KCNE3

Summary

Gene Symbol: KCNE3
Description: potassium voltage-gated channel, Isk-related family, member 3
Alias: HOKPP, HYPP, MiRP2, cardiac voltage-gated potassium channel accessory subunit, minK-related peptide 2, minimum potassium ion channel-related peptide 2, potassium channel subunit beta MiRP2, potassium voltage-gated channel subfamily E member 3, voltage-gated K+ channel subunit MIRP2
Species: human

Top Publications

  1. ncbi A mutation in the KCNE3 potassium channel gene is associated with susceptibility to thyrotoxic hypokalemic periodic paralysis
    Magnus R Dias da Silva
    Laboratory of Molecular Endocrinology, Division of Endocrinology, Department of Medicine, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Rua Pedro de Toledo 781, 04039 032 Sao Paulo, SP, Brazil
    J Clin Endocrinol Metab 87:4881-4. 2002
  2. ncbi Protease-activated receptor regulation of Cl- secretion in Calu-3 cells requires prostaglandin release and CFTR activation
    Melissa L Palmer
    Department of Physiology, 495 Animal Science Veterinary Medicine Bldg, 1988 Fitch Ave, University of Minnesota, St Paul, 55110, USA
    Am J Physiol Cell Physiol 290:C1189-98. 2006
  3. ncbi Stretch-sensitive KCNQ1 mutation A link between genetic and environmental factors in the pathogenesis of atrial fibrillation?
    Robyn Otway
    Sr Bernice Research Program in Inherited Heart Diseases, Darlinghurst, New South Wales, Australia
    J Am Coll Cardiol 49:578-86. 2007
  4. pmc Molecular determinants of cardiac transient outward potassium current (I(to)) expression and regulation
    Noriko Niwa
    Department of Developmental Biology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8103, St Louis, MO 63110 1093, USA
    J Mol Cell Cardiol 48:12-25. 2010
  5. doi Severe respiratory phenotype caused by a de novo Arg528Gly mutation in the CACNA1S gene in a patient with hypokalemic periodic paralysis
    Tae Hwan Kil
    Department of Pediatrics, College of Medicine, Konyang University, 685 Gasoowon Dong, Su goo, Daejun, Choongnam 302 718, South Korea
    Eur J Paediatr Neurol 14:278-81. 2010
  6. pmc Functional effects of KCNE3 mutation and its role in the development of Brugada syndrome
    Eva Delpón
    Department of Pharmacology, School of Medicine, Universidad Complutense, Madrid, Spain
    Circ Arrhythm Electrophysiol 1:209-18. 2008
  7. ncbi Up-regulation and increased activity of KV3.4 channels and their accessory subunit MinK-related peptide 2 induced by amyloid peptide are involved in apoptotic neuronal death
    A Pannaccione
    Division of Pharmacology, Department of Neuroscience, School of Medicine, University of Naples Federico II, Naples, Italy
    Mol Pharmacol 72:665-73. 2007
  8. doi Novel KCNE3 mutation reduces repolarizing potassium current and associated with long QT syndrome
    Seiko Ohno
    Department of Cardiovascular Medicine, Kyoto University Graduate School of Medicine, Kyoto, Japan
    Hum Mutat 30:557-63. 2009
  9. ncbi Ménière's disease is associated with single nucleotide polymorphisms in the human potassium channel genes, KCNE1 and KCNE3
    Katsumi Doi
    Department of Otolaryngology and Sensory Organ Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
    ORL J Otorhinolaryngol Relat Spec 67:289-93. 2005
  10. ncbi MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis
    G W Abbott
    Departments of Pediatrics and Cellular, Molecular Physiology, Boyer Center for Molecular Medicine, Yale University School of Medicine, New Haven, CT 06536, USA
    Cell 104:217-31. 2001

Research Grants

  1. Structural Basis for KCNE Modulation of the KCNQ1 Channel
    Carlos G Vanoye; Fiscal Year: 2010
  2. MinK-related peptides(MiRPs): structure and function
    Steve Goldstein; Fiscal Year: 2004
  3. Molecular Physiology of KCNE Potassium Channel Subunits
    ALFRED GEORGE; Fiscal Year: 2007
  4. Modulation of cardiac K+ channels by drugs
    Michael Sanguinetti; Fiscal Year: 2004
  5. Regulation of Kv3.1 by MiRPs in Auditory Neurons
    GEOFFREY ABBOTT; Fiscal Year: 2006
  6. Molecular Basis of Automaticity in the Myometrium
    Glenna Bett; Fiscal Year: 2007

Scientific Experts

Detail Information

Publications99

  1. ncbi A mutation in the KCNE3 potassium channel gene is associated with susceptibility to thyrotoxic hypokalemic periodic paralysis
    Magnus R Dias da Silva
    Laboratory of Molecular Endocrinology, Division of Endocrinology, Department of Medicine, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Rua Pedro de Toledo 781, 04039 032 Sao Paulo, SP, Brazil
    J Clin Endocrinol Metab 87:4881-4. 2002
    ..FHypoKPP is caused by mutations in ionic channel genes calcium (CACN1AS), sodium (SCN4A) and potassium (KCNE3)...
  2. ncbi Protease-activated receptor regulation of Cl- secretion in Calu-3 cells requires prostaglandin release and CFTR activation
    Melissa L Palmer
    Department of Physiology, 495 Animal Science Veterinary Medicine Bldg, 1988 Fitch Ave, University of Minnesota, St Paul, 55110, USA
    Am J Physiol Cell Physiol 290:C1189-98. 2006
    ..for CFTR, the Ca(2+)-activated KCNN4 K(+) channel, and the KCNQ1 K(+) channel subunit, which, in association with KCNE3, is known to be regulated by cAMP...
