HERG

Summary

Gene Symbol: HERG
Description: potassium voltage-gated channel subfamily H member 2
Alias: ERG-1, ERG1, H-ERG, HERG, HERG1, Kv11.1, LQT2, SQT1, potassium voltage-gated channel subfamily H member 2, eag homolog, eag-related protein 1, ether-a-go-go-related gene potassium channel 1, ether-a-go-go-related potassium channel protein, 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, voltage-gated potassium channel subunit Kv11.1
Species: human
Products:     HERG

Top Publications

  1. Bai Y, Wang J, Shan H, Lu Y, Zhang Y, Luo X, et al. Sphingolipid metabolite ceramide causes metabolic perturbation contributing to HERG K+ channel dysfunction. Cell Physiol Biochem. 2007;20:429-40 pubmed
    ..how ceramide is involved in modulating cardiac repolarization, we performed whole-cell patch-clamp studies on HERG current (I(HERG)), a critical determinant of cardiac repolarization, expressed in HEK293 cells...
  2. Van Slyke A, Rezazadeh S, Snopkowski M, Shi P, Allard C, Claydon T. Mutations within the S4-S5 linker alter voltage sensor constraints in hERG K+ channels. Biophys J. 2010;99:2841-52 pubmed publisher
    Human ether-a-go-go related gene (hERG) channel gating is associated with slow activation, yet the mechanistic basis for this is unclear...
  3. Sun Y, Quan X, Fromme S, Cox R, Zhang P, Zhang L, et al. A novel mutation in the KCNH2 gene associated with short QT syndrome. J Mol Cell Cardiol. 2011;50:433-41 pubmed publisher
    A gain of function mutation N588K in the KCNH2 gene that encodes HERG channels has been shown to underlie the SQT1 form of short QT syndrome (SQTS)...
  4. Balijepalli R, Delisle B, Balijepalli S, Foell J, Slind J, Kamp T, et al. Kv11.1 (ERG1) K+ channels localize in cholesterol and sphingolipid enriched membranes and are modulated by membrane cholesterol. Channels (Austin). 2007;1:263-72 pubmed
    ..We determined the membrane localization of human Kv11.1 (hERG1) alpha-subunit protein, which underlies the rapidly activating, delayed rectifier K(+) current (I(Kr)) in the ..
  5. 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
  6. 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
    Congenital long QT syndrome 2 (LQT2) is caused by loss-of-function mutations in the human ether-á-go-go-related gene (hERG) voltage-gated potassium (K(+)) channel...
  7. Pietilä E, Fodstad H, Niskasaari E, Laitinen P P, Swan H, Savolainen M, et al. Association between HERG K897T polymorphism and QT interval in middle-aged Finnish women. J Am Coll Cardiol. 2002;40:511-4 pubmed
    The aim of this study was to test whether a recently reported polymorphism in the HERG gene coding for the rapidly activating delayed rectifier K+ channel has influence on myocardial repolarization...
  8. Chugh S, Senashova O, Watts A, Tran P, Zhou Z, Gong Q, et al. Postmortem molecular screening in unexplained sudden death. J Am Coll Cardiol. 2004;43:1625-9 pubmed
    ..We performed analysis of KCNQ1 (KVLQT1), KCNH2 (HERG), SCN5A, KCNE1, and KCNE2 defects in a subgroup of 12 adult subjects with unexplained sudden death, derived from a ..
  9. Christé G, Thériault O, Chahine M, Millat G, Rodriguez Lafrasse C, Rousson R, et al. A new C-terminal hERG mutation A915fs+47X associated with symptomatic LQT2 and auditory-trigger syncope. Heart Rhythm. 2008;5:1577-86 pubmed publisher
    A novel mutation of hERG (A915fs+47X) was discovered in a 32-year-old woman with torsades de pointes, long QTc interval (515 ms), and syncope upon auditory trigger...

More Information

Publications149 found, 100 shown here

  1. Harley C, Jesus C, Carvalho R, Brito R, MORAIS CABRAL J. Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel. PLoS ONE. 2012;7:e32654 pubmed publisher
    Inherited human long-QT2 syndrome (LQTS) results from mutations in the gene encoding the HERG channel...
  2. Arnestad M, Crotti L, Rognum T, Insolia R, Pedrazzini M, Ferrandi C, et al. Prevalence of long-QT syndrome gene variants in sudden infant death syndrome. Circulation. 2007;115:361-7 pubmed
  3. Farrelly A, Ro S, Callaghan B, Khoyi M, Fleming N, Horowitz B, et al. Expression and function of KCNH2 (HERG) in the human jejunum. Am J Physiol Gastrointest Liver Physiol. 2003;284:G883-95 pubmed publisher
    ..Messages for two different variants of the KCNH2 gene were detected: KCNH2 V1 human ERG (HERG) (28) and KCNH2 V2 (HERG(USO)) (13). The amount of V2 message was greater than V1 in both human jejunum and brain...
  4. Gong Q, Zhang L, Vincent G, Horne B, Zhou Z. Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome. Circulation. 2007;116:17-24 pubmed
    Long-QT syndrome type 2 (LQT2) is caused by mutations in the human ether-a-go-go-related gene (hERG). More than 30% of the LQT2 mutations result in premature termination codons...
