Gene Symbol: Kcnh2
Description: potassium voltage-gated channel subfamily H member 2
Alias: ERG1, potassium voltage-gated channel subfamily H member 2, ERG-1, RERG, eag-related protein 1, ether-a-go-go-related gene potassium channel 1, ether-a-go-go-related protein 1, potassium channel, voltage gated eag related subfamily H, member 2, potassium voltage-gated channel, subfamily H (eag-related), member 2, r-ERG, voltage-gated potassium channel subunit Kv11.1
Species: rat
Products:     Kcnh2

Top Publications

  1. Rasmussen H, Møller M, Knaus H, Jensen B, Olesen S, Jørgensen N. Subcellular localization of the delayed rectifier K(+) channels KCNQ1 and ERG1 in the rat heart. Am J Physiol Heart Circ Physiol. 2004;286:H1300-9 pubmed
    ..and subcellular localization of the two delayed rectifier K(+) channels, KCNQ1 and ether-a-go-go-related gene-1 (ERG1), was investigated in the adult rat heart...
  2. Trudeau M, Warmke J, Ganetzky B, Robertson G. HERG, a human inward rectifier in the voltage-gated potassium channel family. Science. 1995;269:92-5 pubmed
    ..Because mutations in HERG cause a form of long-QT syndrome, these properties of HERG channel function may be critical to the maintenance of normal cardiac rhythmicity. ..
  3. Kim D, Oh J, Lee E, Lee J, Park H, Kim C, et al. The stoichiometric relationship between KCNH-2 and KCNE-2 in I(Kr) channel formation. Int J Cardiol. 2010;145:272-274 pubmed publisher
    ..This finding, together with previous data from electrophysiological and molecular biological studies, supports that KCNH-2 and KCNE-2 are molecular components of I(Kr) channels. ..
  4. Lin J, Lin S, Choy P, Shen X, Deng C, Kuang S, et al. The regulation of the cardiac potassium channel (HERG) by caveolin-1. Biochem Cell Biol. 2008;86:405-15 pubmed publisher
    ..The finding from this study clearly demonstrates the regulatory role of caveolin-1 on HERG channels, and may help to understand biochemical events leading to arrhythmogenesis in the long QT syndrome in cardiac patients. ..
  5. Chun K, Koenen M, Katus H, Zehelein J. Expression of the IKr components KCNH2 (rERG) and KCNE2 (rMiRP1) during late rat heart development. Exp Mol Med. 2004;36:367-71 pubmed
    ..expression pattern of two rat potassium voltage-gated channels, namely subfamily H (eag-related), member2 (KCNH2) (alias name: rERG) and Isk-related family, member2 (KCNE2) (alias name: rMiRP1) during late embryonic development ..
  6. Shi W, Wymore R, Wang H, Pan Z, Cohen I, McKinnon D, et al. Identification of two nervous system-specific members of the erg potassium channel gene family. J Neurosci. 1997;17:9423-32 pubmed
    ..These two genes form a small gene family with the previously described erg1 (HERG) gene...
  7. Hong K, Bjerregaard P, Gussak I, Brugada R. Short QT syndrome and atrial fibrillation caused by mutation in KCNH2. J Cardiovasc Electrophysiol. 2005;16:394-6 pubmed
    ..A genetic basis has been identified linking the disease to mutations in KCNH2 in the familial forms and a mutation in KCNQ1 in a sporadic form of the disease...
  8. Wulfsen I, Hauber H, Schiemann D, Bauer C, Schwarz J. Expression of mRNA for voltage-dependent and inward-rectifying K channels in GH3/B6 cells and rat pituitary. J Neuroendocrinol. 2000;12:263-72 pubmed
    ..2, Kv1.4, Kv1.5, Kv2.1, Kv3.2, Kv4.1, Kv5.1; six EAG: eag1, erg1, erg2, elk1-elk3; three KCNQ: KCNQ1-KCNQ3) and for five different Kir channel alpha-subunits (Kir1.1, Kir2.3, Kir3...
