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

Top Publications

  1. ncbi A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel
    M C Sanguinetti
    Eccles Program in Human Molecular Biology and Genetics, University of Utah Health Sciences Center, Salt Lake City 84112, USA
    Cell 81:299-307. 1995
  2. ncbi Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2
    I Splawski
    Department of Human Genetics, Howard Hughes Medical Institute, Division of Cardiology, Salt Lake City, Utah, USA
    Circulation 102:1178-85. 2000
  3. pmc A family of potassium channel genes related to eag in Drosophila and mammals
    J W Warmke
    Laboratory of Genetics, University of Wisconsin, Madison 53706
    Proc Natl Acad Sci U S A 91:3438-42. 1994
  4. ncbi Crystal structure and functional analysis of the HERG potassium channel N terminus: a eukaryotic PAS domain
    J H Morais Cabral
    Laboratory of Molecular Neurobiology and Biophysics, Rockefeller University, New York, New York 10021, USA
    Cell 95:649-55. 1998
  5. ncbi Prevalence of long-QT syndrome gene variants in sudden infant death syndrome
    Marianne Arnestad
    Institute of Forensic Medicine, University of Oslo, Oslo, Norway
    Circulation 115:361-7. 2007
  6. doi NMR solution structure of the N-terminal domain of hERG and its interaction with the S4-S5 linker
    Qingxin Li
    Experimental Therapeutics Center, The Agency for Science, Technology and Research, 31 Biopolis Way Nanos, 03 01, Singapore 138669, Singapore
    Biochem Biophys Res Commun 403:126-32. 2010
  7. ncbi 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
    Tania Ferrer
    Department of Pediatrics, University of Utah, Salt Lake City, Utah 94113, USA
    J Biol Chem 281:12858-64. 2006
  8. pmc 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
    Pilar de la Pena
    Department of Biochemistry and Molecular Biology, University of Oviedo, 33006 Oviedo, Spain
    J Biol Chem 286:19065-75. 2011
  9. pmc Absence of direct cyclic nucleotide modulation of mEAG1 and hERG1 channels revealed with fluorescence and electrophysiological methods
    Tinatin I Brelidze
    Department of Physiology and Biophysics, University of Washington School of Medicine, Seattle, Washington 98195, USA
    J Biol Chem 284:27989-97. 2009
  10. ncbi Four potassium channel mutations account for 73% of the genetic spectrum underlying long-QT syndrome (LQTS) and provide evidence for a strong founder effect in Finland
    Heidi Fodstad
    Research Program in Molecular Medicine, Biomedicum Helsinki, Finland
    Ann Med 36:53-63. 2004

Research Grants

  1. Carlos G Vanoye; Fiscal Year: 2014
  2. ROBERT C FOEHRING; Fiscal Year: 2016
  3. The voltage-gated Kv1.3 channel in psoriatic disease: A novel therapeutic target
    Siba P Raychaudhuri; Fiscal Year: 2012
  4. Christine Beeton; Fiscal Year: 2015
  5. Bert L Semler; Fiscal Year: 2016
  6. KEITH ALAN VOSSEL; Fiscal Year: 2015
  7. High Throughput Screening for Chemical Modifiers of Long QT Syndrome
    David J Milan; Fiscal Year: 2013
  8. The Role of KV1.3 in Effector T cells
    Peter A Calabresi; Fiscal Year: 2012
  9. BENJAMIN JORGE; Fiscal Year: 2014
  10. Leslie C Griffith; Fiscal Year: 2014

Detail Information

Publications275 found, 100 shown here

  1. ncbi A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel
    M C Sanguinetti
    Eccles Program in Human Molecular Biology and Genetics, University of Utah Health Sciences Center, Salt Lake City 84112, USA
    Cell 81:299-307. 1995
    Mutations in HERG cause an inherited cardiac arrhythmia, long QT syndrome (LQT). To define the function of HERG, we expressed the protein in Xenopus oocytes...
  2. ncbi Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2
    I Splawski
    Department of Human Genetics, Howard Hughes Medical Institute, Division of Cardiology, Salt Lake City, Utah, USA
    Circulation 102:1178-85. 2000
    ..Five genes have been implicated in Romano-Ward syndrome, the autosomal dominant form of LQTS: KVLQT1, HERG, SCN5A, KCNE1, and KCNE2...
  3. pmc A family of potassium channel genes related to eag in Drosophila and mammals
    J W Warmke
    Laboratory of Genetics, University of Wisconsin, Madison 53706
    Proc Natl Acad Sci U S A 91:3438-42. 1994
    ....
  4. ncbi Crystal structure and functional analysis of the HERG potassium channel N terminus: a eukaryotic PAS domain
    J H Morais Cabral
    Laboratory of Molecular Neurobiology and Biophysics, Rockefeller University, New York, New York 10021, USA
    Cell 95:649-55. 1998
    The HERG voltage-dependent K+ channel plays a role in cardiac electrical excitability, and when defective, it underlies one form of the long QT syndrome...
  5. ncbi Prevalence of long-QT syndrome gene variants in sudden infant death syndrome
    Marianne Arnestad
    Institute of Forensic Medicine, University of Oslo, Oslo, Norway
    Circulation 115:361-7. 2007
    ..Given the importance and potential implications of these observations, we performed a study to more accurately quantify the contribution to SIDS of LQTS gene mutations and rare variants...
  6. doi NMR solution structure of the N-terminal domain of hERG and its interaction with the S4-S5 linker
    Qingxin Li
    Experimental Therapeutics Center, The Agency for Science, Technology and Research, 31 Biopolis Way Nanos, 03 01, Singapore 138669, Singapore
    Biochem Biophys Res Commun 403:126-32. 2010
    The human Ether-à-go-go Related Gene (hERG) potassium channel mediates the rapid delayed rectifier current (IKr) in the cardiac action potential...
  7. ncbi 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
    Tania Ferrer
    Department of Pediatrics, University of Utah, Salt Lake City, Utah 94113, USA
    J Biol Chem 281:12858-64. 2006
    ..that the S4-S5 linker couples voltage sensor movement to the S6 domain in the human ether-a'-go-go-related gene (hERG) K+ channel. The recently solved crystal structure of the voltage-gated Kv1...
  8. pmc 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
    Pilar de la Pena
    Department of Biochemistry and Molecular Biology, University of Oviedo, 33006 Oviedo, Spain
    J Biol Chem 286:19065-75. 2011
    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...
  9. pmc Absence of direct cyclic nucleotide modulation of mEAG1 and hERG1 channels revealed with fluorescence and electrophysiological methods
    Tinatin I Brelidze
    Department of Physiology and Biophysics, University of Washington School of Medicine, Seattle, Washington 98195, USA
    J Biol Chem 284:27989-97. 2009
    ..Here we explored cyclic nucleotide binding and modulation of mEAG1 and hERG1 channels with fluorescence and electrophysiology...
  10. ncbi Four potassium channel mutations account for 73% of the genetic spectrum underlying long-QT syndrome (LQTS) and provide evidence for a strong founder effect in Finland
    Heidi Fodstad
    Research Program in Molecular Medicine, Biomedicum Helsinki, Finland
    Ann Med 36:53-63. 2004
    Mutations in five cardiac voltage-gated ion channel genes, including KCNQ1, HERG, SCN5A, KCNE1 and KCNE2, constitute the principal cause of inherited long-QT syndrome (LQTS)...