  3. ncbi Stretch-sensitive KCNQ1 mutation A link between genetic and environmental factors in the pathogenesis of atrial fibrillation?
    Robyn Otway
    Sr Bernice Research Program in Inherited Heart Diseases, Darlinghurst, New South Wales, Australia
    J Am Coll Cardiol 49:578-86. 2007
    ..This study sought to evaluate mutations in genes encoding the slow component of the cardiac delayed rectifier K+ current (I(Ks)) channel in familial atrial fibrillation (AF)...
  4. pmc Molecular determinants of cardiac transient outward potassium current (I(to)) expression and regulation
    Noriko Niwa
    Department of Developmental Biology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8103, St Louis, MO 63110 1093, USA
    J Mol Cell Cardiol 48:12-25. 2010
    ..Indeed, a recently identified Brugada syndrome mutation in KCNE3 (MiRP2) has been suggested to result in increased I(t)(o,f) densities...
  5. doi Severe respiratory phenotype caused by a de novo Arg528Gly mutation in the CACNA1S gene in a patient with hypokalemic periodic paralysis
    Tae Hwan Kil
    Department of Pediatrics, College of Medicine, Konyang University, 685 Gasoowon Dong, Su goo, Daejun, Choongnam 302 718, South Korea
    Eur J Paediatr Neurol 14:278-81. 2010
    Hypokalemic periodic paralysis (HOKPP) is a rare disorder characterized by episodic muscle weakness with hypokalemia...
  6. pmc Functional effects of KCNE3 mutation and its role in the development of Brugada syndrome
    Eva Delpón
    Department of Pharmacology, School of Medicine, Universidad Complutense, Madrid, Spain
    Circ Arrhythm Electrophysiol 1:209-18. 2008
    ..Although the transient outward current (I(to)) is thought to play a prominent role in the expression of the syndrome, mutations in I(to)-related genes have not been identified as yet...
  7. ncbi Up-regulation and increased activity of KV3.4 channels and their accessory subunit MinK-related peptide 2 induced by amyloid peptide are involved in apoptotic neuronal death
    A Pannaccione
    Division of Pharmacology, Department of Neuroscience, School of Medicine, University of Naples Federico II, Naples, Italy
    Mol Pharmacol 72:665-73. 2007
    ..4 channel subunit and its accessory subunit, MinK-related peptide 2 (MIRP2); 2) whether the increase in K(V)3...
  8. doi Novel KCNE3 mutation reduces repolarizing potassium current and associated with long QT syndrome
    Seiko Ohno
    Department of Cardiovascular Medicine, Kyoto University Graduate School of Medicine, Kyoto, Japan
    Hum Mutat 30:557-63. 2009
    ..The third KCNE gene, KCNE3, is expressed in cardiac myocytes and interacts with KCNQ1 to change the channel properties...
  9. ncbi Ménière's disease is associated with single nucleotide polymorphisms in the human potassium channel genes, KCNE1 and KCNE3
    Katsumi Doi
    Department of Otolaryngology and Sensory Organ Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
    ORL J Otorhinolaryngol Relat Spec 67:289-93. 2005
    ..The SNPs analyses identified 112G/A SNP in the KCNE1 gene and 198T/C SNP in the KCNE3 gene in 63 definite MD cases as well as 205 and 237 non-MD control subjects...
  10. ncbi MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysis
    G W Abbott
    Departments of Pediatrics and Cellular, Molecular Physiology, Boyer Center for Molecular Medicine, Yale University School of Medicine, New Haven, CT 06536, USA
    Cell 104:217-31. 2001
    The subthreshold, voltage-gated potassium channel of skeletal muscle is shown to contain MinK-related peptide 2 (MiRP2) and the pore-forming subunit Kv3.4. MiRP2-Kv3.4 channels differ from Kv3...
  11. ncbi Lack of association of the potassium channel-associated peptide MiRP2-R83H variant with periodic paralysis
    D Sternberg
    Federations of Biochemistry, Centre Hospitalier Universitaire Pitie Salpetriere, Assistance Publique Hôpitaux de Paris et Université Paris VI, Paris, France
    Neurology 61:857-9. 2003
    ..variant (R83H) of the gene (KCNE3) encoding a potassium channel-associated peptide, MinK-related peptide 2 (MiRP2), has been reported in periodic paralysis patients...
  12. doi Polymorphisms in KCNE1 or KCNE3 are not associated with Ménière disease in the Caucasian population
    Colleen A Campbell
    Molecular Otolaryngology Research Laboratories, Department of Otolaryngology Head and Neck Surgery, University of Iowa, Iowa City, IA 52242, USA
    Am J Med Genet A 152:67-74. 2010
    ..Relat Spec 67:289-293] recently reported that two single nucleotide polymorphisms (SNPs) in KCNE1 and KCNE3 are associated with MD in Japanese subjects...
  13. ncbi Periodic paralysis mutation MiRP2-R83H in controls: Interpretations and general recommendation
    Karin Jurkat-Rott
    Department of Applied Physiology, Ulm University, Germany
    Neurology 62:1012-5. 2004
    An R83H point mutation in KCNE3-encoded MiRP2 has been reported to cause 2% of all cases of familial periodic paralysis. The authors found MiRP2-R83H in 3 of 321 control subjects and in 5 unaffected related individuals...
  14. doi KCNE3 mutation V17M identified in a patient with lone atrial fibrillation
    Alicia Lundby
    The Danish National Research Foundation Centre for Cardiac Arrhythmia, The Panum Institute, University of Copenhagen, Copenhagen N, Denmark
    Cell Physiol Biochem 21:47-54. 2008
    ..In this study we aim for the functional assessment of a mutation in KCNE3 identified in a proband with early-onset lone AF.
