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    GENE:

    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)

    i
    Other names: KCNH2, Potassium Voltage-Gated Channel Subfamily H Member 2, HERG, Kv11.1, Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2, Ether-A-Go-Go-Related Gene Potassium Channel 1, Voltage-Gated Potassium Channel Subunit Kv11.1, Ether-A-Go-Go-Related Protein 1, Long QT Syndrome Type 2, Eag-Related Protein 1, Eag Homolog, ERG-1, H-ERG, HERG1, Erg1, ERG1, LQT2, Potassium Channel, Voltage Gated Eag Related Subfamily H, Member 2, Ether-A-Go-Go-Related Potassium Channel Protein, Human Ether-A-Go-Go-Related Gene, Human Ether-A-Go-Go-Related, HERG-1, SQT1, ERG
    Associations

    News

    Trials
    1m
    Wave II: Evaluation of Efficacy and Safety of THRV-1268 in Long QT Syndrome Type 2 (LQTS 2) (clinicaltrials.gov)
    P2/3, N=64, Recruiting, Thryv Therapeutics, Inc. | Not yet recruiting --> Recruiting
    Enrollment open
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    4ms
    Association of antipsychotic drug target genes and risk of lung cancer: a Mendelian randomization study. (PubMed, Discov Oncol)
    These findings provide insights into the potential oncologic implications of antipsychotic drug use and may inform clinical management.
    Journal
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    4ms
    Machine learning prediction model combined with network toxicology analysis identifies potential cardiotoxic components and mechanisms among 741 pesticides. (PubMed, Environ Int)
    The integrated machine learning and network toxicology approach developed in this study is reliable. Pesticides predicted to have potential cardiotoxicity require prioritized prevention during use.
    Journal
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    7ms
    LQT-1213-0061: Study of LQT-1213 on QTc-induced Prolongation in Healthy Adult Subjects (Part1) and on Congenital Long QT in Patients Diagnosed With Type 2 or 3 Long QT Syndrome (Part 2). (clinicaltrials.gov)
    P1/2, N=42, Terminated, Thryv Therapeutics, Inc. | N=28 --> 42 | Active, not recruiting --> Terminated; Advancing an alternative SGK1 inhibitor.
    Enrollment change • Trial termination
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    9ms
    CarDiag: Building of a Diagnostic/Prognostic Database for Human ERG Variant Effects (clinicaltrials.gov)
    P=N/A, N=600, Recruiting, Nantes University Hospital | Trial completion date: Mar 2025 --> Mar 2026
    Trial completion date
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    10ms
    MAST2: Mutation-specific Therapy for the Long QT Syndrome (clinicaltrials.gov)
    P2, N=16, Completed, Istituto Auxologico Italiano | Recruiting --> Completed
    Trial completion
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    12ms
    In silico development of HASDI-G2 as a novel agent for selective recognition of the DNA sequence. (PubMed, Sci Rep)
    Such complexes showed significant conformational rearrangements, accompanied by a sharp decrease in the hydrogen bonds quantity, a drop in the binding free energy, and local melting of the DNA duplex. Moreover, the latter applied not only to sites of direct incompatibility, but also to parts where HASDI-G2 fully corresponded to the sequence of intercalation.
    Licensing / partnership • Journal
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    12ms
    LncRNA MALAT1/Calpain-1 Axis in ATO Induced hERG Channel Deficiency. (PubMed, Drug Des Devel Ther)
    Arsenic trioxide (ATO) is an effective therapeutic agent for acute promyelocytic leukemia; however, its long-term use can lead to cardiotoxicity, particularly in cases of acquired long QT syndrome (acLQTS), which may result in torsade de pointes (TdP)...Tanshinone IIA and fexofenadine restored the hERG protein levels potentially by decreasing MALAT1 expression and counteracting ATO's effects on the MALAT1/calpain-1 pathway. Collectively, our research uncovers a previously unreported regulatory mechanism underlying ATO-induced acLQTS. Moreover, it identifies potential molecular targets and intervention strategies for acLQTS therapy.