  5. Dolderer J, Schuldes H, Bockhorn H, Altmannsberger M, Lambers C, von Zabern D, et al. HERG1 gene expression as a specific tumor marker in colorectal tissues. Eur J Surg Oncol. 2010;36:72-7 pubmed publisher
    ..cytokeratin-19 (CK19) and cytokeratin-20 (CK20) to that of a novel marker, the human ether-a-go-go-related gene (HERG1) K(+) channel, a suggested regulator of tumor cell proliferation...
  6. Tseng G, Sonawane K, Korolkova Y, Zhang M, Liu J, Grishin E, et al. Probing the outer mouth structure of the HERG channel with peptide toxin footprinting and molecular modeling. Biophys J. 2007;92:3524-40 pubmed
    Previous studies have shown that the unusually long S5-P linker lining human ether a-go-go related gene's (hERG's) outer vestibule is critical for its channel function: point mutations at high-impact positions here can interfere with the ..
  7. Jones E, Roti Roti E, Wang J, Delfosse S, Robertson G. Cardiac IKr channels minimally comprise hERG 1a and 1b subunits. J Biol Chem. 2004;279:44690-4 pubmed
    ..suggest native cardiac IKr channels are composed of alpha subunits encoded solely by the 1a transcript of the ERG1 gene...
  8. Akhavan A, Atanasiu R, Shrier A. Identification of a COOH-terminal segment involved in maturation and stability of human ether-a-go-go-related gene potassium channels. J Biol Chem. 2003;278:40105-12 pubmed
    Mutations in the potassium channel encoded by the human ether-a-go-go-related gene (HERG) have been linked to the congenital long QT syndrome (LQTS), a cardiac disease associated with an increased preponderance of ventricular arrhythmias ..
  9. Yang H, Sun C, Cui C, Xue X, Zhang A, Li H, et al. HERG-F463L potassium channels linked to long QT syndrome reduce I(Kr) current by a trafficking-deficient mechanism. Clin Exp Pharmacol Physiol. 2009;36:822-7 pubmed publisher
    ..A mutant F463L HERG channel was expressed in HEK293 cells using a lipofectamine method...
  10. Afrasiabi E, Hietamäki M, Viitanen T, Sukumaran P, Bergelin N, Törnquist K. Expression and significance of HERG (KCNH2) potassium channels in the regulation of MDA-MB-435S melanoma cell proliferation and migration. Cell Signal. 2010;22:57-64 pubmed publisher
    The human ether-a-go-go related gene (HERG) potassium channel has elicited intense scientific interest due to its role in cardiac repolarization and its association with arrhythmia and sudden cardiac death...
  11. Kolbe K, Schönherr R, Gessner G, Sahoo N, Hoshi T, Heinemann S. Cysteine 723 in the C-linker segment confers oxidative inhibition of hERG1 potassium channels. J Physiol. 2010;588:2999-3009 pubmed publisher
    ..b>hERG1 (KCNH2) potassium channels terminate the cardiac action potential and malfunction can lead to long-QT syndrome and ..
  12. Stansfeld P, Gedeck P, Gosling M, Cox B, Mitcheson J, Sutcliffe M. Drug block of the hERG potassium channel: insight from modeling. Proteins. 2007;68:568-80 pubmed
    Many commonly used, structurally diverse, drugs block the human ether-a-go-go-related gene (hERG) K(+) channel to cause acquired long QT syndrome, which can lead to sudden death via lethal cardiac arrhythmias...
  13. Guasti L, Crociani O, Redaelli E, Pillozzi S, Polvani S, Masselli M, et al. Identification of a posttranslational mechanism for the regulation of hERG1 K+ channel expression and hERG1 current density in tumor cells. Mol Cell Biol. 2008;28:5043-60 pubmed publisher
    ..K(+) channels that belong to the human ether-a-go-go-related gene 1 (herg1) family are frequently misexpressed in cancer cells compared to their healthy counterparts...
  14. Tan P, Perry M, Ng C, Vandenberg J, Hill A. Voltage-sensing domain mode shift is coupled to the activation gate by the N-terminal tail of hERG channels. J Gen Physiol. 2012;140:293-306 pubmed publisher
    Human ether-a-go-go-related gene (hERG) potassium channels exhibit unique gating kinetics characterized by unusually slow activation and deactivation...
  15. Paulussen A, Raes A, Matthijs G, Snyders D, Cohen N, Aerssens J. A novel mutation (T65P) in the PAS domain of the human potassium channel HERG results in the long QT syndrome by trafficking deficiency. J Biol Chem. 2002;277:48610-6 pubmed
    ..Mutations in the HERG (human ether-à-go-go-related gene KCNH2 can cause a reduction in I(Kr), one of the currents responsible for ..
  16. Wang J, Wang H, Zhang Y, Gao H, Nattel S, Wang Z. Impairment of HERG K(+) channel function by tumor necrosis factor-alpha: role of reactive oxygen species as a mediator. J Biol Chem. 2004;279:13289-92 pubmed
    ..We studied the effects of TNF-alpha on cloned HERG K(+) channel (human ether-a-go-go-related gene) in HEK293 cells and native I(Kr) in canine cardiomyocytes with ..