  15. ncbi The alpha(1S) subunit of the L-type calcium channel is not a predisposition gene for thyrotoxic periodic paralysis
    Nelson L S Tang
    Department of Chemical Pathology, Faculty of Medicine, The Chinese Univerisity of Hong Kong SAR, China
    Clin Endocrinol (Oxf) 66:229-34. 2007
    ..Mutations in CACNA1S are known to account for the majority of cases of familial hypokalaemic periodic paralysis (HOKPP)...
  16. ncbi Genotype-phenotype correlation and therapeutic rationale in hyperkalemic periodic paralysis
    Karin Jurkat-Rott
    Department of Applied Physiology, Ulm University, Ulm, Germany
    Neurotherapeutics 4:216-24. 2007
    ..Last, because hyperkalemic PP has been described to be associated with an R83H mutation of a MiRP2 potassium channel subunit, evidence refuting disease-causality in this case will be discussed.
  17. ncbi Regulation and properties of KCNQ1 (K(V)LQT1) and impact of the cystic fibrosis transmembrane conductance regulator
    A Boucherot
    Department of Physiology and Pharmacology, University of Queensland, St Lucia, QLD 4072, Australia
    J Membr Biol 182:39-47. 2001
    ..KCNQ1 (K(V)LQT1) is a voltage-gated K+ channel, coexpressed with regulatory subunits such as KCNE1 (IsK, mink) or KCNE3, depending on the tissue examined...
  18. ncbi The cardiac K+ channel KCNQ1 is essential for gastric acid secretion
    F Grahammer
    Institute of Physiology, Albert Ludwigs University, Freiburg, Germany
    Gastroenterology 120:1363-71. 2001
    ..However, the molecular identity of luminal K+ channels enabling K+ recycling in parietal cells is unknown. This study was aimed to investigate these luminal K+ channels...
  19. ncbi KCNE2 modulates current amplitudes and activation kinetics of HCN4: influence of KCNE family members on HCN4 currents
    Niels Decher
    Department of Physiology, University of Utah, Eccles Institute of Human Genetics, 15 N 2030 E, Salt Lake City, UT 84112, USA
    Pflugers Arch 446:633-40. 2003
    ..In contrast, the related family members KCNE1, KCNE3 and KCNE4 did not change current characteristics of HCN4...
  20. pmc KCNE4 domains required for inhibition of KCNQ1
    Lauren J Manderfield
    Department of Pharmacology, Department of Medicine, Vanderbilt University, Nashville, TN 37232 0275, USA
    J Physiol 587:303-14. 2009
    ..has a dramatic inhibitory effect on KCNQ1 that differs substantially from the activating effects of KCNE1 and KCNE3. The structural features of KCNE4 that enable this behaviour are unknown...
  21. ncbi Expression of multiple KCNE genes in human heart may enable variable modulation of I(Ks)
    Andrew L Lundquist
    Department of Pharmacology, Vanderbilt University School of Medicine, Nashville, TN 37232, USA
    J Mol Cell Cardiol 38:277-87. 2005
    ..genes are expressed in human heart with a relative abundance ranking of KCNE1 > KCNE4 > KCNE5 approximately KCNE3 >> KCNE2. In situ hybridization revealed prominent expression of KCNE1 and KCNE3-5 in human atrial myocytes...
  22. ncbi The small conductance K+ channel, KCNQ1: expression, function, and subunit composition in murine trachea
    F Grahammer
    Institute of Physiology, Albert Ludwigs Universitat, Hermann Herder Strabetae 7, D 79104 Freiburg, Germany
    J Biol Chem 276:42268-75. 2001
    ..In large and small intestine a channel complex consisting of KCNQ1 and the beta-subunit KCNE3 (MiRP2) is known to mediate the cAMP-activated basolateral K(+) current, which is essential for luminal Cl(-) secretion...
  23. ncbi Divergent expression of delayed rectifier K(+) channel subunits during mouse heart development
    D Franco
    Experimental Molecular Cardiology Group, AMC, University of Amsterdam, Amsterdam, The Netherlands
    Cardiovasc Res 52:65-75. 2001
    ..subunits KCNQ1 and KCNH2, respectively, in association with regulatory beta-subunit KCNE1, KCNE2 and perphaps KCNE3. In the present study we have investigated the distribution of transcripts encoding these five potassium channel-..
  24. ncbi An arrhythmia susceptibility gene in Caenorhabditis elegans
    Ki Ho Park
    University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, Department of Physiology and Biophysics, Piscataway, New Jersey 08854, USA
    J Biol Chem 282:19799-807. 2007
    ..Missense mutations in kcne1, kcne2, and kcne3 are linked to congenital and acquired channelopathies in Homo sapiens...
  25. pmc KCNE1 constrains the voltage sensor of Kv7.1 K+ channels
    Liora Shamgar
    Department of Physiology and Pharmacology, Sackler Medical School, Tel Aviv University, Tel Aviv, Israel
    PLoS ONE 3:e1943. 2008
    ..1 with either KCNE1 or KCNE3. Many S4 perturbations compromise the ability of KCNE1 to properly regulate Kv7.1 channel gating...
  26. doi Cell cycle-dependent expression of Kv3.4 channels modulates proliferation of human uterine artery smooth muscle cells
    Eduardo Miguel-Velado
    Departamento de Bioquímica y Biología Molecular y Fisiología e Instituto de Biología y Genética Molecular, Universidad de Valladolid y CSIC, Valladolid, Spain
    Cardiovasc Res 86:383-91. 2010
    ..4 channels with proliferation of human uterine VSMCs. Here, we sought to gain deeper insight on the relationship between Kv3.4 channels and cell cycle progression in this preparation...