  11. doi Regulation of the voltage-insensitive step of HERG activation by extracellular pH
    Qinlian Zhou
    Department of Physiology and Biophysics, 124 Sherman Hall, State Univ of New York, Univ at Buffalo, Buffalo, NY 14214, USA
    Am J Physiol Heart Circ Physiol 298:H1710-8. 2010
    Human ether-à-go-go-related gene (HERG, Kv11.1, KCNH2) voltage-gated K(+) channels dominate cardiac action potential repolarization...
  12. doi Chemotherapy resistance in acute lymphoblastic leukemia requires hERG1 channels and is overcome by hERG1 blockers
    Serena Pillozzi
    Department of Experimental Pathology and Oncology, University of Firenze, Firenze, Italy
    Blood 117:902-14. 2011
    ..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 β(1)-integrin subunit, and the chemokine receptor CXC ..
  13. ncbi A novel mutation (T65P) in the PAS domain of the human potassium channel HERG results in the long QT syndrome by trafficking deficiency
    Aimee Paulussen
    Department of Pharmacogenomics, Johnson and Johnson Pharmaceutical Research and Development, Beerse B 2340, Belgium
    J Biol Chem 277:48610-6. 2002
    ..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 ..
  14. pmc Molecular basis of slow activation of the human ether-a-go-go related gene potassium channel
    Rajesh N Subbiah
    Electrophysiology and Biophysics Program, Victor Chang Cardiac Research Institute, University of New South Wales, Victoria Street, Sydney, NSW 2010, Australia
    J Physiol 558:417-31. 2004
    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...
  15. pmc Probing the outer mouth structure of the HERG channel with peptide toxin footprinting and molecular modeling
    Gea Ny Tseng
    Department of Physiology, Virginia Commonwealth University, Richmond, Virginia 23298, USA
    Biophys J 92:3524-40. 2007
    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 ..
  16. doi The hERG potassium channel and hERG screening for drug-induced torsades de pointes
    Jules C Hancox
    Department of Physiology and Pharmacology, Cardiovascular Research Laboratories, Bristol Heart Institute, School of Medical Sciences, The University of Bristol, University Walk, Bristol, BS8 1TD, United Kingdom
    Pharmacol Ther 119:118-32. 2008
    ..b>hERG (human ether-à-go-go-related gene, alternative nomenclature KCNH2) is responsible for channels mediating the '..
  17. doi FRET with multiply labeled HERG K(+) channels as a reporter of the in vivo coarse architecture of the cytoplasmic domains
    Pablo Miranda
    Departamento de Bioquimica y Biologia Molecular, Edificio Santiago Gascon, Campus del Cristo, Universidad de Oviedo E 33006 Oviedo, Asturias, Spain
    Biochim Biophys Acta 1783:1681-99. 2008
    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 ..
  18. ncbi Downregulation of the HERG (KCNH2) K(+) channel by ceramide: evidence for ubiquitin-mediated lysosomal degradation
    Hugh Chapman
    Minerva Foundation Institute for Medical Research, Biomedicum Helsinki, Haartmaninkatu 8, FI 00290, Helsinki, Finland
    J Cell Sci 118:5325-34. 2005
    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...
  19. pmc Mechanisms of pharmacological rescue of trafficking-defective hERG mutant channels in human long QT syndrome
    Qiuming Gong
    Division of Cardiovascular Medicine, Department of Medicine, Oregon Health and Science University, Portland, 97239, USA
    J Biol Chem 281:4069-74. 2006
    Long QT syndrome type 2 is caused by mutations in the human ether-a-go-go-related gene (hERG)...
  20. pmc Structural basis of action for a human ether-a-go-go-related gene 1 potassium channel activator
    Matthew Perry
    Nora Eccles Harrison Cardiovascular Research and Training Institute and Department of Physiology, University of Utah, 95 South 2000 East, Salt Lake City, UT 84112, USA
    Proc Natl Acad Sci U S A 104:13827-32. 2007
    Activation of human ether-a-go-go-related gene 1 (hERG1) K(+) channels mediates cardiac action potential repolarization...
  21. pmc A primate-specific, brain isoform of KCNH2 affects cortical physiology, cognition, neuronal repolarization and risk of schizophrenia
    Stephen J Huffaker
    Clinical Brain Disorders Branch, National Institute of Mental Health, Bethesda, Maryland, USA
    Nat Med 15:509-18. 2009
    ..These results identify a previously undescribed KCNH2 channel isoform involved in cortical physiology, cognition and psychosis, providing a potential new therapeutic drug target...
  22. ncbi Drug block of the hERG potassium channel: insight from modeling
    Phillip J Stansfeld
    Department of Cell Physiology and Pharmacology, University of Leicester, Leicester, United Kingdom
    Proteins 68:568-80. 2007
    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...
  23. pmc Homozygous missense N629D hERG (KCNH2) potassium channel mutation causes developmental defects in the right ventricle and its outflow tract and embryonic lethality
    Guo Qi Teng
    Libin Cardiovascular Institute, University of Calgary, Canada
    Circ Res 103:1483-91. 2008
    Loss-of-function mutations in the human ERG1 potassium channel (hERG1) frequently underlie the long QT2 (LQT2) syndrome...
  24. doi Human ether-a-go-go-related gene K+ channels regulate shedding of leukemia cell-derived microvesicles
    Fang Zheng
    Center for Stem Cell Research and Application, Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, PR China
    Leuk Lymphoma 53:1592-8. 2012
    ..Human ether-a-go-go-related gene (hERG1) K(+) channels are highly expressed in cancer cells and appear of exceptional importance in favoring cancer ..
  25. pmc Modulation of human ether-à-go-go-related K+ (HERG) channel inactivation by Cs+ and K+
    Shetuan Zhang
    Department of Physiology, University of British Columbia, 2146 Health Sciences Mall, Vancouver, BC, Canada V6T 1Z3
    J Physiol 548:691-702. 2003
    Unlike many other native and cloned K+ channels, human ether-à-go-go-related K+ (HERG) channels show significant Cs+ permeability with a PCs/PK (the permeability of Cs+ relative to that of K+) of 0.36 +/- 0.03 (n = 10)...
  26. ncbi Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism
    Corey L Anderson
    Department of Medicine, University of Wisconsin Madison, WI, USA
    Circulation 113:365-73. 2006
    ..Type 2 congenital long-QT syndrome (LQT2) results from KCNH2 mutations that cause loss of Kv11.1 channel function...
  27. pmc A splice site mutation in hERG leads to cryptic splicing in human long QT syndrome
    Qiuming Gong
    Division of Cardiovascular Medicine, Oregon Health and Science University, Portland, OR, USA
    J Mol Cell Cardiol 44:502-9. 2008
    Mutations in the human ether-a-go-go-related gene (hERG) cause type 2 long QT syndrome...
  28. ncbi Characterization of a novel missense mutation E637K in the pore-S6 loop of HERG in a patient with long QT syndrome
    Kenshi Hayashi
    Molecular Genetics of Cardiovascular Disorders, Division of Cardiovascular Medicine, Graduate School of Medical Science, Kanazawa University 13 1, Takara machi, Ishikawa 920 8640, Japan
    Cardiovasc Res 54:67-76. 2002
    ..61 s) and repeated episodes of syncope, we identified a single pertinent base substitution (G to A at 1909) in HERG by genetic analysis...
  29. ncbi herg1 gene and HERG1 protein are overexpressed in colorectal cancers and regulate cell invasion of tumor cells
    Elena Lastraioli
    Department of Experimental Pathology and Oncology, University of Firenze, Firenze, Italy
    Cancer Res 64:606-11. 2004
    ..The herg1 gene and HERG1 protein are expressed in many colon cancer cell lines, and the activity of HERG channels modulates ..