  17. Zhang D, Wang Y, Lau C, Tse H, Li G. Both EGFR kinase and Src-related tyrosine kinases regulate human ether-à-go-go-related gene potassium channels. Cell Signal. 2008;20:1815-21 pubmed publisher
    Human ether-à-go-go-related gene (hERG or Kv11.1) encodes the rapidly activated delayed rectifier K(+) current (I(Kr)) in the human heart. Potential regulation of hERG channel by protein tyrosine kinases (PTKs) is not understood...
  18. Huffaker S, Chen J, Nicodemus K, Sambataro F, Yang F, Mattay V, et al. A primate-specific, brain isoform of KCNH2 affects cortical physiology, cognition, neuronal repolarization and risk of schizophrenia. Nat Med. 2009;15:509-18 pubmed publisher
    ..These results identify a previously undescribed KCNH2 channel isoform involved in cortical physiology, cognition and psychosis, providing a potential new therapeutic drug target. ..
  19. Marjamaa A, Newton Cheh C, Porthan K, Reunanen A, Lahermo P, Väänänen H, et al. Common candidate gene variants are associated with QT interval duration in the general population. J Intern Med. 2009;265:448-58 pubmed publisher
    ..In addition, we robustly confirm the previous findings that three independent KCNH2 and NOSA1P variants are associated with adjusted QT interval. ..
  20. Piper D, Hinz W, Tallurri C, Sanguinetti M, Tristani Firouzi M. Regional specificity of human ether-a'-go-go-related gene channel activation and inactivation gating. J Biol Chem. 2005;280:7206-17 pubmed
    ..produce inward rectification of the current-voltage relationship for human ether-a'-go-go-related gene (hERG) channels...
  21. Zhang L, Vincent G, Baralle M, Baralle F, Anson B, Benson D, et al. An intronic mutation causes long QT syndrome. J Am Coll Cardiol. 2004;44:1283-91 pubmed
    ..422 +/- 20 ms, Ser QTc-max 508 +/- 48 ms versus 448 +/- 10 ms, Ex QTc-max 513 +/- 54 ms versus 444 +/- 11 ms, and LQT2 T waves in 87% versus 0%. Linkage analysis demonstrated a logarithm of odds score of 10.22...
  22. de la Pena P, Alonso Ron C, Machin A, Fernández Trillo J, Carretero L, Dominguez P, et al. 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;286:19065-75 pubmed publisher
    Potassium channels encoded by the human ether-à-go-go-related gene (hERG) contribute to cardiac repolarization as a result of their characteristic gating properties. The hERG channel N terminus acts as a crucial determinant in gating...
  23. Miranda P, Manso D, Barros F, Carretero L, Hughes T, Alonso Ron C, et al. FRET with multiply labeled HERG K(+) channels as a reporter of the in vivo coarse architecture of the cytoplasmic domains. Biochim Biophys Acta. 2008;1783:1681-99 pubmed publisher
    The intracellular N-terminus of human ether-a-go-go-related gene (HERG) potassium channels constitutes a key determinant of activation and deactivation characteristics and is necessary for hormone-induced modifications of gating ..
  24. Ramström C, Chapman H, Viitanen T, Afrasiabi E, Fox H, Kivelä J, et al. Regulation of HERG (KCNH2) potassium channel surface expression by diacylglycerol. Cell Mol Life Sci. 2010;67:157-69 pubmed publisher
    The HERG (KCNH2) channel is a voltage-sensitive potassium channel mainly expressed in cardiac tissue, but has also been identified in other tissues like neuronal and smooth muscle tissue, and in various tumours and tumour cell lines...
  25. 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
    The hERG (human ether-a-go-go-related gene) encodes the ? subunit of the rapidly activating delayed rectifier potassium channel (IKr)...
  26. Munoz C, Saxena A, Pakladok T, Bogatikov E, Wilmes J, Seebohm G, et al. Stimulation of HERG channel activity by ?-catenin. PLoS ONE. 2012;7:e43353 pubmed publisher
    ..The present study explored the possibility that ß-catenin participates in the regulation of the HERG K(+) channel...
  27. Luo X, Xiao J, Lin H, Lu Y, Yang B, Wang Z. Genomic structure, transcriptional control, and tissue distribution of HERG1 and KCNQ1 genes. Am J Physiol Heart Circ Physiol. 2008;294:H1371-80 pubmed publisher
    The long QT syndrome genes human ether-a-go-go-related gene (HERG1) and voltage-gated K+ channel, KQT-like subfamily, member 1, gene (KCNQ1), encoding K+ channels critical to the repolarization rate and repolarization reserve in cardiac ..
  28. Jongbloed R, Marcelis C, Velter C, Doevendans P, Geraedts J, Smeets H. DHPLC analysis of potassium ion channel genes in congenital long QT syndrome. Hum Mutat. 2002;20:382-91 pubmed
    ..Two potassium-channel genes, KCNQ1 (LQT1) and KCNH2 (LQT2 or HERG), are frequently involved in LQTS. Potassium-channel defects account for approximately 50-60% of LQTS...