  27. ncbi Electrophysiological and molecular identification of hepatocellular volume-activated K+ channels
    W Z Lan
    GI Diseases Research Unit, Hotel Dieu Hospital and Queen s University, Kingston, Ontario, Canada K7L 5G2
    Biochim Biophys Acta 1668:223-33. 2005
    ..detected the expression of members of the KCNQ family from KCNQ1 to KCNQ5 and of the accessory proteins KCNE1 to KCNE3 in the rat hepatocytes, but not KCNQ2 and KCNE2 in human liver...
  28. ncbi [The mutation R672H in SCN4A gene exists in Chinese patients with hypokalaemic periodic paralysis]
    Qing Ke
    Department of Neurology, General Hospital of PLA, Beijing 100853, China
    Zhonghua Yi Xue Za Zhi 86:724-7. 2006
    Mutation screening was performed on two Chinese families with HOKPP to locat the corresponding mutations and to specify the clinical features associated with the mutation.
  29. ncbi [KCNE3 R53H substitution in familial atrial fibrillation]
    Dai Fu Zhang
    Department of Cardiology, East Hospital, Shanghai 200120, China
    Chin Med J (Engl) 118:1735-8. 2005
  30. ncbi MinK, MiRP1, and MiRP2 diversify Kv3.1 and Kv3.2 potassium channel gating
    Anthony Lewis
    Division of Cardiology, Department of Medicine, Weill Medical College of Cornell University, New York, New York 10021, USA
    J Biol Chem 279:7884-92. 2004
    ..Kv3.1 alpha subunits can also form slower activating channels by coassembling with MinK-related peptide 2 (MiRP2), a single transmembrane domain potassium channel ancillary subunit...
  31. pmc Dynamic subunit stoichiometry confers a progressive continuum of pharmacological sensitivity by KCNQ potassium channels
    Haibo Yu
    The Solomon H Snyder Department of Neuroscience, High Throughput Biology Center and Johns Hopkins Ion Channel Center, Johns Hopkins University, Baltimore, MD 21205, USA
    Proc Natl Acad Sci U S A 110:8732-7. 2013
    ..Progressive increase of KCNE1 or KCNE3 expression reduces efficacy of ML277 and eventually abolishes ML277-mediated augmentation...
  32. pmc P2Y6 receptor mediates colonic NaCl secretion via differential activation of cAMP-mediated transport
    Michael Köttgen
    Medizinische Universitatsklinik, Freiburg, Germany
    J Clin Invest 111:371-9. 2003
    ..Cl(-) secretion, which was completely inhibited by 293B, a specific inhibitor of cAMP-stimulated basolateral KCNQ1/KCNE3 K(+) channels. We therefore speculated that a basolateral P2Y(6) receptor could increase cAMP...
  33. ncbi Genetic and clinical aspects of Brugada syndrome: an update
    Giuseppe Lippi
    U O Diagnostica Ematochimica, Dipartimento di Patologia e Medicina di Laboratorio, Azienda Ospedaliero Universitaria di Parma, Parma, Italy
    Adv Clin Chem 56:197-208. 2012
    ..BS (from BS1 to BS8) have already been described, involving mutations in SCN5A, GPD1-L, CACNA1c, CACNB2b, SCN1B, KCNE3, SCN3B, and HCN4 genes. The vast majority (i.e...
  34. ncbi Electrolyte transport in the mouse trachea: no evidence for a contribution of luminal K(+) conductance
    R Schreiber
    Department of Physiology and Pharmacology, University of Queensland, St Lucia, QLD 4072, Brisbane, Australia
    J Membr Biol 189:143-51. 2002
    ..Similarly, the compound 293B, a blocker of basolateral KCNQ1/KCNE3 K(+) channels effectively blocked Cl(-) secretion when applied to either the luminal or basolateral side of the ..
  35. ncbi KCNE3 T4A as the genetic basis of Brugada-pattern electrocardiogram
    Tadashi Nakajima
    Department of Medicine and Biological Science, Gunma University Graduate School of Medicine, Maebashi, Japan
    Circ J 76:2763-72. 2012
    ..Brugada syndrome (BrS) is genetically heterogeneous. In Japanese BrS patients, except for SCN5A and KCNE5, mutations in the responsible genes have not yet been identified, and therefore the genetic heterogeneity remains poorly elucidated...
  36. doi West Nile virus protease activity in detergent solutions and application for affinity tag removal
    Qiwei Huang
    Experimental Therapeutics Centre, Agency for Science, Technology and Research A STAR, Singapore 138669, Singapore
    Anal Biochem 435:44-6. 2013
    ..In a detergent solution, the WNV protease can remove the fusion tag from a recombinant protein containing KCNE3 and a WNV protease site...
  37. pmc Oestrogen promotes KCNQ1 potassium channel endocytosis and postendocytic trafficking in colonic epithelium
    Raphaël Rapetti-Mauss
    Department of Molecular Medicine, RCSI ERC, Beaumont Hospital, PO Box 9063, Dublin 9, Ireland
    J Physiol 591:2813-31. 2013
    The cAMP-regulated potassium channel KCNQ1:KCNE3 plays an essential role in transepithelial Cl(-) secretion. Recycling of K(+) across the basolateral membrane provides the driving force necessary to maintain apical Cl(-) secretion...