  30. ncbi Novel C-terminus frameshift mutation, 1122fs/147, of HERG in LQT2: additional amino acids generated by frameshift cause accelerated inactivation
    Tetsuo Sasano
    Department of Cardiovascular Disease, Medical Research Institute, Tokyo Medical and Dental University, 1 5 45 Yushima, Bunkyo ku, Tokyo 113 8510, Japan
    J Mol Cell Cardiol 37:1205-11. 2004
    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...
  31. pmc A new C-terminal hERG mutation A915fs+47X associated with symptomatic LQT2 and auditory-trigger syncope
    Georges Christé
    INSERM, ADR Lyon, Lyon, F 69003 France
    Heart Rhythm 5:1577-86. 2008
    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.
  32. doi Aminoglycoside antibiotics restore functional expression of truncated HERG channels produced by nonsense mutations
    Yan Yao
    Center for Arrhythmia Diagnosis and Treatment, Cardiovascular Institute and Fu Wai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China
    Heart Rhythm 6:553-60. 2009
    Pharmacologic restoration of the trafficking defects of HERG missense mutations has been documented. However, whether correction of HERG nonsense mutations is possible is unknown.
  33. ncbi A K+ channel splice variant common in human heart lacks a C-terminal domain required for expression of rapidly activating delayed rectifier current
    S Kupershmidt
    Departments of Medicine and Pharmacology, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA
    J Biol Chem 273:27231-5. 1998
    ..rectifier (IKr), from human heart, and we find that its mRNA is approximately 2-fold more abundant than that for HERG1 (the originally described cDNA). After transfection of HERG USO in Ltk- cells, no current was observed...
  34. pmc Physiological properties of hERG 1a/1b heteromeric currents and a hERG 1b-specific mutation associated with Long-QT syndrome
    Harinath Sale
    Department of Physiology, University of Wisconsin, Madison, WI, USA
    Circ Res 103:e81-95. 2008
    ..Biochemical and functional studies have demonstrated that I Kr channels are heteromers composed of both hERG 1a and 1b subunits, yet our current understanding of I Kr functional properties derives primarily from studies of ..
  35. ncbi A minK-HERG complex regulates the cardiac potassium current I(Kr)
    T V McDonald
    Section of Molecular Cardiology, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    Nature 388:289-92. 1997
    ..b>HERG, a human homologue of the ether-a-go-go gene of the fruitfly Drosophila melanogaster, encodes a protein that ..
  36. ncbi Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing
    David J Tester
    Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA
    Heart Rhythm 2:507-17. 2005
    ..The purpose of this study was to determine the spectrum and prevalence of cardiac channel mutations among a large cohort of consecutive, unrelated patients referred for long QT syndrome (LQTS) genetic testing...
  37. ncbi Bradycardia-induced long QT syndrome caused by a de novo missense mutation in the S2-S3 inner loop of HERG
    H Yoshida
    Department of Cardiovascular Medicine, Kyoto University Graduate School of Medicine, Shogoin Kawara 54, Sakyo, Kyoto, 606 8507, Japan
    Am J Med Genet 98:348-52. 2001
    ..We report a case of a novel HERG mutation (A490T) that caused a bradycardia-associated form of long QT syndrome...
  38. ncbi The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations
    Eckhard Ficker
    Rammelkamp Center for Education and Research, MetroHealth Campus, Case Western Reserve University, Cleveland, Ohio 44109, USA
    J Biol Chem 277:4989-98. 2002
    Mutations in the human ether-a-gogo-related gene (HERG) K(+) channel gene cause chromosome 7-linked long QT syndrome type 2 (LQT2), which is characterized by a prolonged QT interval in the electrocardiogram and an increased susceptibility ..
  39. ncbi Increased risk of arrhythmic events in long-QT syndrome with mutations in the pore region of the human ether-a-go-go-related gene potassium channel
    Arthur J Moss
    Cardiology Unit of the Department of Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA
    Circulation 105:794-9. 2002
    ..Mutations involving the human ether-a-go-go-related gene (HERG) channel are responsible for the LQT2 form of long-QT syndrome, and in cellular expression studies these mutations are associated with reduction in the ..
  40. ncbi Association between HERG K897T polymorphism and QT interval in middle-aged Finnish women
    Eeva Pietilä
    Division of Cardiology, Department of Medicine, University of Oulu, Oulu, Finland
    J Am Coll Cardiol 40:511-4. 2002
    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.
  41. ncbi Common variants in myocardial ion channel genes modify the QT interval in the general population: results from the KORA study
    Arne Pfeufer
    Institute of Human Genetics, Technical University Munich, Munich, Germany
    Circ Res 96:693-701. 2005
    ....
  42. doi Genomic structure, transcriptional control, and tissue distribution of HERG1 and KCNQ1 genes
    Xiaobin Luo
    Research Center, Montreal Heart Institute, University of Montreal, Montreal, PQ, Canada H1T 1C8
    Am J Physiol Heart Circ Physiol 294:H1371-80. 2008
    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 ..
  43. doi Human ether-a-go-go related gene (hERG) K+ channels: function and dysfunction
    Mark J Perrin
    Victor Chang Cardiac Research Institute, 405 Liverpool Street, Darlinghurst, NSW 2010, Australia
    Prog Biophys Mol Biol 98:137-48. 2008
    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...
  44. ncbi HERG, a human inward rectifier in the voltage-gated potassium channel family
    M C Trudeau
    Department of Physiology, University of Wisconsin Medical School, Madison 53706, USA
    Science 269:92-5. 1995
    ..members of the Eag family of voltage-gated, outwardly rectifying potassium channels, the human eag-related gene (HERG) has now been shown to encode an inwardly rectifying potassium channel...
  45. ncbi C-terminal HERG mutations: the role of hypokalemia and a KCNQ1-associated mutation in cardiac event occurrence
    M Berthet
    INSERM U153, Service de Biochimie, Groupe Hospitalier Pitie Salpetriere, Paris Service de Cardiologie, Hopital Lariboisiere, Paris, France
    Circulation 99:1464-70. 1999
    ..Most of the mutations have been determined in the transmembrane domains of the cardiac potassium channel genes KCNQ1 and HERG. In this study, we investigated the 3' part of HERG for mutations.
  46. ncbi Relevance of the proximal domain in the amino-terminus of HERG channels for regulation by a phospholipase C-coupled hormone receptor
    David Gomez-Varela
    Departamento de Bioquimica y Biologia Molecular, Edificio Santiago Gascon, Campus del Cristo, Universidad de Oviedo, E 33006 Oviedo, Asturias, Spain
    FEBS Lett 535:125-30. 2003
    ..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 ..
  47. ncbi Identification of a COOH-terminal segment involved in maturation and stability of human ether-a-go-go-related gene potassium channels
    Armin Akhavan
    Department of Physiology, McGill University, Montreal, Quebec H3G 1Y6, Canada
    J Biol Chem 278:40105-12. 2003
    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 ..
  48. ncbi Postmortem molecular screening in unexplained sudden death
    Sumeet S Chugh
    Division of Cardiology, Oregon Health and Science University, Portland, 97239, USA
    J Am Coll Cardiol 43:1625-9. 2004
    ..We examined the prevalence of defects in arrhythmia-related candidate genes among patients with unexplained sudden cardiac death (SCD)...