  29. Lupoglazoff J, Denjoy I, Villain E, Fressart V, Simon F, Bozio A, et al. Long QT syndrome in neonates: conduction disorders associated with HERG mutations and sinus bradycardia with KCNQ1 mutations. J Am Coll Cardiol. 2004;43:826-30 pubmed
    ..Conduction disorders were associated with LQT2, whereas sinus bradycardia was associated with LQT1...
  30. Wang Y, Shi C, Dong F, Sheng J, Xu Y. Inhibition of the rapid component of the delayed rectifier potassium current in ventricular myocytes by angiotensin II via the AT1 receptor. Br J Pharmacol. 2008;154:429-39 pubmed publisher
    ..cardiocytes and in human embryonic kidney (HEK) 293 cells, co-transfected with human ether-a-go-go-related gene (hERG) encoding the alpha-subunit of I(Kr) and the human Ang II type 1 (AT(1)) receptor gene...
  31. Anderson C, Delisle B, Anson B, Kilby J, Will M, Tester D, et al. Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism. Circulation. 2006;113:365-73 pubmed
    ..Type 2 congenital long-QT syndrome (LQT2) results from KCNH2 mutations that cause loss of Kv11.1 channel function...
  32. Gong Q, Anderson C, January C, Zhou Z. Role of glycosylation in cell surface expression and stability of HERG potassium channels. Am J Physiol Heart Circ Physiol. 2002;283:H77-84 pubmed
    The human ether-à-go-go-related gene (HERG) encodes the pore-forming subunit of the rapidly activating delayed rectifier potassium channel in the heart. We previously showed that HERG channel protein is modified by N-linked glycosylation...
  33. Newton Cheh C, Guo C, Larson M, Musone S, Surti A, Camargo A, et al. Common genetic variation in KCNH2 is associated with QT interval duration: the Framingham Heart Study. Circulation. 2007;116:1128-36 pubmed
    ..Mutations in the KCNH2 gene encoding the HERG potassium channel cause 30% of long-QT syndrome, and binding to this channel leads to drug-induced QT prolongation...
  34. Perrin M, Subbiah R, Vandenberg J, Hill A. Human ether-a-go-go related gene (hERG) K+ channels: function and dysfunction. Prog Biophys Mol Biol. 2008;98:137-48 pubmed publisher
    The human Ether-a-go-go Related Gene (hERG) potassium channel plays a central role in regulating cardiac excitability and maintenance of normal cardiac rhythm...
  35. Zhang Y, Colenso C, Sessions R, Dempsey C, Hancox J. The hERG K(+) channel S4 domain L532P mutation: characterization at 37°C. Biochim Biophys Acta. 2011;1808:2477-87 pubmed publisher
    b>hERG (human Ether-à-go-go Related Gene) is responsible for ion channels mediating rapid delayed rectifier potassium current, I(Kr), which is key to cardiac action potential repolarization...
  36. Gomez Varela D, Barros F, Viloria C, Giraldez T, Manso D, Dupuy S, et al. Relevance of the proximal domain in the amino-terminus of HERG channels for regulation by a phospholipase C-coupled hormone receptor. FEBS Lett. 2003;535:125-30 pubmed
    ..Xenopus oocytes co-expressing thyrotropin-releasing hormone (TRH) receptors and human ether-a-go-go-related gene (HERG) K+ channel variants carrying different amino-terminal modifications to check the relevance of the proximal domain ..
  37. Gouas L, Nicaud V, Berthet M, Forhan A, Tiret L, Balkau B, et al. Association of KCNQ1, KCNE1, KCNH2 and SCN5A polymorphisms with QTc interval length in a healthy population. Eur J Hum Genet. 2005;13:1213-22 pubmed
  38. Chapman H, Ramström C, Korhonen L, Laine M, Wann K, Lindholm D, et al. Downregulation of the HERG (KCNH2) K(+) channel by ceramide: evidence for ubiquitin-mediated lysosomal degradation. J Cell Sci. 2005;118:5325-34 pubmed
    The HERG (KCNH2) potassium channel underlies the rapid component of the delayed rectifier current (I(kr)), a current contributing to the repolarisation of the cardiac action potential...
  39. Clarke C, Hill A, Zhao J, Kondo M, Subbiah R, Campbell T, et al. Effect of S5P alpha-helix charge mutants on inactivation of hERG K+ channels. J Physiol. 2006;573:291-304 pubmed
    ..The human ether-à-go-go related gene (hERG) K(+) channel has been of significant interest because loss of function in the hERG channel is associated with a ..
  40. Dennis A, Nassal D, Deschenes I, Thomas D, Ficker E. Antidepressant-induced ubiquitination and degradation of the cardiac potassium channel hERG. J Biol Chem. 2011;286:34413-25 pubmed publisher
    ..acLQTS is often caused by direct block of the cardiac potassium current I(Kr)/hERG, which is crucial for terminal repolarization in human heart...
  41. Sun Z, Milos P, Thompson J, Lloyd D, Mank Seymour A, Richmond J, et al. Role of a KCNH2 polymorphism (R1047 L) in dofetilide-induced Torsades de Pointes. J Mol Cell Cardiol. 2004;37:1031-9 pubmed
    ..A few cases appear to be devoid of these factors. To determine what role genetic variation in the hERG gene plays in drug-induced arrhythmias, we screened DNA samples collected from 105 atrial-fibrillation patients ..
