Published in Proc Natl Acad Sci U S A on August 19, 2003
S4-based voltage sensors have three major conformations. Proc Natl Acad Sci U S A (2008) 3.21
A recombinant N-terminal domain fully restores deactivation gating in N-truncated and long QT syndrome mutant hERG potassium channels. Proc Natl Acad Sci U S A (2009) 1.40
Revealing the structural basis of action of hERG potassium channel activators and blockers. J Physiol (2010) 1.37
Gating transitions in the selectivity filter region of a sodium channel are coupled to the domain IV voltage sensor. Proc Natl Acad Sci U S A (2012) 1.28
Mode shift of the voltage sensors in Shaker K+ channels is caused by energetic coupling to the pore domain. J Gen Physiol (2011) 1.28
Gating charges in the activation and inactivation processes of the HERG channel. J Gen Physiol (2004) 1.22
Conformational changes in the M2 muscarinic receptor induced by membrane voltage and agonist binding. J Physiol (2011) 1.21
Charge movement of a voltage-sensitive fluorescent protein. Biophys J (2009) 1.21
Properties of deactivation gating currents in Shaker channels. Biophys J (2011) 1.20
Mechanisms of closed-state inactivation in voltage-gated ion channels. J Physiol (2010) 1.18
Molecular basis of slow activation of the human ether-a-go-go related gene potassium channel. J Physiol (2004) 1.18
Mapping the sequence of conformational changes underlying selectivity filter gating in the K(v)11.1 potassium channel. Nat Struct Mol Biol (2010) 1.16
Role of intracellular domains in the function of the herg potassium channel. Eur Biophys J (2009) 1.15
Effect of S5P alpha-helix charge mutants on inactivation of hERG K+ channels. J Physiol (2006) 1.14
PD-118057 contacts the pore helix of hERG1 channels to attenuate inactivation and enhance K+ conductance. Proc Natl Acad Sci U S A (2009) 1.12
Tryptophan scanning mutagenesis of the HERG K+ channel: the S4 domain is loosely packed and likely to be lipid exposed. J Physiol (2005) 1.10
Mutations within the S4-S5 linker alter voltage sensor constraints in hERG K+ channels. Biophys J (2010) 1.08
Interactions between charged residues in the transmembrane segments of the voltage-sensing domain in the hERG channel. J Membr Biol (2005) 1.06
Demonstration of physical proximity between the N terminus and the S4-S5 linker of the human ether-a-go-go-related gene (hERG) potassium channel. J Biol Chem (2011) 1.05
Modification of hERG1 channel gating by Cd2+. J Gen Physiol (2010) 1.03
Molecular mechanism for depolarization-induced modulation of Kv channel closure. J Gen Physiol (2012) 1.03
Molecular determinants for activation of human ether-à-go-go-related gene 1 potassium channels by 3-nitro-n-(4-phenoxyphenyl) benzamide. Mol Pharmacol (2011) 1.02
Fluorescence-tracking of activation gating in human ERG channels reveals rapid S4 movement and slow pore opening. PLoS One (2010) 1.02
The S4-S5 linker acts as a signal integrator for HERG K+ channel activation and deactivation gating. PLoS One (2012) 0.97
Voltage-sensing domain mode shift is coupled to the activation gate by the N-terminal tail of hERG channels. J Gen Physiol (2012) 0.95
Molecular mapping of a site for Cd2+-induced modification of human ether-à-go-go-related gene (hERG) channel activation. J Physiol (2005) 0.93
Multiple interactions between cytoplasmic domains regulate slow deactivation of Kv11.1 channels. J Biol Chem (2014) 0.93
Voltage-dependent gating of HERG potassium channels. Front Pharmacol (2012) 0.93
Thermodynamic and kinetic properties of amino-terminal and S4-S5 loop HERG channel mutants under steady-state conditions. Biophys J (2008) 0.92
Cytoplasmic domains and voltage-dependent potassium channel gating. Front Pharmacol (2012) 0.90
Components of gating charge movement and S4 voltage-sensor exposure during activation of hERG channels. J Gen Physiol (2013) 0.90
S3-S4 linker length modulates the relaxed state of a voltage-gated potassium channel. Biophys J (2013) 0.90
A gating charge interaction required for late slow inactivation of the bacterial sodium channel NavAb. J Gen Physiol (2013) 0.89
Getting to the heart of hERG K(+) channel gating. J Physiol (2015) 0.87