  49. ncbi Regional specificity of human ether-a'-go-go-related gene channel activation and inactivation gating
    David R Piper
    Department of Physiology, Nora Eccles Harrison Cardiovascular Research and Training Institute, University of Utah, Salt Lake City, Utah 84112, USA
    J Biol Chem 280:7206-17. 2005
    ..produce inward rectification of the current-voltage relationship for human ether-a'-go-go-related gene (hERG) channels...
  50. ncbi Novel mutation in the Per-Arnt-Sim domain of KCNH2 causes a malignant form of long-QT syndrome
    Tom Rossenbacker
    Centre for Transgene Technology and Gene Therapy, Flanders Interuniversitary Institute for Biotechnology, KU Leuven, Leuven, Belgium
    Circulation 111:961-8. 2005
    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...
  51. ncbi Degradation of trafficking-defective long QT syndrome type II mutant channels by the ubiquitin-proteasome pathway
    Qiuming Gong
    Division of Molecular Medicine, Department of Medicine, Oregon Health and Science University, Portland, Oregon 97239, USA
    J Biol Chem 280:19419-25. 2005
    Mutations in the human ether-a-go-go-related gene (hERG) cause chromosome 7-linked long QT syndrome type II (LQT2)...
  52. ncbi Short QT syndrome and atrial fibrillation caused by mutation in KCNH2
    Kui Hong
    Molecular Genetics, Masonic Medical Research Laboratory, Utica, New York, USA
    J Cardiovasc Electrophysiol 16:394-6. 2005
    ..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...
  53. ncbi Association of KCNQ1, KCNE1, KCNH2 and SCN5A polymorphisms with QTc interval length in a healthy population
    Laetitia Gouas
    INSERM U582, Institut de Myologie, IFR 14, UPMC, Groupe Hospitalier Pitie Salpetriere, Paris, France
    Eur J Hum Genet 13:1213-22. 2005
    ..This suggests that genetic determinants located in KCNQ1, KCNE1, KCNH2 and SCN5A influence QTc length in healthy individuals and may represent risk factors for arrhythmias or cardiac sudden death in patients with cardiovascular diseases...
  54. ncbi VEGFR-1 (FLT-1), beta1 integrin, and hERG K+ channel for a macromolecular signaling complex in acute myeloid leukemia: role in cell migration and clinical outcome
    Serena Pillozzi
    Department of Experimental Pathology and Oncology, University of Firenze, Firenze, Italy
    Blood 110:1238-50. 2007
    ..cross talk among (1) the vascular endothelial growth factor receptor-1, FLT-1; (2) the human eag-related gene 1 (hERG1) K(+) channels; and (3) integrin receptors in acute myeloid leukemia (AML) cells...
  55. pmc Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome
    Qiuming Gong
    Division of Cardiovascular Medicine, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, Portland, OR 97239, USA
    Circulation 116:17-24. 2007
    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...
  56. ncbi Common genetic variation in KCNH2 is associated with QT interval duration: the Framingham Heart Study
    Christopher Newton-Cheh
    National Heart, Lung, and Blood Institute s Framingham Heart Study, Framingham, Mass, USA
    Circulation 116:1128-36. 2007
    ..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...
  57. doi Cooperative interactions between R531 and acidic residues in the voltage sensing module of hERG1 channels
    David R Piper
    Nora Eccles Harrison Cardiovascular Research and Training Institute, University of Utah, Salt Lake City, UT 84113, USA
    Cell Physiol Biochem 21:37-46. 2008
    b>HERG1 K(+) channels are critical for modulating the duration of the cardiac action potential...
  58. pmc Inhibition of the rapid component of the delayed rectifier potassium current in ventricular myocytes by angiotensin II via the AT1 receptor
    Y H Wang
    Department of Pharmacology, Hebei Medical University, Shijiazhuang, China
    Br J Pharmacol 154:429-39. 2008
    ..The rapid component of the delayed rectifier K(+) current (I(Kr)) plays a critical role in cardiac repolarization. Hence, the aim of this study was to assess the effect of Ang II on I(Kr) in guinea-pig ventricular myocytes...
  59. pmc Stimulation of N-terminal truncated isoform of androgen receptor stabilizes human ether-á-go-go-related gene-encoded potassium channel protein via activation of extracellular signal regulated kinase 1/2
    Zhi yuan Wu
    Department of Pharmacology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 117597, Singapore
    Endocrinology 149:5061-9. 2008
    ..to determine whether androgens regulate the function and expression of the human ether-á-go-go-related gene (HERG) encoded K+ channel, which is largely responsible for determining the QT interval...
  60. doi Both EGFR kinase and Src-related tyrosine kinases regulate human ether-à-go-go-related gene potassium channels
    De Yong Zhang
    Department of Medicine, The University of Hong Kong, Pokfulam, Hong Kong SAR, China
    Cell Signal 20:1815-21. 2008
    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...
  61. pmc Common candidate gene variants are associated with QT interval duration in the general population
    A Marjamaa
    Research Program in Molecular Medicine, Biomedicum Helsinki, University of Helsinki, Helsinki, Finland
    J Intern Med 265:448-58. 2009
    ..As 30-40% of the QT-interval variability is heritable, we tested the association of common LQTS and NOS1AP gene variants with QT interval in a Finnish population-based sample...
  62. doi Role of intracellular domains in the function of the herg potassium channel
    Moza Al-Owais
    Faculty of Biological Sciences, University of Leeds, Leeds, UK
    Eur Biophys J 38:569-76. 2009
    ..regions (which include the cyclic nucleotide binding domain, cNBD, and the Per-Arnt-Sim domain, PAS) in the herg channel is not well understood...
  63. doi Long QT syndrome with compound mutations is associated with a more severe phenotype: a Japanese multicenter study
    Hideki Itoh
    Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science, Otsu, Japan
    Heart Rhythm 7:1411-8. 2010
    ..Long QT syndrome (LQTS) can be caused by mutations in the cardiac ion channels. Compound mutations occur at a frequency of 4% to 11% among genotyped LQTS cases...
  64. pmc Novel characteristics of a trafficking-defective G572R-hERG channel linked to hereditary long QT syndrome
    Jiangfang Lian
    Lihuili Hospital, Medical School of Ningbo University, People s Republic of China
    Can J Cardiol 26:417-22. 2010
    ..Type 2 congenital long QT syndrome (LQT2) results from KCNH2 or hERG gene mutations. hERG encodes the K(v)11...
  65. doi Mapping the sequence of conformational changes underlying selectivity filter gating in the K(v)11.1 potassium channel
    David T Wang
    Mark Cowley Lidwill Research Program in Cardiac Electrophysiology, Molecular Cardiology and Biophysics Division, Victor Chang Cardiac Research Institute, New South Wales, Australia
    Nat Struct Mol Biol 18:35-41. 2011
    ..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, ..
  66. pmc The role of monoubiquitination in endocytic degradation of human ether-a-go-go-related gene (hERG) channels under low K+ conditions
    Tao Sun
    Department of Physiology, Queen s University, Kingston, Ontario K7L 3N6, Canada
    J Biol Chem 286:6751-9. 2011
    ..rapidly activating delayed rectifier potassium channel (I(Kr)) encoded by the human ether-a-go-go-related gene (hERG)...
  67. doi Nedd4-2-dependent ubiquitylation and regulation of the cardiac potassium channel hERG1
    Maxime Albesa
    Department of Clinical Research, University of Bern, Switzerland
    J Mol Cell Cardiol 51:90-8. 2011
    The voltage-gated cardiac potassium channel hERG1 (human ether-à-gogo-related gene 1) plays a key role in the repolarization phase of the cardiac action potential (AP)...