  38. ncbi A single transmembrane site in the KCNE-encoded proteins controls the specificity of KvLQT1 channel gating
    Yonathan F Melman
    Section of Molecular Cardiology, Departments of Medicine and Molecular Pharmacology, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    J Biol Chem 277:25187-94. 2002
    ..The specificity of KCNE1 (minK) and KCNE3 control of activation of the potassium channel KvLQT1 maps to a triplet of amino acids within the KCNE ..
  39. doi A replication study on proposed candidate genes in Ménière's disease, and a review of the current status of genetic studies
    Elina Hietikko
    Oulu Center for Cell Matrix Research, Biocenter and Department of Medical Biochemistry and Molecular Biology, Institute of Biomedicine, University of Oulu, Finland
    Int J Audiol 51:841-5. 2012
    ..We review here all the previously proposed candidate genes for MD and report our results on the analysis of six such genes, AQP2, KCNE1, KCNE3, HCFC1, COCH, and ADD1.
  40. doi Molecular architecture of the human sinus node: insights into the function of the cardiac pacemaker
    Natalie J Chandler
    Cardiovascular Research Group, Faculty of Medical and Human Sciences, University of Manchester, Core Technology Facility, 46 Grafton St, Manchester M139NT, United Kingdom
    Circulation 119:1562-75. 2009
    ..The aims of the present study were to investigate the expression of ion channels in the human SN and to use the data to predict electrical activity...
  41. pmc Hypokalemic periodic paralysis; two different genes responsible for similar clinical manifestations
    Hunmin Kim
    Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea
    Korean J Pediatr 54:473-6. 2011
    Primary hypokalemic periodic paralysis (HOKPP) is an autosomal dominant disorder manifesting as recurrent periodic flaccid paralysis and concomitant hypokalemia. HOKPP is divided into type 1 and type 2 based on the causative gene...
  42. doi The genetic basis of Brugada syndrome: a mutation update
    Paula L Hedley
    Department of Clinical Biochemistry and Immunology, Statens Serum Institut, Copenhagen, Denmark
    Hum Mutat 30:1256-66. 2009
    ..2 ion channel; SCN1B and SCN3B, which, in the heart, encodes beta-subunits of the Na(v)1.5 sodium ion channel, and KCNE3, which encodes the ancillary inhibitory beta-subunit of several potassium channels including the Kv4...
  43. pmc Functional delivery of a membrane protein into oocyte membranes using bicelles
    Congbao Kang
    Department of Biochemistry, Center for Structural Biology, Vanderbilt University School of Medicine, Nashville, Tennessee 37232 8725, USA
    Biochemistry 49:653-5. 2010
    Voltage-gated potassium channel modulatory membrane protein KCNE3 was overexpressed and purified into both micelles and bicelles...
  44. doi Thyrotoxic periodic paralysis in an Italian man: clinical manifestation and genetic analysis
    Francesco Vendrame
    Diabetes Research Institute, Miller School of Medicine, University of Miami, Miami, FL 33136, USA
    Ann Clin Biochem 45:218-20. 2008
    ..Genetic analysis showed no mutations in the candidate exons of calcium (CACN1AS), potassium (KCNE3) and sodium (SCN4A) channel genes...
  45. pmc KCNE1 and KCNE3 beta-subunits regulate membrane surface expression of Kv12.2 K(+) channels in vitro and form a tripartite complex in vivo
    Sinead M Clancy
    Department of Cell Biology, Institute for Childhood and Neglected Diseases, The Scripps Research Institute, La Jolla, CA, USA
    PLoS ONE 4:e6330. 2009
    ..Here we demonstrate that the auxiliary MinK (KCNE1) and MiRP2 (KCNE3) proteins are important regulators of Kv12.2 channel function...
  46. ncbi Autonomously functioning thyroid nodule associated with thyrotoxic periodic paralysis
    Hiroshi Ozaki
    Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan
    Endocr J 55:113-9. 2008
    ..TSHR), the alpha-subunit of the stimulatory G protein (Gsalpha), calcium channel CACNA1S and potassium channel KCNE3, and found that the patient does not carry the known mutations in these genes...
  47. pmc Sexual dimorphism and oestrogen regulation of KCNE3 expression modulates the functional properties of KCNQ1 K⁺ channels
    Rodrigo Alzamora
    Department of Molecular Medicine, Education and Research Centre, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin, Republic of Ireland
    J Physiol 589:5091-107. 2011
    ..Co-assembly with KCNE3 produces a current with nearly instantaneous activation, some time-dependent activation at very positive ..
  48. ncbi Serum- and glucocorticoid-inducible kinases (SGK) regulate KCNQ1/KCNE potassium channels
    Nathalie Strutz-Seebohm
    Department of Physiology I, University of Tubingen, Tubingen, Germanyå
    Channels (Austin) 3:88-90. 2009
    ..Apart from KCNE1, several other KCNE beta subunits including KCNE3 and KCNE5 have been detected at the mRNA level in cardiac tissue as well as in the inner ear and the gastro-..
  49. pmc Deep resequencing of the voltage-gated potassium channel subunit KCNE3 gene in chronic tinnitus
    Philipp G Sand
    Department of Otorhinolaryngology, University of Regensburg, Franz Josef Strauss Allee 11, Regensburg, Germany
    Behav Brain Funct 7:39. 2011
    ..The present study addresses the potassium channel subunit gene KCNE3 as a potential candidate for tinnitus susceptibility...
  50. pmc Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport
    Patricia Preston
    Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, 13125 Berlin, Germany
    J Biol Chem 285:7165-75. 2010
    The KCNE3 beta-subunit constitutively opens outwardly rectifying KCNQ1 (Kv7.1) K(+) channels by abolishing their voltage-dependent gating...