Voltage clamp fluorimetry reveals a novel outer pore instability in a mammalian voltage-gated potassium channel. J Gen Physiol (2008) 0.85
Molecular coupling in the human ether-a-go-go-related gene-1 (hERG1) K+ channel inactivation pathway. J Biol Chem (2011) 0.85
Regional flexibility in the S4-S5 linker regulates hERG channel closed-state stabilization. Pflugers Arch (2014) 0.84
Proline scan of the HERG channel S6 helix reveals the location of the intracellular pore gate. Biophys J (2014) 0.83
Computational cardiology and risk stratification for sudden cardiac death: one of the grand challenges for cardiology in the 21st century. J Physiol (2016) 0.83
Transfer of rolf S3-S4 linker to HERG eliminates activation gating but spares inactivation. Biophys J (2009) 0.83
Bimodal voltage dependence of TRPA1: mutations of a key pore helix residue reveal strong intrinsic voltage-dependent inactivation. Pflugers Arch (2013) 0.82
Bimodal regulation of an Elk subfamily K+ channel by phosphatidylinositol 4,5-bisphosphate. J Gen Physiol (2015) 0.80
Retigabine holds KV7 channels open and stabilizes the resting potential. J Gen Physiol (2016) 0.79
Gating charge movement precedes ionic current activation in hERG channels. Channels (Austin) (2013) 0.79
A functional Kv1.2-hERG chimaeric channel expressed in Pichia pastoris. Sci Rep (2014) 0.78
Sequence of gating charge movement and pore gating in HERG activation and deactivation pathways. Biophys J (2015) 0.78
NS1643 interacts around L529 of hERG to alter voltage sensor movement on the path to activation. Biophys J (2015) 0.77
Kinetic model for NS1643 drug activation of WT and L529I variants of Kv11.1 (hERG1) potassium channel. Biophys J (2015) 0.77
Hysteresis in voltage-gated channels. Channels (Austin) (2016) 0.76
The isolated voltage sensing domain of the Shaker potassium channel forms a voltage-gated cation channel. Elife (2016) 0.76
The electric heart of hERG. J Gen Physiol (2013) 0.75
Hysteresis of KcsA potassium channel's activation- deactivation gating is caused by structural changes at the channel's selectivity filter. Proc Natl Acad Sci U S A (2017) 0.75
Stabilization of the Activated hERG Channel Voltage Sensor by Depolarization Involves the S4-S5 Linker. Biophys J (2017) 0.75
Tyrosine Residues from the S4-S5 Linker of Kv11.1 Channels Are Critical for Slow Deactivation. J Biol Chem (2016) 0.75
Inactivation of the sodium channel. II. Gating current experiments. J Gen Physiol (1977) 14.17
A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel. Cell (1995) 11.62
Shaker potassium channel gating. III: Evaluation of kinetic models for activation. J Gen Physiol (1994) 7.23
The voltage sensor in voltage-dependent ion channels. Physiol Rev (2000) 6.86
The inward rectification mechanism of the HERG cardiac potassium channel. Nature (1996) 6.29
Molecular and cellular mechanisms of cardiac arrhythmias. Cell (2001) 5.41
Activation of Shaker potassium channels. III. An activation gating model for wild-type and V2 mutant channels. J Gen Physiol (1998) 4.56
Inward rectifier potassium channels. Annu Rev Physiol (1997) 4.53
Fast inactivation causes rectification of the IKr channel. J Gen Physiol (1996) 3.88
Novel voltage clamp to record small, fast currents from ion channels expressed in Xenopus oocytes. Biophys J (1992) 3.75
Correlation between charge movement and ionic current during slow inactivation in Shaker K+ channels. J Gen Physiol (1997) 3.54
Molecular determinants for activation and inactivation of HERG, a human inward rectifier potassium channel. J Physiol (1996) 3.29
Gating of Shaker K+ channels: I. Ionic and gating currents. Biophys J (1994) 3.28
Cut-open oocyte voltage-clamp technique. Methods Enzymol (1998) 2.67
Interactions between S4-S5 linker and S6 transmembrane domain modulate gating of HERG K+ channels. J Biol Chem (2002) 2.34
Fast and slow voltage sensor movements in HERG potassium channels. J Gen Physiol (2002) 1.57
A mutation in the pore region of HERG K+ channels expressed in Xenopus oocytes reduces rectification by shifting the voltage dependence of inactivation. J Physiol (1998) 1.54