  42. Torres A, Bansal P, Sunde M, Clarke C, Bursill J, Smith D, et al. Structure of the HERG K+ channel S5P extracellular linker: role of an amphipathic alpha-helix in C-type inactivation. J Biol Chem. 2003;278:42136-48 pubmed
    The HERG K+ channel has very unusual kinetic behavior that includes slow activation but rapid inactivation. These features are critical for normal cardiac repolarization as well as in preventing lethal ventricular arrhythmias...
  43. Brugada R, Hong K, Dumaine R, Cordeiro J, Gaita F, Borggrefe M, et al. Sudden death associated with short-QT syndrome linked to mutations in HERG. Circulation. 2004;109:30-5 pubmed
    ..mutations resulting in the same amino acid change (N588K) in the S5-P loop region of the cardiac IKr channel HERG (KCNH2)...
  44. Rossenbacker T, Mubagwa K, Jongbloed R, Vereecke J, Devriendt K, Gewillig M, et al. Novel mutation in the Per-Arnt-Sim domain of KCNH2 causes a malignant form of long-QT syndrome. Circulation. 2005;111:961-8 pubmed
    It has been proposed that the highest risk for cardiac events in patients with long-QT syndrome subtype 2 (LQT2) is related to mutations in the pore region of the KCNH2 channel...
  45. Sauer A, Moss A, McNitt S, Peterson D, Zareba W, Robinson J, et al. Long QT syndrome in adults. J Am Coll Cardiol. 2007;49:329-37 pubmed
    ..Female gender, corrected QT (QTc) interval, LQT2 genotype, and frequency of cardiac events before age 18 years were associated with increased risk of having any ..
  46. Perry M, Sachse F, Sanguinetti M. 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;104:13827-32 pubmed
    Activation of human ether-a-go-go-related gene 1 (hERG1) K(+) channels mediates cardiac action potential repolarization...
  47. Piper D, Rupp J, Sachse F, Sanguinetti M, Tristani Firouzi M. Cooperative interactions between R531 and acidic residues in the voltage sensing module of hERG1 channels. Cell Physiol Biochem. 2008;21:37-46 pubmed publisher
    b>HERG1 K(+) channels are critical for modulating the duration of the cardiac action potential...
  48. Teng G, Zhao X, Lees Miller J, Quinn F, Li P, Rancourt D, et al. Homozygous missense N629D hERG (KCNH2) potassium channel mutation causes developmental defects in the right ventricle and its outflow tract and embryonic lethality. Circ Res. 2008;103:1483-91 pubmed publisher
    Loss-of-function mutations in the human ERG1 potassium channel (hERG1) frequently underlie the long QT2 (LQT2) syndrome...
  49. Brelidze T, Carlson A, Zagotta W. Absence of direct cyclic nucleotide modulation of mEAG1 and hERG1 channels revealed with fluorescence and electrophysiological methods. J Biol Chem. 2009;284:27989-97 pubmed publisher
    ..Here we explored cyclic nucleotide binding and modulation of mEAG1 and hERG1 channels with fluorescence and electrophysiology...
  50. Lian J, Huang N, Zhou J, Ge S, Huang X, Huo J, et al. Novel characteristics of a trafficking-defective G572R-hERG channel linked to hereditary long QT syndrome. Can J Cardiol. 2010;26:417-22 pubmed
    ..Type 2 congenital long QT syndrome (LQT2) results from KCNH2 or hERG gene mutations. hERG encodes the K(v)11...
  51. Muskett F, Thouta S, Thomson S, Bowen A, Stansfeld P, Mitcheson J. Mechanistic insight into human ether-à-go-go-related gene (hERG) K+ channel deactivation gating from the solution structure of the EAG domain. J Biol Chem. 2011;286:6184-91 pubmed publisher
    Human ether-à-go-go-related gene (hERG) K(+) channels have a critical role in cardiac repolarization...
  52. Zhang Y, Wang H, Wang J, Han H, Nattel S, Wang Z. Normal function of HERG K+ channels expressed in HEK293 cells requires basal protein kinase B activity. FEBS Lett. 2003;534:125-32 pubmed
    The potential role of protein kinase B (PKB), a serine/threonine protein kinase, in regulating HERG (human ether-a-go-go related gene) K(+) channel function was investigated...
  53. Suzuki T, Takimoto K. Selective expression of HERG and Kv2 channels influences proliferation of uterine cancer cells. Int J Oncol. 2004;25:153-9 pubmed
    ..1 and the silent regulatory subunit for Kv2.1 channels, Kv9.3. Thus, squamous/endometrial cancer cells contain HERG-KCNE channel complexes, whereas Kv2.1-Kv9...
  54. Pellegrino P, Bafunno V, Ieva R, Brunetti N, Mavilio G, Sessa F, et al. A novel mutation in human ether-a-go-go-related gene, alanine to proline at position 490, found in a large family with autosomal dominant long QT syndrome. Am J Cardiol. 2007;99:1737-40 pubmed
    ..In conclusion, the Ala490Pro mutation of the human ether-a-go-go-related gene is a rare, novel mutation that was inherited in this family, leading to Romano-Ward syndrome with complete penetrance. ..