  51. pmc A shared mechanism for lipid- and beta-subunit-coordinated stabilization of the activated K+ channel voltage sensor
    Eun Choi
    Department of Pharmacology, Weill Medical College of Cornell University, 1300 York Ave, New York, NY 10021, USA
    FASEB J 24:1518-24. 2010
    ..activation when in complexes with transmembrane beta subunits, MinK-related peptide (MiRP) 1 (KCNE2) or MiRP2 (KCNE3)...
  52. ncbi Bacterial synthesis, purification, and solubilization of membrane protein KCNE3, a regulator of voltage-gated potassium channels
    S A Goncharuk
    Shemyakin and Ovchinnikov Institute of Bioorganic Chemistry, Russian Academy of Sciences, Moscow, 117997, Russia
    Biochemistry (Mosc) 74:1344-9. 2009
    An efficient method is described for production of membrane protein KCNE3 and its isotope labeled derivatives ((15)N-, (15)N-/13C-) in amounts sufficient for structural-functional investigations...
  53. doi Functional implications of KCNE subunit expression for the Kv7.5 (KCNQ5) channel
    Meritxell Roura-Ferrer
    Departament de Bioquimica i Biologia Molecular, Universitat de Barcelona, Spain
    Cell Physiol Biochem 24:325-34. 2009
    ..An extensive study in Xenopus oocytes and HEK-293 cells demonstrates that KCNE1 and KCNE3, but none of the other KCNE subunits, affect Kv7.5 currents...
  54. doi Impact of KCNE subunits on KCNQ1 (Kv7.1) channel membrane surface targeting
    Meritxell Roura-Ferrer
    Molecular Physiology Laboratory, Departament de Bioquimica i Biologia Molecular, Institut de Biomedicina IBUB, Universitat de Barcelona, Barcelona, Spain
    J Cell Physiol 225:692-700. 2010
    ..Only KCNQ1 and KCNE3, when expressed alone, co-localized in raft fractions...
  55. pmc Genetic dissection reveals unexpected influence of beta subunits on KCNQ1 K+ channel polarized trafficking in vivo
    Torsten K Roepke
    Department of Pharmacology, Weill Medical College of Cornell University, New York, NY 10021, USA
    FASEB J 25:727-36. 2011
    ..Quantitative RT-PCR and Western blotting revealed that Kcne2 deletion remodeled fundic Kcne3 (2.9±0.8-fold mRNA increase, n=10; 5.3±0...
  56. pmc Novel female sex-dependent actions of oestrogen in the intestine
    Fiona O'Mahony
    Royal College of Surgeons in Ireland, Department of Molecular Medicine, Dublin 17, Ireland
    J Physiol 587:5039-44. 2009
    ..Work from our laboratory has identified the KCNQ1/KCNE3 channel as one of the principal targets for oestrogen-induced signalling cascades in the distal colon...
  57. pmc Distinct subdomains of the KCNQ1 S6 segment determine channel modulation by different KCNE subunits
    Carlos G Vanoye
    Division of Genetic Medicine, Department of Medicine, Center for Structural Biology, Vanderbilt University, Nashville, TN 37232, USA
    J Gen Physiol 134:207-17. 2009
    ..This dipeptide motif is distinct from neighboring S6 sequences that enable modulation by KCNE1 and KCNE3. Conversely, S6 mutations (S338C and F340C) that alter KCNE1 and KCNE3 effects on KCNQ1 do not abrogate KCNE4 ..
  58. pmc Extracellular potassium inhibits Kv7.1 potassium channels by stabilizing an inactivated state
    Anders Peter Larsen
    The Danish National Research Foundation Centre for Cardiac Arrhythmia, Department of Biomedical Sciences, University of Copenhagen, Copenhagen, Denmark
    Biophys J 101:818-27. 2011
    ..1 channels. The effect of extracellular potassium was absent in noninactivating Kv7.1/KCNE1 and Kv7.1/KCNE3 channels, further supporting a stabilized inactivated state as the underlying mechanism...
  59. doi Endocannabinoids and cannabinoid analogues block human cardiac Kv4.3 channels in a receptor-independent manner
    Irene Amorós
    Department of Pharmacology, School of Medicine, Universidad Complutense de Madrid, 28040 Madrid, Spain
    J Mol Cell Cardiol 48:201-10. 2010
    ..by modifications in the lipid order and microviscosity of the membrane and were independent of the presence of MiRP2 or DPP6 subunits in the channel complex...
  60. pmc KCNE peptides differently affect voltage sensor equilibrium and equilibration rates in KCNQ1 K+ channels
    Jessica M Rocheleau
    Department of Biochemistry and Molecular Pharmacology, University of Massachusetts, Worcester, MA 01605, USA
    J Gen Physiol 131:59-68. 2008
    ..KCNQ1/KCNE1 complexes generate the very slowly activating cardiac I(Ks) current, whereas assembly with KCNE3 produces a constitutively conducting complex involved in K(+) recycling in epithelia...
  61. ncbi KCNQ1 mutation Q147R is associated with atrial fibrillation and prolonged QT interval
    Alicia Lundby
    Danish National Research Foundation Centre for Cardiac Arrhythmia, Department of Biomedical Sciences, The Panum Institute, University of Copenhagen, Copenhagen, Denmark
    Heart Rhythm 4:1532-41. 2007
    ..Atrial fibrillation (AF) and long QT syndrome (LQTS) are cardiac arrhythmia disorders that have been related to dysfunction of the voltage-gated potassium channel subunit Kv7.1 encoded by the KCNQ1 gene...