  68. ncbi hERG K(+) channels: structure, function, and clinical significance
    Jamie I Vandenberg
    Mark Cowley Lidwill Research Programme in Cardiac Electrophysiology, Victor Chang Cardiac Research Institute, Sydney, New South Wales, Australia
    Physiol Rev 92:1393-478. 2012
    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...
  69. ncbi A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome
    M E Curran
    Department of Human Genetics, University of Utah Health Sciences Center, Salt Lake City 84112
    Cell 80:795-803. 1995
    ..Here, linkage and physical mapping place LQT2 and a putative potassium channel gene, HERG, on chromosome 7q35-36...
  70. ncbi 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
    X Li
    Department of Physiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Biol Chem 272:705-8. 1997
    b>HERG (human eag-related gene) encodes an inward-rectifier potassium channel formed by the assembly of four subunits...
  71. ncbi Multiple different missense mutations in the pore region of HERG in patients with long QT syndrome
    C A Satler
    Department of Cardiology, Children s Hospital, Boston, MA 02115, USA
    Hum Genet 102:265-72. 1998
    ..for mutations in the pore region of HERG , the K+ channel gene previously associated with chromosome 7-linked LQT2. In six unrelated LQTS kindreds, single-strand conformation polymorphism analyses identified aberrant conformers in ..
  72. ncbi Long QT syndrome with a high mortality rate caused by a novel G572R missense mutation in KCNH2
    L A Larsen
    Department of Clinical Biochemistry, Statens Serum Institut, Copenhagen, Denmark
    Clin Genet 57:125-30. 2000
    ..causing the substitution of a glycine residue at position 572, at the end of the S5 transmembrane segment of the HERG K(+)-channel, with an arginine residue...
  73. ncbi Cyclic AMP regulates the HERG K(+) channel by dual pathways
    J Cui
    Department of Medicine and Molecular Pharmacology, Section of Molecular Cardiology, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    Curr Biol 10:671-4. 2000
    ..Here we show that the second messenger cAMP regulates the K(+) channel mutated in the LQT2 form of LQTS, HERG [2], both directly and indirectly...
  74. ncbi A common polymorphism in KCNH2 (HERG) hastens cardiac repolarization
    Connie R Bezzina
    Experimental and Molecular Cardiology Group, Room M0 052, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
    Cardiovasc Res 59:27-36. 2003
    ..Since the current encoded by KCNH2 (HERG; I(Kr)) is a primary determinant of repolarization, we conducted association studies between the respective alleles ..
  75. ncbi Sudden death associated with short-QT syndrome linked to mutations in HERG
    Ramon Brugada
    Molecular Genetics Program, Masonic Medical Research Laboratory, 2150 Bleecker St, Utica, NY 13501, USA
    Circulation 109:30-5. 2004
    ..The present study describes the genetic basis for a new clinical entity characterized by sudden death and short-QT intervals in the ECG...
  76. ncbi Impairment of HERG K(+) channel function by tumor necrosis factor-alpha: role of reactive oxygen species as a mediator
    Jingxiong Wang
    Research Center, Montreal Heart Institute, Montreal, Quebec H1T 1C8
    J Biol Chem 279:13289-92. 2004
    ..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 ..
  77. ncbi Molecular and functional characterization of common polymorphisms in HERG (KCNH2) potassium channels
    Blake D Anson
    Department of Medicine, University of Wisconsin, 1300 University Ave, Madison, WI 53711, USA
    Am J Physiol Heart Circ Physiol 286:H2434-41. 2004
    ..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 ..
  78. pmc Mechanism of block of hEag1 K+ channels by imipramine and astemizole
    Rafael E García-Ferreiro
    Abteilung Molekulare Biologie Neuronaler Signale, Max Planck Institut fur experimentelle Medizin, 37075 Gottingen, Germany
    J Gen Physiol 124:301-17. 2004
    ..The significance of these findings, in the context of structure-function of channels of the eag family is discussed...
  79. pmc Tryptophan scanning mutagenesis of the HERG K+ channel: the S4 domain is loosely packed and likely to be lipid exposed
    Rajesh N Subbiah
    Victor Chang Cardiac Research Institute, 384 Victoria Street, Darlinghurst, NSW 2010, Australia
    J Physiol 569:367-79. 2005
    ..mutations or drug-induced block of voltage-gated ion channels, including the human ether-à-go-go-related gene (HERG) K+ channel, are significant causes of malignant arrhythmias and sudden death...
  80. doi Regulation of HERG (KCNH2) potassium channel surface expression by diacylglycerol
    Cia Ramström
    Department of Biology, Abo Akademi University, 20520, Turku, Finland
    Cell Mol Life Sci 67:157-69. 2010
    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...
  81. pmc HERG1 channelopathies
    Michael C Sanguinetti
    Department of Physiology, Nora Eccles Harrison Cardiovascular Research and Training Institute, University of Utah, 95 South 2000 East, Salt Lake, UT 84112, USA
    Pflugers Arch 460:265-76. 2010
    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...
  82. pmc Involvement of caveolin in low K+-induced endocytic degradation of cell-surface human ether-a-go-go-related gene (hERG) channels
    Hamid Massaeli
    Department of Physiology, Queen s University, Kingston, Ontario K7L 3N6, Canada
    J Biol Chem 285:27259-64. 2010
    ..delayed rectifier K(+) channel current (I(Kr)) due to either mutations in the human ether-a-go-go-related gene (hERG) or drug block causes inherited or drug-induced long QT syndrome...
  83. pmc 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
    Elena C Gianulis
    Program in Molecular Medicine, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA
    J Biol Chem 286:22160-9. 2011
    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...
  84. doi A422T mutation in HERG potassium channel retained in ER is rescurable by pharmacologic or molecular chaperones
    Jia Guo
    Jiangsu Key Laboratory for Molecular and Medical Biotechnology, College of Life Science, Nanjing Normal University, Nanjing 210046, China
    Biochem Biophys Res Commun 422:305-10. 2012
    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 ..
  85. pmc The serum- and glucocorticoid-inducible kinases SGK1 and SGK3 regulate hERG channel expression via ubiquitin ligase Nedd4-2 and GTPase Rab11
    Shawn M Lamothe
    Department of Biomedical and Molecular Sciences, Queen s University, Kingston, Ontario K7L 3N6, Canada
    J Biol Chem 288:15075-84. 2013
    The hERG (human ether-a-go-go-related gene) encodes the α subunit of the rapidly activating delayed rectifier potassium channel (IKr)...
  86. ncbi The inward rectification mechanism of the HERG cardiac potassium channel
    P L Smith
    Department of Neurobiology, Harvard Medical School, Boston, MA 02114 2698, USA
    Nature 379:833-6. 1996
    ..of cardiac rhythm involving the repolarization of the action potential, was recently found to lie in the HERG gene, which codes for a potassium channel...
  87. ncbi Four novel KVLQT1 and four novel HERG mutations in familial long-QT syndrome
    T Tanaka
    Laboratory of Molecular Medicine, University of Tokyo, Japan
    Circulation 95:565-7. 1997
    ..Three genes responsible for this syndrome (KVLQT1, HERG, and SCN5A) have been identified so far. We investigated mutations of these genes in LQTS families.
  88. ncbi Electrophysiological characterization of an alternatively processed ERG K+ channel in mouse and human hearts
    J P Lees-Miller
    Department of Medicine, University of Calgary, Alberta, Canada
    Circ Res 81:719-26. 1997
    Mutants of HERG, the human form of ERG (the ether-a-go-go-related K+ channel gene), are responsible for some forms of the long-QT syndrome, an abnormality of cardiac repolarization...