Extracellular Mg(2+) modulates slow gating transitions and the opening of Drosophila ether-à-Go-Go potassium channels. J Gen Physiol (2000) 1.39
Single HERG delayed rectifier K+ channels expressed in Xenopus oocytes. Am J Physiol (1997) 1.37
Effects of premature stimulation on HERG K(+) channels. J Physiol (2001) 1.28
Pathways of HERG inactivation. Am J Physiol (1999) 1.22
Cardiac electrophysiologic and antiarrhythmic actions of two long-acting spirobenzopyran piperidine class III agents, L-702,958 and L-706,000 [MK-499]. J Pharmacol Exp Ther (1994) 0.97
Ca(V)1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism. Cell (2004) 7.45
hERG potassium channels and cardiac arrhythmia. Nature (2006) 6.63
Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST-segment elevation, short QT intervals, and sudden cardiac death. Circulation (2007) 4.37
Severe arrhythmia disorder caused by cardiac L-type calcium channel mutations. Proc Natl Acad Sci U S A (2005) 4.14
International Union of Pharmacology. LIII. Nomenclature and molecular relationships of voltage-gated potassium channels. Pharmacol Rev (2005) 3.50
Interactions between S4-S5 linker and S6 transmembrane domain modulate gating of HERG K+ channels. J Biol Chem (2002) 2.34
An Fgf8 mouse mutant phenocopies human 22q11 deletion syndrome. Development (2002) 2.31
Compound mutations: a common cause of severe long-QT syndrome. Circulation (2004) 2.29
A new oral therapy for long QT syndrome: long-term oral potassium improves repolarization in patients with HERG mutations. J Am Coll Cardiol (2003) 2.28
De novo KCNQ1 mutation responsible for atrial fibrillation and short QT syndrome in utero. Cardiovasc Res (2005) 2.27
Zebrafish model for human long QT syndrome. Proc Natl Acad Sci U S A (2007) 2.13
Genetic and physiologic dissection of the vertebrate cardiac conduction system. PLoS Biol (2008) 2.08
KCNQ2 and KCNQ3 potassium channel genes in benign familial neonatal convulsions: expansion of the functional and mutation spectrum. Brain (2003) 1.92
Electrocardiographic features in Andersen-Tawil syndrome patients with KCNJ2 mutations: characteristic T-U-wave patterns predict the KCNJ2 genotype. Circulation (2005) 1.77
Stretch-sensitive KCNQ1 mutation A link between genetic and environmental factors in the pathogenesis of atrial fibrillation? J Am Coll Cardiol (2007) 1.67
The S4-S5 linker directly couples voltage sensor movement to the activation gate in the human ether-a'-go-go-related gene (hERG) K+ channel. J Biol Chem (2006) 1.64
Voltage-dependent gating of hyperpolarization-activated, cyclic nucleotide-gated pacemaker channels: molecular coupling between the S4-S5 and C-linkers. J Biol Chem (2004) 1.60
Physicochemical features of the HERG channel drug binding site. J Biol Chem (2003) 1.54
Regulation of endocytic recycling of KCNQ1/KCNE1 potassium channels. Circ Res (2007) 1.52
Position of aromatic residues in the S6 domain, not inactivation, dictates cisapride sensitivity of HERG and eag potassium channels. Proc Natl Acad Sci U S A (2002) 1.51
Molecular determinants of HERG channel block. Mol Pharmacol (2006) 1.49
Mechanism of action of a novel human ether-a-go-go-related gene channel activator. Mol Pharmacol (2005) 1.43
Molecular determinants of voltage-dependent human ether-a-go-go related gene (HERG) K+ channel block. J Biol Chem (2002) 1.33
Structural basis of action for a human ether-a-go-go-related gene 1 potassium channel activator. Proc Natl Acad Sci U S A (2007) 1.32
Molecular basis for Kv1.5 channel block: conservation of drug binding sites among voltage-gated K+ channels. J Biol Chem (2003) 1.30
The molecular basis of chloroquine block of the inward rectifier Kir2.1 channel. Proc Natl Acad Sci U S A (2008) 1.30
Temperature dependence of human ether-a-go-go-related gene K+ currents. Am J Physiol Cell Physiol (2006) 1.29
Structural determinants of HERG channel block by clofilium and ibutilide. Mol Pharmacol (2004) 1.29
Defective potassium channel Kir2.1 trafficking underlies Andersen-Tawil syndrome. J Biol Chem (2003) 1.27
Regional specificity of human ether-a'-go-go-related gene channel activation and inactivation gating. J Biol Chem (2004) 1.26