  62. ncbi In vitro molecular interactions and distribution of KCNE family with KCNQ1 in the human heart
    Said Bendahhou
    Institut de Pharmacologie Moleculaire et Cellulaire, UMR 6097 CNRS and Université de Nice Sophia Antipolis, 660 route des Lucioles, Sophia Antipolis, 06560 Valbonne, France
    Cardiovasc Res 67:529-38. 2005
    ..The expression level and the putative functions of the different KCNE subunits in the human heart still require further investigation...
  63. ncbi K+ channel KVLQT1 located in the basolateral membrane of distal colonic epithelium is not essential for activating Cl- secretion
    Tianjiang Liao
    Dept of Neuroscience, Cell Biology and Physiology, Wright State University, Dayton, OH 45435, USA
    Am J Physiol Cell Physiol 289:C564-75. 2005
    ..and then probed with antibodies to the K(+) channel proteins K(V)LQT1 (Kcnq1) and minK-related peptide 2 (MiRP2, Kcne3)...
  64. ncbi Manifestation, management and molecular analysis of candidate genes in two rare cases of thyrotoxic hypokalemic periodic paralysis
    Camilla Schalin-Jantti
    Division of Endocrinology, Department of Medicine, University of Helsinki, Finland
    Horm Res 63:139-44. 2005
    ..Ion channel gene mutations may underlie these diseases. We describe the first pediatric and a rare adult Caucasian case of THypoKPP in Finland...
  65. ncbi Correlating phenotype and genotype in the periodic paralyses
    T M Miller
    Department of Neurology, University of California San Francisco 94143 2922, USA
    Neurology 63:1647-55. 2004
    ..without one of the classically known mutations, the authors analyzed the entire coding region of the SCN4A, KCNE3, and KCNJ2 genes and portions of the coding region of the CACNA1S gene in order to identify new mutations...
  66. pmc Identification of a KCNE2 gain-of-function mutation in patients with familial atrial fibrillation
    Yiqing Yang
    Department of Cardiology, Tongji Hospital, Shanghai, China
    Am J Hum Genet 75:899-905. 2004
    ..28 unrelated Chinese kindreds with AF and sequenced eight genes of potassium channels (KCNQ1, HERG, KCNE1, KCNE2, KCNE3, KCNE4, KCNE5, and KCNJ2)...
  67. ncbi No mutation in the KCNE3 potassium channel gene in Chinese thyrotoxic hypokalaemic periodic paralysis patients
    Nelson L S Tang
    Department of Chemical Pathology, Faculty of Medicine, The Chinese University of Hong Kong, Kong Kong
    Clin Endocrinol (Oxf) 61:109-12. 2004
    Mutation in KCNE3 gene (Isk-related family potassium voltage-gated channel member 3 gene) was recently associated with the aetiology of thyrotoxic periodic paralysis (TPP)...
  68. pmc Mechanisms of anion secretion in Calu-3 human airway epithelial cells by 7,8-benzoquinoline
    A W Cuthbert
    Department of Medicine, University of Cambridge, Addenbrooke s Hospital Level 5, Box 157, Hills Road, Cambridge CB2 2QQ
    Br J Pharmacol 140:81-90. 2003
    ..intermediate-conductance calcium-sensitive potassium channel (KCNN4) and a cAMP-sensitive potassium channel (KCNQ1/KCNE3) in the basolateral epithelial membranes...
  69. ncbi KCNQ1 gain-of-function mutation in familial atrial fibrillation
    Yi Han Chen
    Department of Cardiology, Tongji Hospital, and Institute of Medical Genetics, Tongji University, 399 Xin Cun Road, Shanghai 200065, People s Republic of China
    Science 299:251-4. 2003
    ..encodes the pore-forming alpha subunit of the cardiac I(Ks) channel (KCNQ1/KCNE1), the KCNQ1/KCNE2 and the KCNQ1/KCNE3 potassium channels...
  70. pmc Ectopic expression of KCNE3 accelerates cardiac repolarization and abbreviates the QT interval
    Reza Mazhari
    Department of Medicine, Institute of Molecular Cardiobiology, Johns Hopkins University, 720 Rutland Avenue, Baltimore, MD 21205, USA
    J Clin Invest 109:1083-90. 2002
    Regulatory subunit KCNE3 (E3) interacts with KCNQ1 (Q1) in epithelia, regulating its activation kinetics and augmenting current density...
  71. pmc Characterization of basolateral K+ channels underlying anion secretion in the human airway cell line Calu-3
    Elizabeth A Cowley
    Department of Physiology and Biophysics, Dalhousie University, Halifax, Nova Scotia, Canada B3H 4H7
    J Physiol 538:747-57. 2002
    ..chain reaction, we found that Calu-3 cells express the K+ channel genes KCNN4 and KCNQ1 and the subunits KCNE2 and KCNE3. We conclude that while KCNN4 contributes to Ca2+-activated anion secretion by Calu-3 cells, basal and cAMP-..
  72. doi KCNQ1 K+ channels are involved in lipopolysaccharide-induced apoptosis of distal kidney cells
    Christophe Duranton
    CNRS FRE 3093, Transport Ionique, Aspects Normaux et Pathologiques Université de Nice Sophia Antipolis, Nice, France
    Cell Physiol Biochem 25:367-78. 2010
    ..biophysical properties of the current suggest that the KCNQ1 pore-forming subunit is associated with a KCNE2 or KCNE3 ancillary subunit...
  73. ncbi KCNQ potassium channels: physiology, pathophysiology, and pharmacology
    J Robbins
    Sensory Function Group, Centre for Neuroscience Research, King s College, Guy s Campus, London SE1 1UL, UK
    Pharmacol Ther 90:1-19. 2001
    ..KCNQ1 can also co-assemble with KCNE3, and may be the molecular correlate of the cyclic AMP-regulated K(+) current present in colonic crypt cells...