  9. Winston N, Johnson M, McConnell J, Cook D, Day M. Expression and role of the ether-à-go-go-related (MERG1A) potassium-channel protein during preimplantation mouse development. Biol Reprod. 2004;70:1070-9 pubmed
    ..In the present study, we investigated the pattern of expression of the mouse ether-à-go-go-related (KCNH2; MERG1A) potassium channel during mouse embryogenic development...

More Information


  1. Muhlbauer E, Bazwinsky I, Wolgast S, Klemenz A, Peschke E. Circadian changes of ether-a-go-go-related-gene (Erg) potassium channel transcripts in the rat pancreas and beta-cell. Cell Mol Life Sci. 2007;64:768-80 pubmed
    ..was used to monitor the circadian expression of ether-a-go-go-related gene (Erg) potassium channel isoforms and Erg1 splice variants...
  2. Kagan A, Melman Y, Krumerman A, McDonald T. 14-3-3 amplifies and prolongs adrenergic stimulation of HERG K+ channel activity. EMBO J. 2002;21:1889-98 pubmed
    ..The net result is a prolongation of the effect of adrenergic stimulation upon HERG activity. Thus, 14-3-3 interactions with HERG may provide a unique mechanism for plasticity in the control of membrane excitability and cardiac rhythm. ..
  3. Cayabyab F, Schlichter L. Regulation of an ERG K+ current by Src tyrosine kinase. J Biol Chem. 2002;277:13673-81 pubmed
    ..In this first report of regulation by tyrosine phosphorylation we show that MLS-9 cells express transcripts for r-erg1 (rat homologue of HERG) and r-erg2, and an immunoreactive doublet was identified using an anti-HERG antibody...
  4. Phan K, Ng C, David E, Shishmarev D, Kuchel P, Vandenberg J, et al. The S1 helix critically regulates the finely tuned gating of Kv11.1 channels. J Biol Chem. 2017;292:7688-7705 pubmed publisher
    ..1 channels in cardiac myocytes, as well as the current passed in response to premature depolarizations that normally helps protect against the formation of ectopic beats. ..
  5. Lamothe S, Zhang S. The serum- and glucocorticoid-inducible kinases SGK1 and SGK3 regulate hERG channel expression via ubiquitin ligase Nedd4-2 and GTPase Rab11. J Biol Chem. 2013;288:15075-84 pubmed publisher
    ..These results show that SGK enhances the expression level of mature hERG channels by inhibiting Nedd4-2 as well as by promoting Rab11-mediated hERG recycling. ..
  6. Guo J, Wang T, Li X, Shallow H, Yang T, Li W, et al. Cell surface expression of human ether-a-go-go-related gene (hERG) channels is regulated by caveolin-3 protein via the ubiquitin ligase Nedd4-2. J Biol Chem. 2012;287:33132-41 pubmed
    ..Our data demonstrate that hERG expression in the plasma membrane is regulated by Cav3 via Nedd4-2. These findings extend our understanding of the regulation of hERG channels and cardiac electrophysiology. ..
  7. Gianulis E, Trudeau M. Rescue of aberrant gating by a genetically encoded PAS (Per-Arnt-Sim) domain in several long QT syndrome mutant human ether-á-go-go-related gene potassium channels. J Biol Chem. 2011;286:22160-9 pubmed publisher
  8. Wimmers S, Wulfsen I, Bauer C, Schwarz J. Erg1, erg2 and erg3 K channel subunits are able to form heteromultimers. Pflugers Arch. 2001;441:450-5 pubmed
    ..After co-expression of these mutants with the wild-type erg1, erg2, or erg3 in Chinese hamster ovary (CHO) cells no erg currents could be detected...
  9. Smith P, Baukrowitz T, Yellen G. The inward rectification mechanism of the HERG cardiac potassium channel. Nature. 1996;379:833-6 pubmed
    ..The characteristics of this gating suggest a specific role for this channel in the normal suppression of arrhythmias. ..