  55. Massaeli H, Guo J, Xu J, Zhang S. Extracellular K+ is a prerequisite for the function and plasma membrane stability of HERG channels. Circ Res. 2010;106:1072-82 pubmed publisher
    The human ether-a-go-go-related gene (HERG) encodes the pore-forming subunits of the rapidly activating delayed rectifier potassium channel (I(Kr)) that is important for cardiac repolarization...
  56. Ganapathi S, Fox T, Kester M, Elmslie K. Ceramide modulates HERG potassium channel gating by translocation into lipid rafts. Am J Physiol Cell Physiol. 2010;299:C74-86 pubmed publisher
    Human ether-à-go-go-related gene (HERG) potassium channels play an important role in cardiac action potential repolarization, and HERG dysfunction can cause cardiac arrhythmias...
  57. Pillozzi S, Arcangeli A. Physical and functional interaction between integrins and hERG1 channels in cancer cells. Adv Exp Med Biol. 2010;674:55-67 pubmed
    ..b>hERG1 channels can induce such diverse effects since they trigger and modulate intracellular signaling cascades...
  58. Bauer C, Wulfsen I, Schafer R, Glassmeier G, Wimmers S, Flitsch J, et al. HERG K(+) currents in human prolactin-secreting adenoma cells. Pflugers Arch. 2003;445:589-600 pubmed
    ..Transcripts for HERG1 were present in all adenomas and although transcripts for HERG2 and HERG3 were also detected, their expression ..
  59. Sasano T, Ueda K, Orikabe M, Hirano Y, Kawano S, Yasunami M, et al. Novel C-terminus frameshift mutation, 1122fs/147, of HERG in LQT2: additional amino acids generated by frameshift cause accelerated inactivation. J Mol Cell Cardiol. 2004;37:1205-11 pubmed
    The function of the C-terminus region of the human ether-a-go-go related gene (HERG) has not been well characterized except for its involvement in trafficking...
  60. Zhao J, Hill A, Varghese A, Cooper A, Swan H, Laitinen Forsblom P, et al. 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;20:923-30 pubmed publisher
    Mutations in the pore domain of the human ether-a-go-go-related gene (hERG) potassium channel are associated with higher risk of sudden death. However, in many kindreds clinical presentation is variable, making it hard to predict risk...
  61. Li Q, Gayen S, Chen A, Huang Q, Raida M, Kang C. NMR solution structure of the N-terminal domain of hERG and its interaction with the S4-S5 linker. Biochem Biophys Res Commun. 2010;403:126-32 pubmed publisher
    The human Ether-à-go-go Related Gene (hERG) potassium channel mediates the rapid delayed rectifier current (IKr) in the cardiac action potential...
  62. Wu Z, Yu D, Soong T, Dawe G, Bian J. Progesterone impairs human ether-a-go-go-related gene (HERG) trafficking by disruption of intracellular cholesterol homeostasis. J Biol Chem. 2011;286:22186-94 pubmed publisher
    ..implies that higher levels of progesterone may regulate the function of the human ether-a-go-go-related gene (HERG) potassium channel, a key ion channel responsible for controlling the length of QT intervals...
  63. Zheng F, Li H, Du W, Huang S. Role of hERG1 K(+) channels in leukemia cells as a positive regulator in SDF-1a-induced proliferation. Hematology. 2011;16:177-84 pubmed publisher
    Previous work from our laboratory has confirmed that human ether-à-go-go-related gene 1 (hERG1) K(+) channels are constitutively expressed in leukemia cells and enhanced cell proliferation...
  64. Peterson L, Eskew J, Vielhauer G, Blagg B. The hERG channel is dependent upon the Hsp90? isoform for maturation and trafficking. Mol Pharm. 2012;9:1841-6 pubmed publisher
    ..However, some toxicological detriments have arisen, such as cardiotoxicity resulting from hERG inhibition following the administration of Hsp90 inhibitors...
  65. 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 females, ..
  66. Wang D, Hill A, Mann S, Tan P, Vandenberg J. Mapping the sequence of conformational changes underlying selectivity filter gating in the K(v)11.1 potassium channel. Nat Struct Mol Biol. 2011;18:35-41 pubmed publisher
    ..We show that closure of the selectivity filter gate in the human K(v)11.1 K(+) channel (also known as hERG, for ether-a-go-go-related gene), a key regulator of the rhythm of the heartbeat, is initiated by K(+) exit, ..
  67. Wang Y, Huang X, Zhou J, Yang X, Li D, Mao H, et al. Trafficking-deficient G572R-hERG and E637K-hERG activate stress and clearance pathways in endoplasmic reticulum. PLoS ONE. 2012;7:e29885 pubmed publisher
    Long QT syndrome type 2 (LQT2) is the second most common type of all long QT syndromes. It is well-known that trafficking deficient mutant human ether-a-go-go-related gene (hERG) proteins are often involved in LQT2...