Mechanisms by which atrial fibrillation-associated mutations in the S1 domain of KCNQ1 slow deactivation of IKs channels. J Physiol (2008) 1.22
Conformational changes in the M2 muscarinic receptor induced by membrane voltage and agonist binding. J Physiol (2011) 1.21
Voltage-dependent profile of human ether-a-go-go-related gene channel block is influenced by a single residue in the S6 transmembrane domain. Mol Pharmacol (2003) 1.20
Binding site of a novel Kv1.5 blocker: a "foot in the door" against atrial fibrillation. Mol Pharmacol (2006) 1.19
Lethal arrhythmias in Tbx3-deficient mice reveal extreme dosage sensitivity of cardiac conduction system function and homeostasis. Proc Natl Acad Sci U S A (2011) 1.19
Pharmacological activation of normal and arrhythmia-associated mutant KCNQ1 potassium channels. Circ Res (2003) 1.19
Tight coupling of rubidium conductance and inactivation in human KCNQ1 potassium channels. J Physiol (2003) 1.15
Molecular determinants of KCNQ1 channel block by a benzodiazepine. Mol Pharmacol (2003) 1.14
Differential roles of S6 domain hinges in the gating of KCNQ potassium channels. Biophys J (2005) 1.13
PD-118057 contacts the pore helix of hERG1 channels to attenuate inactivation and enhance K+ conductance. Proc Natl Acad Sci U S A (2009) 1.12
Somatic mosaicism contributes to phenotypic variation in Timothy syndrome. Am J Med Genet A (2011) 1.09
Novel KChIP2 isoforms increase functional diversity of transient outward potassium currents. J Physiol (2004) 1.05
Mutation of colocalized residues of the pore helix and transmembrane segments S5 and S6 disrupt deactivation and modify inactivation of KCNQ1 K+ channels. J Physiol (2005) 1.05
Molecular determinants of hERG channel block by terfenadine and cisapride. J Pharmacol Sci (2008) 1.05
Role of store-operated calcium channels and calcium sensitization in normoxic contraction of the ductus arteriosus. Circulation (2006) 1.04
Modification of hERG1 channel gating by Cd2+. J Gen Physiol (2010) 1.03
Structural basis for competition between drug binding and Kvbeta 1.3 accessory subunit-induced N-type inactivation of Kv1.5 channels. Mol Pharmacol (2005) 1.03
Molecular determinants for activation of human ether-à-go-go-related gene 1 potassium channels by 3-nitro-n-(4-phenoxyphenyl) benzamide. Mol Pharmacol (2011) 1.02
Structural determinants of Kvbeta1.3-induced channel inactivation: a hairpin modulated by PIP2. EMBO J (2008) 1.02
Not all hERG pore domain mutations have a severe phenotype: G584S has an inactivation gating defect with mild phenotype compared to G572S, which has a dominant negative trafficking defect and a severe phenotype. J Cardiovasc Electrophysiol (2009) 1.02
Side pockets provide the basis for a new mechanism of Kv channel-specific inhibition. Nat Chem Biol (2013) 0.99
Stoichiometry of altered hERG1 channel gating by small molecule activators. J Gen Physiol (2014) 0.99
Structural determinants for high-affinity block of hERG potassium channels. Novartis Found Symp (2005) 0.97
Relaxation gating of the acetylcholine-activated inward rectifier K+ current is mediated by intrinsic voltage sensitivity of the muscarinic receptor. J Physiol (2011) 0.97
Impaired interaction between the slide helix and the C-terminus of Kir2.1: a novel mechanism of Andersen syndrome. Cardiovasc Res (2007) 0.96
Structural determinants and biophysical properties of HERG and KCNQ1 channel gating. J Mol Cell Cardiol (2003) 0.95
Long QT syndrome-associated mutations in KCNQ1 and KCNE1 subunits disrupt normal endosomal recycling of IKs channels. Circ Res (2008) 0.95
Activation of Slo2.1 channels by niflumic acid. J Gen Physiol (2010) 0.94
Blebbistatin effectively uncouples the excitation-contraction process in zebrafish embryonic heart. Cell Physiol Biochem (2010) 0.94
Molecular mapping of a site for Cd2+-induced modification of human ether-à-go-go-related gene (hERG) channel activation. J Physiol (2005) 0.93
Molecular mapping of the binding site for a blocker of hyperpolarization-activated, cyclic nucleotide-modulated pacemaker channels. J Pharmacol Exp Ther (2007) 0.90
