate binding sites for the human ether-a-go-go-related gene 1 potassium channel (KCNH2) activator NS1643.
Durdagi S et al.
The Journal of Pharmacology and Experimental Therapeutics, 2012
hERG K(+) channels: structure, function, and clinical significance.
Vandenberg JI et al.
Physiological Reviews, 2012
Blockade of permeation by potassium but normal gating of the G628S nonconducting hERG channel mutant.
Es-Salah-Lamoureux Z et al.
Biophysical Journal, 2011
HERG1 channelopathies.
Sanguinetti MC
Pflugers Archiv : European Journal of Physiology, 2010
Extracellular K+ is a prerequisite for the function and plasma membrane stability of HERG channels.
Massaeli H et al.
Circulation Research, 2010
Voltage-gated potassium channels as therapeutic targets.
Wulff H et al.
Nature Reviews. Drug Discovery, 2009
Homozygous missense N629D hERG (KCNH2) potassium channel mutation causes developmental defects in the right ventricle and its outflow tract and embryonic lethality.
Teng GQ et al.
Circulation Research, 2008
Functional studies on three novel HCNH2 mutations in Taiwan: identification of distinct mechanisms of channel defect and dissociation between glycosylation defect and assembly defect.
Hsueh CH et al.
Biochemical and Biophysical Research Communications, 2008
Insight into the mechanism of inactivation and pH sensitivity in potassium channels from molecular dynamics simulations.
Stansfeld PJ et al.
Biochemistry, 2008
Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism.
Anderson CL et al.
Circulation, 2006
Modelling and imaging cardiac repolarization abnormalities.
Rudy Y
Journal of Internal Medicine, 2006
Inherited and acquired vulnerability to ventricular arrhythmias: cardiac Na+ and K+ channels.
Clancy CE et al.
Physiological Reviews, 2005
Biology of cardiac arrhythmias: ion channel protein trafficking.
Delisle BP et al.
Circulation Research, 2004
Prolonged repolarization and triggered activity induced by adenoviral expression of HERG N629D in cardiomyocytes derived from stem cells.
Teng G et al.
Cardiovascular Research, 2004
The incorporation of an ion channel gene mutation associated with the long QT syndrome (Q9E-hMiRP1) in a plasmid vector for site-specific arrhythmia gene therapy: in vitro and in vivo feasibility studies.
Burton DY et al.
Human Gene Therapy, 2003
Drug- and mutagenesis-induced changes in the selectivity filter of a cardiac two-pore background K+ channel.
Hajdú P et al.
Cardiovascular Research, 2003
[K(+)](o)-dependent change in conformation of the HERG1 long QT mutation N629D channel results in partial reversal of the in vitro disease phenotype.
Teng GQ et al.
Cardiovascular Research, 2003
Characterization of a novel missense mutation E637K in the pore-S6 loop of HERG in a patient with long QT syndrome.
Hayashi K et al.
Cardiovascular Research, 2002
Cellular consequences of HERG mutations in the long QT syndrome: precursors to sudden cardiac death.
Clancy CE et al.
Cardiovascular Research, 2001
Congenital long QT syndromes and Brugada syndrome: the arrhythmogenic ion channel disorders.
Nemec J et al.
Expert Opinion on Pharmacotherapy, 2001
Long QT syndrome: cellular basis and arrhythmia mechanism in LQT2.
January CT et al.
Journal of Cardiovascular Electrophysiology, 2000
Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2.
Splawski I et al.
Circulation, 2000
Novel gain-of-function mechanism in K(+) channel-related long-QT syndrome: altered gating and selectivity in the HERG1 N629D mutant.
Lees-Miller JP et al.
Circulation Research, 2000
Characterization of a novel missense mutation in the pore of HERG in a patient with long QT syndrome.
Yoshida H et al.
Journal of Cardiovascular Electrophysiology, 1999
N-linked glycosylation sites determine HERG channel surface membrane expression.
Petrecca K et al.
The Journal of Physiology, 1999
Novel KCNQ1 and HERG missense mutations in Dutch long-QT families.
Jongbloed RJ et al.
Human Mutation, 1999
Multiple different missense mutations in the pore region of HERG in patients with long QT syndrome.
Satler CA et al.
Human Genetics, 1998
Human ether-a-go-go related gene (hERG) K+ channels: function and dysfunction.
Perrin MJ et al.
Progress in Biophysics and Molecular Biology, 0