    Journal
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    MALAT1 (Metastasis associated lung adenocarcinoma transcript 1) • CAPN1 (Calpain 1) • KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
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    arsenic trioxide
    12ms
    LQT-1213-0061: Study of LQT-1213 on QTc-induced Prolongation in Healthy Adult Subjects (Part1) and on Congenital Long QT in Patients Diagnosed With Type 1, 2 or 3 Long QT Syndrome (Part 2). (clinicaltrials.gov)
    P1/2, N=28, Active, not recruiting, Thryv Therapeutics, Inc. | Recruiting --> Active, not recruiting
    Enrollment closed
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    1year
    Clinical relevance of macromolecular complexes involving integrins, potassium and sodium ion channels and the sodium/proton antiporter in human breast cancer. (PubMed, Cancer Cell Int)
    hERG1 and nNav1.5 channels along with β1 integrins and the NHE1 antiporter are co-expressed in BCa both at gene and protein levels, assembling into a macromolecular complex. The NHE1/hERG1/β1/nNaV1.5 complex can be considered a novel biomarker and potential target for therapy for BCa patients.
    Journal
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    ITGB1 (Integrin Subunit Beta 1) • KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)
    1year
    Unveiling the substantial role of rutin in the management of drug-induced nephropathy using network pharmacology and molecular docking. (PubMed, Int Immunopharmacol)
    The revelation of mode of action of bioactive constituent rutin against drug-induced nephropathy provides a theoretical basis for designing more promising compounds in future for treatment of nephropathy.
    Journal
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    EGFR (Epidermal growth factor receptor) • ER (Estrogen receptor) • TP53 (Tumor protein P53) • FLT3 (Fms-related tyrosine kinase 3) • MCL1 (Myeloid cell leukemia 1) • KDR (Kinase insert domain receptor) • ABCG2 (ATP Binding Cassette Subfamily G Member 2) • PIK3R1 (Phosphoinositide-3-Kinase Regulatory Subunit 1) • PIK3CG (Phosphatidylinositol-4,5-Bisphosphate 3-Kinase Catalytic Subunit Gamma) • PRKCH (Protein Kinase C Eta) • IL2 (Interleukin 2) • PLK1 (Polo Like Kinase 1) • CHEK1 (Checkpoint kinase 1) • SYK (Spleen tyrosine kinase) • PTGS2 (Prostaglandin-Endoperoxide Synthase 2) • ALDH2 (Aldehyde Dehydrogenase 2 Family Member) • IR (Insulin receptor) • MMP9 (Matrix metallopeptidase 9) • SERPINE1 (Serpin Family E Member 1) • CDK1 (Cyclin-dependent kinase 1) • CXCR1 (Chemokine (C-X-C motif) receptor 1) • HSD17B2 (Hydroxysteroid 17-Beta Dehydrogenase 2) • MAPT (Microtubule Associated Protein Tau) • NOX4 (NADPH Oxidase 4) • PDE5A (Phosphodiesterase 5A) • PRKACA (Protein Kinase CAMP-Activated Catalytic Subunit Alpha) • PRKCA (Protein Kinase C Alpha) • PRKCB (Protein Kinase C Beta) • CYP1B1 (Cytochrome P450 Family 1 Subfamily B Member 1) • KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2) • MMP3 (Matrix metallopeptidase 3) • PACERR (PTGS2 Antisense NFKB1 Complex-Mediated Expression Regulator RNA)
    1year
    Human induced pluripotent stem cell line XXMUFAi001-A generated from a patient harboring KCNH2 mutation (c. 2690 A>C). (PubMed, Stem Cell Res)
    XXMUFAi001-A cell line exhibited expression of pluripotency markers and demonstrated teratoma formation capability in vivo, as well as the potential to differentiate into all three germ layers. The establishment of XXMUFAi001-A is crucial for investigating the pathogenesis and drug screening of LQT2.
    Preclinical • Journal
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    KCNH2 (Potassium Voltage-Gated Channel Subfamily H Member 2)