Kcnh2 mediates FAK/AKT-FOXO3A pathway to attenuate sepsis-induced cardiac dysfunction. PMID: 33263944
Role of ERG1 isoforms in modulation of ERG1 channel trafficking and function. PMID: 20574821
Spectrum and prevalence of mutations from the first 2,500 consecutive unrelated patients referred for the FAMILION long QT syndrome genetic test. PMID: 19716085
Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants. PMID: 19841300
Decoding KCNH2 variants of unknown significance. PMID: 31593778
Cryo-EM Structure of the Open Human Ether-à-go-go-Related K+ Channel hERG. PMID: 28431243
HERG1 channelopathies. PMID: 20544339
KCNH2 pharmacogenomics summary. PMID: 20150828
ERG proteins and functional cardiac I(Kr) channels in rat, mouse, and human heart. PMID: 11709283
HERG channel trafficking. PMID: 16050262
[Short QT syndrome]. PMID: 17497253
Potassium Ion Channel Protein (KCNH) Levels in Patients with Fibromyalgia Syndrome. PMID: 35818223
Protein distribution of Kcnq1, Kcnh2, and Kcne3 potassium channel subunits during mouse embryonic development. PMID: 16463373
Zwitterionic CRTh2 antagonists. PMID: 21355602
Modulation of rat erg1, erg2, erg3 and HERG K+ currents by thyrotropin-releasing hormone in anterior pituitary cells via the native signal cascade. PMID: 11283231
ERG1 plays an essential role in rat cardiomyocyte fate decision by mediating AKT signaling. PMID: 33426760
Gene therapy to keep the QT rhythms "on the QT". PMID: 28789785
Novel bacterial topoisomerase inhibitors: challenges and perspectives in reducing hERG toxicity. PMID: 30215281
Specific serine proteases selectively damage KCNH2 (hERG1) potassium channels and I(Kr). PMID: 16227340
Expression and coassociation of ERG1, KCNQ1, and KCNE1 potassium channel proteins in horse heart. PMID: 12063283
Mission possible: RNA interference rescues the hERG current. PMID: 23063431
New insights of the role of the KCNH2 gene in schizophrenia: An fMRI case-control study. PMID: 35635995
Gene symbol: KCNH2. PMID: 17438608
Long QT Syndrome Type 2: Emerging Strategies for Correcting Class 2 KCNH2 (hERG) Mutations and Identifying New Patients. PMID: 32759882
I(Kr): the hERG channel. PMID: 11343409
Delayed rectifier K(+) currents and cardiac repolarization. PMID: 19683534
Functional study of a KCNH2 mutant: Novel insights on the pathogenesis of the LQT2 syndrome. PMID: 31361068
Loss-of-function variants in Kv 11.1 cardiac channels as a biomarker for SUDEP. PMID: 34002542
Look beyond the hERG mutation: a neutral SCN5A variant may turn lidocaine into a threat. PMID: 18984535
HERG K+ channels: friend and foe. PMID: 11339975
Strategies for the design of selective protein kinase inhibitors. PMID: 18855742
hERG (KCNH2 or Kv11.1) K+ channels: screening for cardiac arrhythmia risk. PMID: 18991593
Deciphering hERG channels: molecular basis of the rapid component of the delayed rectifier potassium current. PMID: 22742967
Tbx20 controls the expression of the KCNH2 gene and of hERG channels. PMID: 28049825
Mutation-Specific Differences in Kv7.1 (KCNQ1) and Kv11.1 (KCNH2) Channel Dysfunction and Long QT Syndrome Phenotypes. PMID: 35806392
Analysis of CACNA1C and KCNH2 Risk Variants on Cardiac Autonomic Function in Patients with Schizophrenia. PMID: 36421807
Kv4.3 Modulates the Distribution of hERG. PMID: 29259226
Pre-Test Probability and Genes and Variants of Uncertain Significance in Familial Long QT Syndrome. PMID: 32044265
New targets for the antitumor activity of gambogic acid in hematologic malignancies. PMID: 23274413
Mutant hERG channel traffic jam. Focus on "Pharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum". PMID: 23986200
Ion channels: structure of a molecular brake. PMID: 10074448
A plethora of mechanisms in the HERG-related long QT syndrome. Genetics meets electrophysiology. PMID: 10690299
Integrated analysis of the voltage-gated potassium channel-associated gene KCNH2 across cancers. PMID: 36792990