  10. Kupershmidt S, Yang I, Hayashi K, Wei J, Chanthaphaychith S, Petersen C, et al. The IKr drug response is modulated by KCR1 in transfected cardiac and noncardiac cell lines. FASEB J. 2003;17:2263-5 pubmed
    ..We propose that KCR1, when coupled to HERG, may limit the sensitivity of HERG to proarrhythmic drug blockade and may be a rational target for modifying the proarrhythmic effects of otherwise clinically useful compounds. ..
  11. Li Q, Ng H, Yoon H, Kang C. Insight into the molecular interaction between the cyclic nucleotide-binding homology domain and the eag domain of the hERG channel. FEBS Lett. 2014;588:2782-8 pubmed publisher
    ..Residues from the CNBHD that were affected by the interaction with the eag domain were also identified. A R56Q mutation does not cause major structural changes in the eag domain and showed reduced interaction with the CNBHD. ..
  12. Carrisoza R, Salvador C, Bobadilla N, Trujillo J, Escobar L. Expression and immunolocalization of ERG1 potassium channels in the rat kidney. Histochem Cell Biol. 2010;133:189-99 pubmed publisher
    ..In our immunohistochemical experiments, we could not differentiate the ERG1 isoforms because we used an antibody against a carboxy-terminal epitope...
  13. Wymore R, Gintant G, Wymore R, Dixon J, McKinnon D, Cohen I. Tissue and species distribution of mRNA for the IKr-like K+ channel, erg. Circ Res. 1997;80:261-8 pubmed
    ..These results strengthen the link between erg and the native IKr in heart and suggest that erg may play an important role in other noncardiac tissues...
  14. Lees Miller J, Kondo C, Wang L, Duff H. Electrophysiological characterization of an alternatively processed ERG K+ channel in mouse and human hearts. Circ Res. 1997;81:719-26 pubmed
    ..We have identified ERG B, an alternatively processed isoform of the ERG gene, expressed selectively in heart and with electrophysiological characteristics similar to those of native cardiac IKr. ..
  15. London B, Trudeau M, Newton K, Beyer A, Copeland N, Gilbert D, et al. Two isoforms of the mouse ether-a-go-go-related gene coassemble to form channels with properties similar to the rapidly activating component of the cardiac delayed rectifier K+ current. Circ Res. 1997;81:870-8 pubmed
    ..This N-terminal Erg isoform may determine the properties of IKr and contribute to the pathogenesis of LQTS. ..
  16. Kirchberger N, Wulfsen I, Schwarz J, Bauer C. Effects of TRH on heteromeric rat erg1a/1b K+ channels are dominated by the rerg1b subunit. J Physiol. 2006;571:27-42 pubmed
    ..Application of TRH induced a reduction of maximal erg conductance for all tested erg1 currents without effects on the voltage dependence of steady-state inactivation...
  17. Massaeli H, Sun T, Li X, Shallow H, Wu J, Xu J, et al. Involvement of caveolin in low K+-induced endocytic degradation of cell-surface human ether-a-go-go-related gene (hERG) channels. J Biol Chem. 2010;285:27259-64 pubmed publisher
    ..Our data indicate that a caveolin-dependent endocytic route is involved in low K(+)(o)-induced degradation of mature hERG channels. ..
  18. Albesa M, Grilo L, Gavillet B, Abriel H. Nedd4-2-dependent ubiquitylation and regulation of the cardiac potassium channel hERG1. J Mol Cell Cardiol. 2011;51:90-8 pubmed publisher
    ..Mutations in its gene, KCNH2, can lead to defects in the biosynthesis and maturation of the channel, resulting in congenital long QT syndrome (..
  19. Li X, Xu J, Li M. The human delta1261 mutation of the HERG potassium channel results in a truncated protein that contains a subunit interaction domain and decreases the channel expression. J Biol Chem. 1997;272:705-8 pubmed
    ..Such a decrease of potassium channel expression can contribute to the longer QT intervals observed in the patients with the HERG mutation. ..