  68. Guo J, Zhang X, Hu Z, Zhuang Z, Zhu Z, Chen Z, et al. A422T mutation in HERG potassium channel retained in ER is rescurable by pharmacologic or molecular chaperones. Biochem Biophys Res Commun. 2012;422:305-10 pubmed publisher
    In the present study, we characterized biologic and electrophysiologic consequences of A422T mutation in HERG K(+) channel and the role of pharmacologic or molecular chaperons by employing a heterogeneous expression system in HEK 293 ..
  69. Paulussen A, Gilissen R, Armstrong M, Doevendans P, Verhasselt P, Smeets H, et al. Genetic variations of KCNQ1, KCNH2, SCN5A, KCNE1, and KCNE2 in drug-induced long QT syndrome patients. J Mol Med (Berl). 2004;82:182-8 pubmed
    ..We conclude that missense mutations in the examined cLQTS genes explain only a minority of aLQTS cases. ..
  70. Gong Q, Keeney D, Molinari M, Zhou Z. Degradation of trafficking-defective long QT syndrome type II mutant channels by the ubiquitin-proteasome pathway. J Biol Chem. 2005;280:19419-25 pubmed
    Mutations in the human ether-a-go-go-related gene (hERG) cause chromosome 7-linked long QT syndrome type II (LQT2)...
  71. McPate M, Duncan R, Milnes J, Witchel H, Hancox J. The N588K-HERG K+ channel mutation in the 'short QT syndrome': mechanism of gain-in-function determined at 37 degrees C. Biochem Biophys Res Commun. 2005;334:441-9 pubmed
    ..that lead to a single amino-acid change (N588K; asparagine to lysine) in the S5-Pore linker region of the cardiac HERG K(+) channel...
  72. Sanguinetti M. HERG1 channelopathies. Pflugers Arch. 2010;460:265-76 pubmed publisher
    Human ether a go-go-related gene type 1 (hERG1) K+ channels conduct the rapid delayed rectifier K+ current and mediate action potential repolarization in the heart...
  73. Hayashi K, Shimizu M, Ino H, Yamaguchi M, Mabuchi H, Hoshi N, et al. Characterization of a novel missense mutation E637K in the pore-S6 loop of HERG in a patient with long QT syndrome. Cardiovasc Res. 2002;54:67-76 pubmed
    ..61 s) and repeated episodes of syncope, we identified a single pertinent base substitution (G to A at 1909) in HERG by genetic analysis...
  74. Pfeufer A, Jalilzadeh S, Perz S, Mueller J, Hinterseer M, Illig T, et al. Common variants in myocardial ion channel genes modify the QT interval in the general population: results from the KORA study. Circ Res. 2005;96:693-701 pubmed
  75. Chen M, Sandow S, Doceul V, Chen Y, Harper H, Hamilton B, et al. Improved functional expression of recombinant human ether-a-go-go (hERG) K+ channels by cultivation at reduced temperature. BMC Biotechnol. 2007;7:93 pubmed
    b>HERG potassium channel blockade is the major cause for drug-induced long QT syndrome, which sometimes cause cardiac disrhythmias and sudden death...
  76. Yao Y, Teng S, Li N, Zhang Y, Boyden P, Pu J. Aminoglycoside antibiotics restore functional expression of truncated HERG channels produced by nonsense mutations. Heart Rhythm. 2009;6:553-60 pubmed publisher
    Pharmacologic restoration of the trafficking defects of HERG missense mutations has been documented. However, whether correction of HERG nonsense mutations is possible is unknown...
  77. Atalar F, Acuner T, Cine N, Oncu F, Yesilbursa D, Ozbek U, et al. Two four-marker haplotypes on 7q36.1 region indicate that the potassium channel gene HERG1 (KCNH2, Kv11.1) is related to schizophrenia: a case control study. Behav Brain Funct. 2010;6:27 pubmed publisher
    ..adverse effect of these medications is the acquired long QT syndrome, which results from the blockade of cardiac HERG1 channels (human ether-a-go-go-related gene potassium channels 1) by antipsychotic agents...
  78. Zheng F, Li J, Du W, Wang N, Li H, Huang S. Human ether-a-go-go-related gene K+ channels regulate shedding of leukemia cell-derived microvesicles. Leuk Lymphoma. 2012;53:1592-8 pubmed publisher
    ..Human ether-a-go-go-related gene (hERG1) K(+) channels are highly expressed in cancer cells and appear of exceptional importance in favoring cancer ..
  79. Anson B, Ackerman M, Tester D, Will M, Delisle B, Anderson C, et al. Molecular and functional characterization of common polymorphisms in HERG (KCNH2) potassium channels. Am J Physiol Heart Circ Physiol. 2004;286:H2434-41 pubmed
    ..Chromosome 7-linked inherited LQTS (LQT2) is caused by mutations in human ether-a-go-go-related gene (HERG; KCNH2), whereas drug-induced LQTS is caused ..
  80. Choe C, Schulze Bahr E, Neu A, Xu J, Zhu Z, Sauter K, et al. C-terminal HERG (LQT2) mutations disrupt IKr channel regulation through 14-3-3epsilon. Hum Mol Genet. 2006;15:2888-902 pubmed
    ..HERG (KCNH2) gene mutations are associated with congenital long-QT syndrome (LQT2) and affect IKr activity, a key determinant in ventricular repolarization...