Cooperative interactions between R531 and acidic residues in the voltage sensing module of hERG1 channels. Cell Physiol Biochem (2008) 0.90
Block of wild-type and inactivation-deficient human ether-a-go-go-related gene K+ channels by halofantrine. Naunyn Schmiedebergs Arch Pharmacol (2004) 0.90
Modeling of IK1 mutations in human left ventricular myocytes and tissue. Am J Physiol Heart Circ Physiol (2006) 0.89
Structural basis for ether-a-go-go-related gene K+ channel subtype-dependent activation by niflumic acid. Mol Pharmacol (2008) 0.89
Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome. J Cardiovasc Electrophysiol (2007) 0.88
Structure-activity relationship of fenamates as Slo2.1 channel activators. Mol Pharmacol (2012) 0.88
Marine algal toxin azaspiracid is an open-state blocker of hERG potassium channels. Chem Res Toxicol (2012) 0.88
Resuscitated sudden cardiac death in Andersen-Tawil syndrome. Heart Rhythm (2009) 0.88
Tuning of EAG K(+) channel inactivation: molecular determinants of amplification by mutations and a small molecule. J Gen Physiol (2012) 0.87
Molecular determinants of Kv1.5 channel block by diphenyl phosphine oxide-1. J Mol Cell Cardiol (2010) 0.87
Andersen-Tawil syndrome: an ever-expanding phenotype? Heart Rhythm (2006) 0.87
Reduced transient outward K+ current and cardiac hypertrophy: causal relationship or epiphenomenon? Circ Res (2002) 0.86
Niflumic acid alters gating of HCN2 pacemaker channels by interaction with the outer region of S4 voltage sensing domains. Mol Pharmacol (2009) 0.86
Structural basis of ion permeation gating in Slo2.1 K+ channels. J Gen Physiol (2013) 0.85
Sudden cardiac death in Andersen-Tawil syndrome. Europace (2007) 0.85
Molecular determinants of human ether-à-go-go-related gene 1 (hERG1) K+ channel activation by NS1643. Mol Pharmacol (2010) 0.85
Molecular coupling in the human ether-a-go-go-related gene-1 (hERG1) K+ channel inactivation pathway. J Biol Chem (2011) 0.85
A highly conserved alanine in the S6 domain of the hERG1 K+ channel is required for normal gating. Cell Physiol Biochem (2008) 0.83
Molecular basis for a high-potency open-channel block of Kv1.5 channel by the endocannabinoid anandamide. Mol Pharmacol (2010) 0.83
A single amino acid difference between ether-a-go-go- related gene channel subtypes determines differential sensitivity to a small molecule activator. Mol Pharmacol (2007) 0.83
Kv1.5 open channel block by the antiarrhythmic drug disopyramide: molecular determinants of block. J Pharmacol Sci (2008) 0.83
Serotonin-induced inhibition of KV current: a supporting role in pulmonary vasoconstriction? Circ Res (2006) 0.83
Congenital long QT 3 in the pediatric population. Am J Cardiol (2012) 0.83
The absence of insulin signaling in the heart induces changes in potassium channel expression and ventricular repolarization. Am J Physiol Heart Circ Physiol (2013) 0.82
The pharmacokinetics of methadone and its metabolites in neonates, infants, and children. Paediatr Anaesth (2014) 0.82
Chloroquine blocks a mutant Kir2.1 channel responsible for short QT syndrome and normalizes repolarization properties in silico. Cell Physiol Biochem (2009) 0.81
Low potassium dextran lung preservation solution reduces reactive oxygen species production. Ann Thorac Surg (2003) 0.81
3-OST-7 regulates BMP-dependent cardiac contraction. PLoS Biol (2013) 0.81
An in vivo cardiac assay to determine the functional consequences of putative long QT syndrome mutations. Circ Res (2013) 0.81
Hysteresis in human HCN4 channels: a crucial feature potentially affecting sinoatrial node pacemaking. Sheng Li Xue Bao (2010) 0.80
Voltage sensor movement in the hERG K+ channel. Novartis Found Symp (2005) 0.80
Voltage sensing and activation gating of HCN pacemaker channels. Trends Cardiovasc Med (2002) 0.80
ICA-105574 interacts with a common binding site to elicit opposite effects on inactivation gating of EAG and ERG potassium channels. Mol Pharmacol (2013) 0.79
Voltage sensitivity of M2 muscarinic receptors underlies the delayed rectifier-like activation of ACh-gated K(+) current by choline in feline atrial myocytes. J Physiol (2013) 0.78