  89. ncbi 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
    B London
    Division of Cardiology, University of Pittsburgh Medical Center, PA 15213 2582, USA
    Circ Res 81:870-8. 1997
    b>HERG, the human ether-a-go-go-related gene, encodes a K(+)-selective channel with properties similar to the rapidly activating component of the delayed rectifier K+ current (IKr)...
  90. ncbi Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivation
    J Chen
    Department of Medicine, Division of Cardiology, University of Utah, Salt Lake City, Utah 84112, USA
    J Biol Chem 274:10113-8. 1999
    Mutations in the human ether-a-go-go-related gene (HERG) cause long QT syndrome, an inherited disorder of cardiac repolarization that predisposes affected individuals to life-threatening arrhythmias...
  91. ncbi MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia
    G W Abbott
    Department of Pediatrics, Boyer Center for Molecular Medicine, Yale University School of Medicine, New Haven, Connecticut 06536, USA
    Cell 97:175-87. 1999
    ..The gene encodes MinK-related peptide 1 (MiRP1), a small integral membrane subunit that assembles with HERG, a pore-forming protein, to alter its function...
  92. ncbi Survey of the coding region of the HERG gene in long QT syndrome reveals six novel mutations and an amino acid polymorphism with possible phenotypic effects
    P Laitinen
    Department of Medicine, University of Helsinki, FIN 00290 Helsinki, Finland
    Hum Mutat 15:580-1. 2000
    Analysis of the entire coding region of the HERG gene of 39 Finnish LQTS patients revealed eight mutations, six of which are hitherto unreported...
  93. ncbi Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias
    P J Schwartz
    Department of Cardiology, Policlinico S Matteo IRCCS and University of Pavia, Pavia, Italy
    Circulation 103:89-95. 2001
    ..Preliminary observations suggested that the conditions ("triggers") associated with cardiac events may in large part be gene specific...
  94. ncbi Defective human Ether-à-go-go-related gene trafficking linked to an endoplasmic reticulum retention signal in the C terminus
    Sabina Kupershmidt
    Departments of Pharmacology and Medicine, Vanderbilt University School of Medicine, 561A Preston Research Building, Nashville, TN 37232, USA
    J Biol Chem 277:27442-8. 2002
    Mutations in the human Ether-à-go-go-Related gene (HERG), encoding the protein underlying the cardiac K(+) current, I(Kr), cause chromosome 7-linked long QT syndrome (LQT2)...
  95. ncbi Role of glycosylation in cell surface expression and stability of HERG potassium channels
    Qiuming Gong
    Division of Molecular Medicine, Department of Medicine, Oregon Health and Science University, Portland, Oregon 97201, USA
    Am J Physiol Heart Circ Physiol 283:H77-84. 2002
    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...
  96. ncbi DHPLC analysis of potassium ion channel genes in congenital long QT syndrome
    Roselie Jongbloed
    Department of Genetics and Cell Biology, University Maastricht, Maastricht, The Netherlands
    Hum Mutat 20:382-91. 2002
    ..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...
  97. ncbi Cell cycle-dependent expression of HERG1 and HERG1B isoforms in tumor cells
    Olivia Crociani
    Department of Experimental Pathology and Oncology, University of Firenze, Viale G B Morgagni 50, 50134 Firenze, Italy
    J Biol Chem 278:2947-55. 2003
    ..We report here that tumor cell lines preferentially express the herg1 gene and a truncated, N-deleted form that corresponds to herg1b...
  98. ncbi HERG K(+) currents in human prolactin-secreting adenoma cells
    C K Bauer
    Institut für Angewandte Physiologie, Universitatsklinikum Hamburg Eppendorf, Martinistrasse 52, Universitat Hamburg, 20246 Hamburg, Germany
    Pflugers Arch 445:589-600. 2003
    ..Transcripts for HERG1 were present in all adenomas and although transcripts for HERG2 and HERG3 were also detected, their expression ..
  99. ncbi Role of the cytosolic chaperones Hsp70 and Hsp90 in maturation of the cardiac potassium channel HERG
    Eckhard Ficker
    Rammelkamp Center, Case Western Reserve University, Cleveland, Ohio 44109, USA
    Circ Res 92:e87-100. 2003
    ..For the trafficking-deficient LQT2 mutants, hERG R752W and hERG G601S, interactions with Hsp90 and Hsp70 are increased as both mutants remained ..
  100. ncbi Structure of the HERG K+ channel S5P extracellular linker: role of an amphipathic alpha-helix in C-type inactivation
    Allan M Torres
    School of Molecular and Microbial Biosciences, University of Sydney, New South Wales 2006, Australia
    J Biol Chem 278:42136-48. 2003
    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...
  101. ncbi Clinical and electrophysiological characterization of a novel mutation R863X in HERG C-terminus associated with long QT syndrome
    Siyong Teng
    Sino German Laboratory for Molecular Medicine and Center for Molecular Cardiology, Fuwai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, 167 Beilishilu, 100037 Beijing, China
    J Mol Med (Berl) 82:189-96. 2004
    We have found a novel nonsense mutation in the C-terminus of HERG in a four-generation Chinese family with long QT syndrome and investigated the molecular mechanism of this mutation in vitro...

Research Grants66

  1. Carlos G Vanoye; Fiscal Year: 2014
    ..Aim 5. Determine the structure of KCNE4 in bilayers and formulate a working model for how KCNE4 inhibits KCNQ1 channel function. ..
  2. ROBERT C FOEHRING; Fiscal Year: 2016
    ..Time coding is important for generation of rhythmic cortical activity such as observed during attention and sensory processing. ..
  3. The voltage-gated Kv1.3 channel in psoriatic disease: A novel therapeutic target
    Siba P Raychaudhuri; Fiscal Year: 2012
    ..3+ lymphocytes pre and post treatment in the transplanted skin. The overall goal of this proposal is to determine whether Kv1.3 blockade constitutes a potential new therapeutic approach to the treatment of psoriasis. ..
  4. Christine Beeton; Fiscal Year: 2015
    ..This project will generate novel peptide blockers of Kv1.3, which we believe will be valuable leads in the development of new treatments for MS and other chronic inflammatory diseases. ..
  5. Bert L Semler; Fiscal Year: 2016
    ..abstract_text> ..
  6. KEITH ALAN VOSSEL; Fiscal Year: 2015
    ..The mentoring and research experience described in this proposal will facilitate the candidate's goal of developing a strong independent research career. ..
  7. High Throughput Screening for Chemical Modifiers of Long QT Syndrome
    David J Milan; Fiscal Year: 2013
    ..carries a mutation in the potassium channel gene, KCNH2, which is the gene mutated in human long QT type 2 (LQT2). Type 2 LQTS is responsible for approximately one third of all human long QT cases...
  8. The Role of KV1.3 in Effector T cells
    Peter A Calabresi; Fiscal Year: 2012
    ..Further, understanding basic mechanisms of T cell function will enhance our knowledge of how the immune system causes disease and allow us to develop novel strategies for correcting abnormal immune responses in diseases like MS. ..
  9. BENJAMIN JORGE; Fiscal Year: 2014
    ....
  10. Leslie C Griffith; Fiscal Year: 2014
    ..Understanding how the context of a protein's location can affect or regulate activity is critical to rational drug design and may even provide new drug targets. ..