Key role for Kv11.1 (ether-a-go-go related gene) channels in rat bladder contractility. PMID: 36750122
Expression and function of KCNH2 (HERG) in the human jejunum. PMID: 12736144
Regulation of the cardiac repolarizing HERG potassium channel by protein kinase A. PMID: 11282296
Identification and Characterization of a Transcribed Distal Enhancer Involved in Cardiac Kcnh2 Regulation. PMID: 31484079
Discovery and optimization of oxadiazole-based FLAP inhibitors. PMID: 28927794
The electric heart of hERG. PMID: 23478994
LQT2 : amplitude reduction and loss of selectivity in the tail that wags the HERG channel. PMID: 10720408
Expression of distinct ERG proteins in rat, mouse, and human heart. Relation to functional I(Kr) channels. PMID: 10681594
AKAP5 anchors PKA to enhance regulation of the HERG channel. PMID: 32173522
Reclassification of a likely pathogenic Dutch founder variant in KCNH2; implications of reduced penetrance. PMID: 36269083
ANK2 functionally interacts with KCNH2 aggravating long QT syndrome in a double mutation carrier. PMID: 30929919
Human ether-a-go-go related gene (HERG): a chemist's perspective. PMID: 15850821
A massively parallel assay accurately discriminates between functionally normal and abnormal variants in a hotspot domain of KCNH2. PMID: 35688148
Digenic heterozygous mutations of KCNH2 and SCN5A induced young and early-onset long QT syndrome and sinoatrial node dysfunction. PMID: 34755423
Regulation of Kv11.1 Isoform Expression by Polyadenylate Binding Protein Nuclear 1. PMID: 33467093
Long QT syndrome: cellular basis and arrhythmia mechanism in LQT2. PMID: 11196567
Exploiting ion channel structure to assess rare variant pathogenicity. PMID: 29330128
Activators of cation channels: potential in treatment of channelopathies. PMID: 15608141
Genetic Variants on SCN5A, KCNQ1, and KCNH2 in Patients with Ventricular Arrhythmias during Acute Myocardial Infarction in a Chinese Population. PMID: 31751991
The long QT syndrome: a clinical counterpart of hERG mutations. PMID: 16050269
Defective protein trafficking in hERG-associated hereditary long QT syndrome (LQT2): molecular mechanisms and restoration of intracellular protein processing. PMID: 14613852
KCNH2-3.1 expression impairs cognition and alters neuronal function in a model of molecular pathology associated with schizophrenia. PMID: 26857598
Drug binding to HERG channels: evidence for a 'non-aromatic' binding site for fluvoxamine. PMID: 12839860
Short QT syndrome: a very rare arrhythmogenic entity. PMID: 19013996
Structural determinants and biophysical properties of HERG and KCNQ1 channel gating. PMID: 12623297
Common Variants in KCNE1, KCNH2, and SCN5A May Impact Cardiac Arrhythmia Risk. PMID: 33517668
Influence of genetic modifiers on sudden cardiac death cases. PMID: 29214556
Cardiac ion channels and antihistamines: possible mechanisms of cardiotoxicity. PMID: 10444235
KCNH2-K897T is a genetic modifier of latent congenital long-QT syndrome. PMID: 16116052
Isogenic Sets of hiPSC-CMs Harboring Distinct KCNH2 Mutations Differ Functionally and in Susceptibility to Drug-Induced Arrhythmias. PMID: 33176122
Novel intracellular transport-refractory mutations in KCNH2 identified in patients with symptomatic long QT syndrome. PMID: 29146210
Regulation of Kv11.1 potassium channel C-terminal isoform expression by the RNA-binding proteins HuR and HuD. PMID: 30377250
Comparison of protein behavior between wild-type and G601S hERG in living cells by fluorescence correlation spectroscopy. PMID: 21573751
Tetrameric Assembly of K+ Channels Requires ER-Located Chaperone Proteins. PMID: 27916661
Co-chaperone FKBP38 promotes HERG trafficking. PMID: 17569659
HERG binding specificity and binding site structure: evidence from a fragment-based evolutionary computing SAR study. PMID: 15288759
hERG channel trafficking: novel targets in drug-induced long QT syndrome. PMID: 17956279
Variant frequencies of KCNQ1, KCNH2, and SCN5A in a Chinese inherited arrhythmia cohort and other disease cohorts undergoing genetic testing. PMID: 31696929