  81. Gong Q, Stump M, Dunn A, Deng V, Zhou Z. Alternative splicing and polyadenylation contribute to the generation of hERG1 C-terminal isoforms. J Biol Chem. 2010;285:32233-41 pubmed publisher
    The human ether-a-go-go-related gene 1 (hERG1) encodes the pore-forming subunit of the rapidly activating delayed rectifier potassium channel. Several hERG1 isoforms with different N- and C-terminal ends have been identified...
  82. Vandenberg J, Perry M, Perrin M, Mann S, Ke Y, Hill A. hERG K(+) channels: structure, function, and clinical significance. Physiol Rev. 2012;92:1393-478 pubmed
    The human ether-a-go-go related gene (hERG) encodes the pore-forming subunit of the rapid component of the delayed rectifier K(+) channel, Kv11...
  83. Crociani O, Guasti L, Balzi M, Becchetti A, Wanke E, Olivotto M, et al. Cell cycle-dependent expression of HERG1 and HERG1B isoforms in tumor cells. J Biol Chem. 2003;278:2947-55 pubmed
    ..We report here that tumor cell lines preferentially express the herg1 gene and a truncated, N-deleted form that corresponds to herg1b...
  84. Ficker E, Dennis A, Wang L, Brown A. Role of the cytosolic chaperones Hsp70 and Hsp90 in maturation of the cardiac potassium channel HERG. Circ Res. 2003;92:e87-100 pubmed
    ..For the trafficking-deficient LQT2 mutants, hERG R752W and hERG G601S, interactions with Hsp90 and Hsp70 are increased as both mutants remained ..
  85. Larsen A, Olesen S, Grunnet M, Jespersen T. Characterization of hERG1a and hERG1b potassium channels-a possible role for hERG1b in the I (Kr) current. Pflugers Arch. 2008;456:1137-48 pubmed publisher
    ..The molecular correlate underlying the I (Kr) current has been identified as the hERG1 channel...
  86. Berge K, Haugaa K, Früh A, Anfinsen O, Gjesdal K, Siem G, et al. Molecular genetic analysis of long QT syndrome in Norway indicating a high prevalence of heterozygous mutation carriers. Scand J Clin Lab Invest. 2008;68:362-8 pubmed publisher
    Mutations in the KCNQ1, HERG, SCN5A, minK and MiRP1 genes cause long QT syndrome (LQTS), of which there are two forms: the Romano Ward syndrome and the Jervell and Lange-Nielsen syndrome...
  87. Pillozzi S, Masselli M, De Lorenzo E, Accordi B, Cilia E, Crociani O, et al. Chemotherapy resistance in acute lymphoblastic leukemia requires hERG1 channels and is overcome by hERG1 blockers. Blood. 2011;117:902-14 pubmed publisher
    ..of ALL cells with MSCs induced on the lymphoblast plasma membrane the expression of a signaling complex formed by hERG1 (human ether-à-go-go-related gene 1) channels, the ?(1)-integrin subunit, and the chemokine receptor CXC ..
  88. Mihic A, Chauhan V, Gao X, Oudit G, Tsushima R. Trafficking defect and proteasomal degradation contribute to the phenotype of a novel KCNH2 long QT syndrome mutation. PLoS ONE. 2011;6:e18273 pubmed publisher
    ..This study characterized the properties of a novel KCNH2 mutation discovered in a LQT2 patient resuscitated from a ventricular fibrillation arrest...
  89. Zheng F, Li H, Liang K, Du Y, Guo D, Huang S. Imatinib has the potential to exert its antileukemia effects by down-regulating hERG1 K+ channels in chronic myelogenous leukemia. Med Oncol. 2012;29:2127-35 pubmed publisher
    ..Beside PTKs, PTK inhibitors alter the activity of a large number of voltage-dependent ion channels. hERG1 K(+) channels are highly expressed in leukemia cells and appear of exceptional importance in favoring ..
  90. Lansu K, Gentile S. Potassium channel activation inhibits proliferation of breast cancer cells by activating a senescence program. Cell Death Dis. 2013;4:e652 pubmed publisher
    Traditionally the hERG1 potassium channel has been known to have a fundamental role in membrane excitability of several mammalian cells including cardiac myocytes...
  91. Subbiah R, Clarke C, Smith D, Zhao J, Campbell T, Vandenberg J. Molecular basis of slow activation of the human ether-a-go-go related gene potassium channel. J Physiol. 2004;558:417-31 pubmed
    The human ether-á-go-go related gene (HERG) encodes the pore forming alpha-subunit of the rapid delayed rectifier K(+) channel which is central to the repolarization phase of the cardiac action potential...
  92. Lai L, Su Y, Hsieh F, Chiang F, Juang J, Liu Y, et al. Denaturing high-performance liquid chromatography screening of the long QT syndrome-related cardiac sodium and potassium channel genes and identification of novel mutations and single nucleotide polymorphisms. J Hum Genet. 2005;50:490-6 pubmed
    ..In total, 25 single nucleotide polymorphisms were identified, 10 of which were novel. In conclusion, DHPLC is a sensitive and rapid method for detection of cardiac sodium and potassium channel gene mutations. ..