  11. A New Family of Voltage-gated Potassium Channel Regulaory Subunits
    Steve A N Goldstein; Fiscal Year: 2011
    ..This revised application seeks to address concerns raised in the first review cycle. ..
  12. Phosphatase Regulation of Olfactory Bulb Mitral Cell Activity
    Melissa Cavallin Johnson; Fiscal Year: 2011
    ..The link between these two proteins in the olfactory system has implications not only for sensory function but also for energy homeostasis. ..
  13. CHERYL LYNN VAN BUSKIRK; Fiscal Year: 2015
    ..We will identify this signal through RNAi-based functional genomics, and characterize its function using molecular-genetic approaches. ..
  14. MOLECULAR BASIS FOR DRUG INDUCED CARDIOTOXICITY IN AIDS
    CRAIG CLARKSON; Fiscal Year: 2001
    ..the applicant's laboratory demonstrate that TNF-alpha can reduce the amplitude of human ether-a-go-go related gene (HERG) encoded K currents, an action that has been previously shown to result in prolongation of the QT interval of the ..
  15. miRNA Regulation of Gene Expression in Alcohol Tolerance and Withdrawal
    Steven N Treistman; Fiscal Year: 2013
    ..In this project we will explore a novel mechanism, acting on gene products, which will provide important insights in our quest for this understanding. ..
  16. Identification of the gene causing spinocerebellar ataxia in a Filipino family
    MICHAEL FARRIS WATERS; Fiscal Year: 2012
    ..abstract_text> ..
  17. Glucose Sensitivity of Mitral Cell Neurons of the Olfactory Bulb
    KRISTAL RAYLONE TUCKER; Fiscal Year: 2009
    ..With the ever increasing obese population in the United States, it will be important to understand how the chemistry of the obese state affects brain function. ..
  18. Behavioral Measures of Temporal Resolution in Mice With or Without Kv1.1 Subunits
    Micheal L Dent; Fiscal Year: 2010
    ..It is unknown, however, how this affects the 'real world'listening situation of these animals, so this will be determined here. ..
  19. Zhengfeng Zhou; Fiscal Year: 2016
    ..Long QT syndrome type 2 (LQT2) is caused by mutations in the human ether-a-go-go-related gene (hERG). hERG encodes the pore-forming subunit of the rapidly activating delayed rectifier potassium channel in the heart...
  20. Neuropathogenesis of Immune Reconstitution Syndrome with HIV Infection
    Avindra Nath; Fiscal Year: 2010
    ..Specific aim 2: To determine the mechanism of GB mediated neurotoxicity Specific aim 3: To determine the mechanism of neuroprotection with Kv1.3 blockers ..
  21. The Microglial Kv1.3 Channel in Alzheimer's Disease
    Heike Wulff; Fiscal Year: 2012
    ..3 is important in microglia-caused damage. We will further test whether an inhibitor for Kv1.3 reduces microglia activity in an animal model of Alzheimer's disease. ..
  22. MICHAEL CRAIG SANGUINETTI; Fiscal Year: 2015
    ..in the past, but little is known about the mechanisms of action or therapeutic utility of the recently discovered hERG1 channel activators...
  23. The role of dynamic changes in repolarization and calcium transients in Long QT r
    Bum Rak Choi; Fiscal Year: 2013
    ..We propose to use novel transgenic rabbit models of long QT syndrome (LQT1 and LQT2) created by Dr...
  24. Sex Differences in Molecular Heterogeneity of Cardiac Repolarization
    Glenna C L Bett; Fiscal Year: 2012
    ..The alpha subunits of IKr and IKs are HERG and KCNQ1 respectively...
  25. The study of HERG-interacting proteins in C. elgans
    Christina Petersen; Fiscal Year: 2003
    DESCRIPTION (the applicant?s description verbatim): Mutations in the human ether-a-go-go related gene (HERG) are linked to acquired and inherited forms of the long QT syndrome (LQTS), which can provoke the life-threatening arrythmia, ..
  26. Sex Hormones and Cardiac Arrhythmia in Transgenic LQT2 Rabbits
    Gideon Koren; Fiscal Year: 2012
    ..SCD through the application of innovative technology and the novel use of new genetic models of long QT syndrome 2 (LQT2)...
  27. Gideon Koren; Fiscal Year: 2016
    ..technology, computer modeling and the use of new genetic models of long QT syndrome type 1and type 2 (LQT1 and LQT2)...
  28. Matthew C Trudeau; Fiscal Year: 2014
    ..b>HERG and IKr are specialized to conduct an outward K+ current that drives repolarization of the late phase of the ..
  29. Delayed Rectifier K Channel Biogenesis is Unveiled in Models of Long QT Syndrome
    Brian P Delisle; Fiscal Year: 2012
    ..Studies suggest that KCNQ1 (LQT1) mutations and KCNH2 (LQT2) mutations typically result in a loss of function. Many LQT1 and most LQT2 mutations cause Kv7.1 and Kv11...
  30. Gail A Robertson; Fiscal Year: 2014
    ..This study focuses on composition and functional analysis of hERG 1a and 1b subunits contributing to channels underlying cardiac IKr, a critical target for inherited and acquired ..
  31. Neural Circulatory Control in the Long QT Syndrome
    Virend Somers; Fiscal Year: 2006
    ..are explained by mutations in cardiac ion channel genes, the commonest known mutations being classified as LQT1, LQT2, and LQT3. The degree of QT prolongation is an independent risk factor for cardiac events...
  32. MULTI-ANALYTE WAVEGUIDE IMMUNOSENSING
    JAMES HERRON; Fiscal Year: 2002
    ..LQTS has been linked to genetic polymorphisms in four genes (KVLQT1,HERG, SCN5A & KCNE1) that encode for cardiac ion channels...
  33. HERG Channel in Acquired and Inherited Long-QT Syndrome
    DENNIS DOUGHERTY; Fiscal Year: 2009
    DESCRIPTION (provided by applicant): The hERG potassium channel is a major cause of drug-induced Iong-QT syndrome...
  34. LONG QT SYNDROME:EMOTIONAL TRIGGERS OF CARDIAC EVENTS
    Richard Lane; Fiscal Year: 2005
    ..insensitive measures suggest that emotions trigger events in all LQTS patients, especially in genotype-2 (LQT2) patients...
  35. A Novel High Throughput Assay for Ion Channel Modulators
    Barbara Wible; Fiscal Year: 2006
    ..The cardiac potassium channel hERG is the most common target of undesired drug interactions; block of hERG can predispose individuals to cardiac ..
  36. MYOCARDIAL CONTROLLED RELEASE IMPLANTS FOR ARRHYTHMIAS
    Robert Levy; Fiscal Year: 2001
    ..our first choice for a model gene for RGT will be HERG (A561V), the point mutation responsible for one of the forms of the Long QT Syndrome, a hereditary disorder ..
  37. TARGETING HERG: A MOUSE MODEL OF THE LONG QT SYNDROME
    Barry London; Fiscal Year: 2002
    Arrhythmias and sudden death remain a major public health problem. Mutations of the K+ channel HERG, the human ether-a-gogo related gene, cause the long QT syndrome (LQTS), a genetic disorder characterized by lethal ventricular ..
  38. A Mouse Model of Mammalian Shaker Channel RNA Editing
    Tarun Bhalla; Fiscal Year: 2006
    ..The studies outlined below aim to understand the functional consequences of RNA editing of Kv1.1 in the mammalian nervous system. ..
  39. MOLECULAR DETERMINANTS OF POTASSIUM CHANNEL DRUG BLOCK
    Dirk Snyders; Fiscal Year: 2003
    ..5, kv4.2, herg, kvlqt1)...