Dysfunction of delayed rectifier potassium channels in an inherited cardiac arrhythmia. PMID: 10414310
QT interval prolongation and cardiac risk assessment for novel drugs. PMID: 12735231
A system-level approach to investigate alloxan-induced toxicity in microtubule-binding protein to lead type 2 diabetes mellitus. PMID: 32219736
A primate-specific, brain isoform of KCNH2 affects cortical physiology, cognition, neuronal repolarization and risk of schizophrenia. PMID: 19412172
Regulation of an ERG K+ current by Src tyrosine kinase. PMID: 11834728
Screening for cardiac HERG potassium channel interacting proteins using the yeast two-hybrid technique. PMID: 24154981
Mutation Analysis of KCNQ1, KCNH2 and SCN5A Genes in Taiwanese Long QT Syndrome Patients. PMID: 26118593
Co-expression of calcium and hERG potassium channels reduces the incidence of proarrhythmic events. PMID: 33002116
Intragenic suppression of trafficking-defective KCNH2 channels associated with long QT syndrome. PMID: 15851652
Generation of three heterozygous KCNH2 mutation-carrying human induced pluripotent stem cell lines for modeling LQT2 syndrome. PMID: 34051449
Role and mechanism of chaperones calreticulin and ERP57 in restoring trafficking to mutant HERG‑A561V protein. PMID: 34212985
A microtranslatome coordinately regulates sodium and potassium currents in the human heart. PMID: 31670657
Human ether-a-gogo related gene (HERG) K+ channels as pharmacological targets: present and future implications. PMID: 9714291
The human ERG1 channel polymorphism, K897T, creates a phosphorylation site that inhibits channel activity. PMID: 18791070
Nonsense-Mediated mRNA Decay of hERG Mutations in Long QT Syndrome. PMID: 29058182
When Coronary Artery Disease Meets a Channelopathy: An Unexpected Dangerous Date. PMID: 31775145
Understanding the structure-activity relationship of the human ether-a-go-go-related gene cardiac K+ channel. A model for bad behavior. PMID: 12747773
Protective effect of KCNH2 single nucleotide polymorphism K897T in LQTS families and identification of novel KCNQ1 and KCNH2 mutations. PMID: 18808722
The non-synonymous coding IKr-channel variant KCNH2-K897T is associated with atrial fibrillation: results from a systematic candidate gene-based analysis of KCNH2 (HERG). PMID: 18222980
Generation of an induced pluripotent stem cell line from a long QT syndrome patient carrying KCNH2/1956C > A mutation. PMID: 35569348
Human induced pluripotent stem cell line ZZUNEUi027-A generated from a long QT syndrome patient with a heterozygous KCNH2 (c. 128 A > G) mutant. PMID: 35700634
Synonymous nucleotide modification of the KCNH2 gene affects both mRNA characteristics and translation of the encoded hERG ion channel. PMID: 29907571
Regulation of HERG (KCNH2) potassium channel surface expression by diacylglycerol. PMID: 19859662
The phosphorylation state of both hERG and KvLQT1 mediates protein-protein interactions between these complementary cardiac potassium channel alpha subunits. PMID: 33444623
A novel life-threatening mutation in long QT2 syndrome. PMID: 25987402
Dynamic control of hERG/I(Kr) by PKA-mediated interactions with 14-3-3. PMID: 16050263
High-throughput discovery of trafficking-deficient variants in the cardiac potassium channel KV11.1. PMID: 32522694
Computational analysis of the effect of KCNH2 L532P mutation on ventricular electromechanical behaviors. PMID: 33721574
Estradiol regulates human QT-interval: acceleration of cardiac repolarization by enhanced KCNH2 membrane trafficking. PMID: 26271031
KCNH2 encodes a nuclear-targeted polypeptide that mediates hERG1 channel gating and expression. PMID: 36626562
Bidirectional ventricular tachycardia and channelopathy. PMID: 14556882
KCNQ1 and KCNH2 mutations associated with long QT syndrome in a Chinese population. PMID: 12442276
Erg1, erg2 and erg3 K channel subunits are able to form heteromultimers. PMID: 11212207