  20. Roder K, Werdich A, Li W, Liu M, Kim T, Organ Darling L, et al. RING finger protein RNF207, a novel regulator of cardiac excitation. J Biol Chem. 2014;289:33730-40 pubmed publisher
    ..Taken together, the evidence is strong that RNF207 is an important regulator of action potential duration, likely via effects on HERG trafficking and localization in a heat shock protein-dependent manner. ..
  21. Yu Z, Liu J, van Veldhoven J, Ijzerman A, Schalij M, Pijnappels D, et al. Allosteric Modulation of Kv11.1 (hERG) Channels Protects Against Drug-Induced Ventricular Arrhythmias. Circ Arrhythm Electrophysiol. 2016;9:e003439 pubmed publisher
    ..1 blockers via pharmacological combination therapy. ..
  22. Hirdes W, Schweizer M, Schuricht K, Guddat S, Wulfsen I, Bauer C, et al. Fast erg K+ currents in rat embryonic serotonergic neurones. J Physiol. 2005;564:33-49 pubmed
    ..gene (erg) channels form one subfamily of the ether-á-go-go (EAG) K(+) channels and all three erg channels (erg1-3) are expressed in the brain...
  23. Eckey K, Strutz Seebohm N, Katz G, Fuhrmann G, Henrion U, Pott L, et al. Modulation of human ether a gogo related channels by CASQ2 contributes to etiology of catecholaminergic polymorphic ventricular tachycardia (CPVT). Cell Physiol Biochem. 2010;26:503-12 pubmed publisher
    ..These in vitro and in silico data suggest a regulatory role of CASQ2 on cytosolic Ca(2+) and hERG channels which may contribute to the etiology of CPVT. ..
  24. Curran M, Splawski I, Timothy K, Vincent G, Green E, Keating M. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell. 1995;80:795-803 pubmed
    ..In one kindred, the mutation arose de novo. Northern blot analyses show that HERG is strongly expressed in the heart. These data indicate that HERG is LQT2 and suggest a likely cellular mechanism for torsade de pointes. ..
  25. Sanguinetti M, Jiang C, Curran M, Keating M. A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel. Cell. 1995;81:299-307 pubmed
    ..Since block of IKr is a known mechanism for drug-induced cardiac arrhythmias, the finding that HERG encodes IKr channels provides a mechanistic link between certain forms of inherited and acquired LQT. ..
  26. Tester D, Will M, Haglund C, Ackerman M. Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing. Heart Rhythm. 2005;2:507-17 pubmed
    ..A cardiac channel gene screen for LQTS-causing mutations in KCNQ1 (LQT1), KCNH2 (LQT2), SCN5A (LQT3), KCNE1 (LQT5), and KCNE2 (LQT6) was performed for 541 consecutive, unrelated patients (358 ..
  27. Ohya S, Asakura K, Muraki K, Watanabe M, Imaizumi Y. Molecular and functional characterization of ERG, KCNQ, and KCNE subtypes in rat stomach smooth muscle. Am J Physiol Gastrointest Liver Physiol. 2002;282:G277-87 pubmed
    ..The region-qualified multicell RT-PCR showed that ERG1/KCNE2 transcripts were expressed in rat stomach fundus and antrum SMCs and that KCNQ1/KCNE1 transcripts were ..
  28. Bauer C, Engeland B, Wulfsen I, Ludwig J, Pongs O, Schwarz J. RERG is a molecular correlate of the inward-rectifying K current in clonal rat pituitary cells. Receptors Channels. 1998;6:19-29 pubmed
    ..b>RERG has a 96% amino acid identify to HERG...
  29. Jeron A, Mitchell G, Zhou J, Murata M, London B, Buckett P, et al. Inducible polymorphic ventricular tachyarrhythmias in a transgenic mouse model with a long Q-T phenotype. Am J Physiol Heart Circ Physiol. 2000;278:H1891-8 pubmed