  40. Study of Ventricular Repolarization in Long QT Syndrome
    Wojciech Zareba; Fiscal Year: 2004
    ..However, there is a substantial overlap of ECG patterns and clinical manifestation between LQT1 and LQT2 types caused by mutations of two different potassium channel genes (KVLQT1 and HERG, respectively)...
  41. Reduced expression of 2 voltage-gated potassium ion-channels in prostate cancer.
    Naseema Hoosein; Fiscal Year: 2009
    ..3 (a Shaker-type channel) and human ether-a-go-go-related gene (HERG, Kv11.1). Strong Kv1...
  42. Chemical Basis for Potassium Channel Modulation
    PAMELA ENGLAND; Fiscal Year: 2006
    ..research seeks to characterize several potential modes of modulation of the human ether-a-go-go related gene (HERG) ion channel, a voltage-gated potassium channel found in brain and heart...
  43. hERG K+ channel trafficking: the role of Cog4
    ADAM RUCKER; Fiscal Year: 2005
    b>hERG voltage-gated potassium channels have been implicated in the determination of resting membrane potential, cell differentiation and spike-frequency adaptation in CNS neurons...
  44. Insulin as a Neuromodulator in the Olfactory Bulb
    David Marks; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  45. Microfluidic patch clamp chips for multi-unit, high-throughput recordings
    Albert Folch; Fiscal Year: 2010
    ..that all drug candidates usually pools of >10,000 compounds be patch clamp-tested for their effects on the hERG channel).Ion channels play key roles in all known brain functions...
  46. INTERCELLULAR COMMUNICATION AND IMPULSE PROPAGATION
    JOSE S JALIFE; Fiscal Year: 2010
    ..Our main focus is the manner in which the strong inward rectifier Kir2.1 (KCNJ2) and the delayed rectifier HERG (KCHN2) and KyLQT1 (KCNQ1)/mink (KCNE1) modify the ability of cardiac electrical waves to propagate through non-..
  47. Markov Models of Potassium Channel Gating
    Randall L Rasmusson; Fiscal Year: 2012
    ..4, Kv4.3, KvLQT1 and HERG gating based on known and hypothesized structure-function relationships and voltage clamp data...
  48. High Throughput Screening for Specific Inhibitors and Modulators of A Novel Potas
    Daniel Weinberger; Fiscal Year: 2013
    ..1 potassium channel, a brain selective isoform of the hERG1 potassium channel, has been shown to be increased in the brains of patients with schizophrenia, a genetic risk ..
  49. RHEEM ANGELA TOTAH; Fiscal Year: 2014
    ..drugs associated with torsade de pointes can be identified that strongly inhibit CYP2J2, but have minimal effect on hERG at physiological concentrations...
  50. Fabio Cerignoli; Fiscal Year: 2014
    ..g. hERG), and physiologically relevant tests are reserved for few candidates during the relatively late stages of ..
  51. Novel ion channel approaches to reentrant arrythymias
    Ira S Cohen; Fiscal Year: 2013
    ..3: Here, we hypothesize that overexpression of a mutant hERG with slowed deactivation kinetics should improve rate responsiveness and prolong ERP compared to APD...
  52. Mechanisms of serum electrolyte effects on block of HERG
    Alan Miller; Fiscal Year: 2006
    unreadable] DESCRIPTION (provided by applicant): The human ether-a-go-go-related gene (HERG) encodes a potassium channel expressed in the ventricular myocardium and is involved in terminating the ventricular action potential...
  53. Small Molecule Kv1.3 Blockers as New Therapeutics for Multiple Sclerosis
    Heike Wulff; Fiscal Year: 2007
    ..Lay: In multiple sclerosis the immune system attacks proteins in the brain of afflicted patients. The aim of this study is to identify and test new drugs that can suppress the white blood cells that are invading the brain. ..
  54. Role of KVS and MPS Subunits in Basic Neuronal Function
    Federico Sesti; Fiscal Year: 2007
    ..3 Aim: To characterize chemosensory neuron physiology in culture and in vivo and to define how KVS and MPS-1 proteins influence amphid neuron basic function. 4 Aim: To establish the superfamily of C. elegans MiRPs. ..
  55. MinK-related peptides(MiRPs): structure and function
    Steve Goldstein; Fiscal Year: 2004
    ..We then found that MiRP1 assembled with the pore-forming subunit HERG to reconstitute attributes of cardiac IKr channels...
  56. MOLECULAR MECHANISMS OF HERG POTASSIUM CHANNEL FUNCTION
    Peter Spector; Fiscal Year: 2001
    ..Block of IKr by certain medications causes long QT syndrome (LQT). The human IKr channel is encoded by the gene HERG, mutations in which are associated with inherited LQT...
  57. A High Throughput Assay for Cancer Drug Cardiotoxicity
    Barbara Wible; Fiscal Year: 2005
    ..The cardiac potassium channel hERG is a frequent target of unwanted drug interactions, often leading to acquired long QT syndrome in patients...
  58. MECHANISMS OF REPOLARIZATION-INDUCED ARRHYTHMIAS
    Guy Salama; Fiscal Year: 2002
    ..iii) A mouse with a targeted mutation of Merg1, the mouse homolog of HERG has a normal QT but is highly susceptible to PVT...
  59. Michael H Gelb; Fiscal Year: 2014
    ..cruzi infection. Selected compounds will be tested for hERG channel inhibition, human receptor profiling, and genotoxicity studies. 3) Screen for synergy partner drugs...
  60. Val S Goodfellow; Fiscal Year: 2014
    ..assays, excellent blood brain barrier penetration, and satisfactory in vitro safety profiles for CYP450s, hERG and genotoxicity...
  61. Development of a Chloroquine Replacement Drug
    MICHAEL KEVIN RISCOE; Fiscal Year: 2010
    ..in vitro tests to assess Pharmachins for specific interactions with biogenic amine transporters/receptors and for hERG channel inhibition, 5. To conduct toxicological assessment of PH128 (and another lead candidate) in mice, and 6...
  62. A Diarylheptanoid Scaffold to Treat Taopathies
    Chad A Dickey; Fiscal Year: 2013
    ..human plasma protein binding, water solubility testing, broad receptor and ion channel profiling including hERG, and initial pharmacokinetic testing in rats...
  63. ETHAN SAMUEL BURSTEIN; Fiscal Year: 2014
    ..studies for genetic toxicity (Ames assay, chromosomal aberration assay, rat micronucleus), cardiovascular toxicity (hERG channel inhibition, cardiovascular monitoring in conscious, telemetered dogs or monkeys) and repeat dose toxicity ..
  64. Azithromycin and Sudden Cardiac Death: Electrophysiologic Mechanisms
    Katherine T Murray; Fiscal Year: 2013
    ..will perform electrophysiologic studies to test the hypothesis that azithromycin alters ionic currents other than hERG to prolong repolarization and increase arrhythmia susceptibility...
  65. Angela M Brodie; Fiscal Year: 2014
    ..that this compound exhibits no genotoxicity in bacterial or mammalian test systems, minimal effect on the hERG mediated potassium currents in Human Embryonic Kidney cells and minimal effects on a panel of cytochrome P450 ..
  66. Novel Thioderivatives as Neuroprotective Anticonvulsants
    DOUGLAS ERIC BRENNEMAN; Fiscal Year: 2010
    ..number of tests will be conducted to assess solubility, drug permeability and potential cardiovascular liabilities (hERG channel interactions)...