Mutation analysis for the detection of long QT-syndrome (LQTS) associated SNPs. PMID: 27613431
Regulation of Isoform Expression by Blocking Polyadenylation Signal Sequences with Morpholinos. PMID: 28364240
Gene symbol: KCNH2. Disease: Long QT syndrome. PMID: 20960616
Identification and expression analysis of kcnh2 genes in the zebrafish. PMID: 20438705
Divergent expression of delayed rectifier K(+) channel subunits during mouse heart development. PMID: 11557234
Identification of Possible Risk Variants of Familial Strabismus Using Exome Sequencing Analysis. PMID: 33435129
H1153Y-KCNH2 Mutation Identified in a Sudden Arrhythmic Death Syndrome Case Alters Channel Gating. PMID: 34502138
Expression signature of miRNAs and the potential role of miR-195-5p in high-glucose-treated rat cardiomyocytes. PMID: 31729781
Functional significance of KCNH2 (HERG) K897T polymorphism for cardiac repolarization assessed by analysis of T-wave morphology. PMID: 16472284
Gene therapy approaches to ventricular tachyarrhythmias. PMID: 17993320
Structural analysis of hERG channel blockers and the implications for drug design. PMID: 36680816
HERG-like K+ channels in microglia. PMID: 9607936
Molecular and functional characterization of DENND3 as a novel regulator of ion channel trafficking., PMID:40423607
A synonymous KCNH2 polymorphism and methadone trough level influence QTc prolongation in Kelantanese Malay recipients of methadone maintenance therapy (MMT) in Malaysia., PMID:40323987
Strategic Design and Optimization of Umifenovir Analogues: Balancing Antiviral Efficacy and hERG Toxicity against SARS-CoV-2., PMID:40263709
Knock-in Kcnh2 rabbit model of long QT syndrome type-2, epilepsy, and sudden death., PMID:40234944
Molecular insights into the rescue mechanism of an HERG activator against severe LQT2 mutations., PMID:40197385
Discovery of New Difluorocyclobutyl Derivatives as Effective Glucagon-Like Peptide-1 Receptor Agonists with Reduced hERG Inhibitory Activities., PMID:40167442
Clinical Impact of Genetic Testing for Long QT Syndrome - Evidence From a Nationwide LQTS Registry in Japan., PMID:40159220
Donepezil-induced degradation of hERG potassium channel via lysosomal pathway is exacerbated by hypoxia., PMID:40157707
Loss-of-function variant in KCNH3 is associated with global developmental delay, autistic behavior, insomnia, and nocturnal seizures., PMID:40157307
GraphDeep-hERG: Graph Neural Network PharmacoAnalytics for Assessing hERG-Related Cardiotoxicity., PMID:40140128
Congenital long QT syndrome caused by a KCNH2 pathogenic variant exhibiting "motor seizures": a case report and literature review., PMID:40091034
ACA-24-3995.Highlighted: Determination of lopinavir and ritonavir in hERG solution to support in vitro hERG block potency assessment using LC-MS/MS: The challenge of poor drug solubility., PMID:40057315
Structure-Based Development of 3,4-Fused Tricyclic Benzofuran Derivatives as Polyketide Synthase 13 Inhibitors with Negligible hERG Inhibition., PMID:40045825
LncRNA MALAT1/Calpain-1 Axis in ATO Induced hERG Channel Deficiency., PMID:40041754
Low Proarrhythmic Risk of Imetelstat, a Novel Oligonucleotide Telomerase Inhibitor: A Translational Analysis., PMID:39980062
Topological Distribution of KCNH2 Variants and Genotype-Phenotype Relationship in Patients With Long QT Syndrome., PMID:39913104
Genetic background and clinical phenotype in a Vietnamese cohort with Brugada syndrome: A whole exome sequencing study., PMID:39895654
Effect of the Membrane Environment on Pharmacologic Inhibition of hERG K+ Channel Activity., PMID:39895450
Clinical relevance of macromolecular complexes involving integrins, potassium and sodium ion channels and the sodium/proton antiporter in human breast cancer., PMID:39865220
Identification of crucial pathways and genes linked to endoplasmic reticulum stress in PCOS through combined bioinformatic analysis., PMID:39850756
Unveiling the substantial role of rutin in the management of drug-induced nephropathy using network pharmacology and molecular docking., PMID:39733639
Human induced pluripotent stem cell line XXMUFAi001-A generated from a patient harboring KCNH2 mutation (c. 2690 A>C)., PMID:39709756
Chelerythrine triggers the prolongation of QT interval and induces cardiotoxicity by promoting the degradation of hERG channels., PMID:39608718
The Yield of Genetic Testing and Putative Genetic Factors of Disease Heterogeneity in Long QT Syndrome Patients., PMID:39596046
Copy Number Variants in Cardiac Channelopathies: Still a Missed Part in Routine Arrhythmic Diagnostics., PMID:39595626
[Propranolol triggered recurrent ventricular tachycardia and electrical storm in an infant with long QT syndrome type 2]., PMID:39563052
hERGBoost: A gradient boosting model for quantitative IC50 prediction of hERG channel blockers., PMID:39550914
Interpreting the actionable clinical role of rare variants associated with short QT syndrome., PMID:39503779
On the relationship between hERG inhibition and the magnitude of QTc prolongation: An in vitro to clinical translational analysis., PMID:39490450
Establishment of a human-induced pluripotent stem cell line from a long QT syndrome type 2 patient harboring a KCNH2 mutation., PMID:39454535
Toxicology profile of a novel GLP-1 receptor biased agonist-SAL0112 in nonhuman primates., PMID:39395609
Clinical Features, Long-Term Prognosis, and Clinical Management of Genotype-Negative Long QT Syndrome Patients., PMID:39387742
Torsades de Pointes electrical storm in children with KCNH2 mutations., PMID:39394151
Purkinje cell hyperexcitability and depressive-like behavior in mice lacking erg3 (ether-à-go-go-related gene) K+ channel subunits., PMID:39365861
[Clinical and genetic analysis of a Chinese pedigree affected with type 2 Long QT syndrome due to variant of KCNH2 gene]., PMID:39344617
Inhibition of hERG K channels by verapamil at physiological temperature: Implications for the CiPA initiative., PMID:39332652
Improved higher resolution cryo-EM structures reveal the binding modes of hERG channel inhibitors., PMID:39321803
Multiplexed Assays of Variant Effect and Automated Patch Clamping Improve KCNH2-LQTS Variant Classification and Cardiac Event Risk Stratification., PMID:39315434
hERG channel agonist NS1643 strongly inhibits invasive astrocytoma cell line SMA-560., PMID:39240809
Potassium dependent structural changes in the selectivity filter of HERG potassium channels., PMID:39209832
Development of an Absolute Quantification Method for hERG Using PRM with Single Isotopologue in-Sample Calibration., PMID:39130540
Potassium voltage-gated channel subfamily H member 2 (KCNH2) is a promising target for incretin secretagogue therapies., PMID:39128897
Investigating the cardiotoxicity of N-n-butyl haloperidol iodide: Inhibition mechanisms on hERG channels., PMID:39128488
Clinical Utility of Protein Language Models in Resolution of Variants of Uncertain Significance in KCNQ1, KCNH2, and SCN5A Compared With Patch-Clamp Functional Characterization., PMID:39119706
Neurocardiac pathologies associated with potassium channelopathies., PMID:39087855
Clinical presentation and genetic characterization of early-onset atrial fibrillation in patients affected by long QT syndrome: A single-center experience., PMID:39082327
Beneficial normalization of cardiac repolarization by carnitine in transgenic short QT syndrome type 1 rabbit models., PMID:39018021
Influence of genetic mutations to atria vulnerability to atrial fibrillation: An in-silico 3D human atria study., PMID:38981143
Rescue of expression and function of long QT syndrome-causing mutant hERG channels by enhancing channel stability in the plasma membrane., PMID:38960041
Therapeutic Efficacy of Mexiletine for Long QT Syndrome Type 2: Evidence From Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes, Transgenic Rabbits, and Patients., PMID:38939955
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