  74. ncbi Structural determinants of KvLQT1 control by the KCNE family of proteins
    Y F Melman
    Section of Molecular Cardiology, Department of Medicine, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    J Biol Chem 276:6439-44. 2001
    ..The emerging family of KCNE1-related peptides includes KCNE1 and KCNE3, both of which complex with KvLQT1 to produce functionally distinct currents...
  75. pmc KCNE2 confers background current characteristics to the cardiac KCNQ1 potassium channel
    N Tinel
    Institut de Pharmacologie Moleculaire et Cellulaire, CNRS UPR 411, 660 route des Lucioles, Sophia Antipolis, 06560 Valbonne, France
    EMBO J 19:6326-30. 2000
    ..b>KCNE3 markedly changes KCNQ1 as well as HERG current properties...
  76. ncbi The very small-conductance K+ channel KvLQT1 and epithelial function
    M Bleich
    Physiologisches Institut, Freiburg, Germany
    Pflugers Arch 440:202-6. 2000
    ..Its specific biophysical and pharmacological properties are determined by the regulatory subunits IsK (KCNE1) and MiRP2 (KCNE3)...
  77. ncbi A constitutively open potassium channel formed by KCNQ1 and KCNE3
    B C Schroeder
    Zentrum fur Molekulare Neurobiologie Hamburg, Hamburg University, Germany
    Nature 403:196-9. 2000
    ..Here we show that the novel beta-subunit KCNE3 markedly changes KCNQ1 properties to yield currents that are nearly instantaneous and depend linearly on voltage...
  78. ncbi Expression and transcriptional control of human KCNE genes
    Andrew L Lundquist
    Department of Pharmacology, Vanderbilt University School of Medicine, Nashville, TN 37232, USA
    Genomics 87:119-28. 2006
    ..We identified alternatively spliced transcripts for both KCNE1 and KCNE3, including a cardiac-specific KCNE1 transcript...
  79. ncbi A novel sodium channel mutation in a family with hypokalemic periodic paralysis
    D E Bulman
    Division of Neurology, Ottawa General Hospital, Ottawa Hospital Research Institute, Ontario, Canada
    Neurology 53:1932-6. 1999
    To identify the cause of hypokalemic periodic paralysis (HOKPP) in a family whose disease is not caused by a mutation in the dihydropyridine-sensitive (DHP) receptor alpha1-subunit gene (CACNA1S).
  80. ncbi Toxic thyroid adenoma presenting as hypokalemic periodic paralysis
    Tetsuya Tagami
    Clinical Research Institute, Division of Endocrinology and Metabolism, Kyoto Medical Center, National Hospital Organization, Japan
    Endocr J 54:797-803. 2007
    ..and no known genetic mutations of ionic channel genes, such as calcium (CACN1S), sodium (SCN4A) and potassium (KCNE3), were found...
  81. ncbi Fenofibrate inhibits intestinal Cl- secretion by blocking basolateral KCNQ1 K+ channels
    Poonam J Bajwa
    Division of Biomedical Sciences, University of California, Riverside, CA 92521 0121, USA
    Am J Physiol Gastrointest Liver Physiol 293:G1288-99. 2007
    ..on T84 cells indicated that fenofibrate inhibits basolateral cAMP-stimulated K(+) channels (putatively KCNQ1/KCNE3) without affecting Ca(2+)-stimulated K(+) channel activity, whereas clofibrate inhibits both K(+) pathways...
  82. pmc KCNE1 and KCNE3 stabilize and/or slow voltage sensing S4 segment of KCNQ1 channel
    Koichi Nakajo
    Division of Biophysics and Neurobiology, Department of Molecular Physiology, National Institute for Physiological Sciences, Okazaki, Aichi, 444 8585, Japan
    J Gen Physiol 130:269-81. 2007
    ..In the presence of KCNE3, on the other hand, the second order rate constant for modification was not state dependent, indicating that the ..
  83. ncbi Contribution of KCNQ1 to the regulatory volume decrease in the human mammary epithelial cell line MCF-7
    Brenna L vanTol
    Dept of Physiology and Biophysics, Dalhousie Univ, Halifax, Nova Scotia B3H 1X5, Canada
    Am J Physiol Cell Physiol 293:C1010-9. 2007
    ..while recordings from mammalian cells heterologously expressing KCNQ1 alone or KCNQ1 with the accessory subunit KCNE3 reveal a volume-sensitive K(+) current, inhibited by 293B...
  84. ncbi The MiRP2-Kv3.4 potassium channel: muscling in on Alzheimer's disease
    Eun Choi
    Greenberg Division of Cardiology, Department of Medicine, Cornell University, Weill Medical College, New York, NY 10021, USA
    Mol Pharmacol 72:499-501. 2007
    ..provide evidence of a role for the voltage-gated potassium channel alpha subunit Kv3.4 and its ancillary subunit MiRP2 in beta-amyloid (Abeta) peptide-mediated neuronal death. The MiRP2-Kv3...
  85. pmc Abolition of Ca2+-mediated intestinal anion secretion and increased stool dehydration in mice lacking the intermediate conductance Ca2+-dependent K+ channel Kcnn4
    Carlos A Flores
    Centro de Estudios Cientificos, Avenida Arturo Prat 514, Valdivia, Chile
    J Physiol 583:705-17. 2007
    ..The K(+) channel involved in cAMP-dependent secretion has been identified as the KCNQ1-KCNE3 complex, but the identity of the K(+) channel driving Ca(2+)-activated Cl- secretion is controversial...
  86. ncbi WTC deafness Kyoto (dfk): a rat model for extensive investigations of Kcnq1 functions
    Hiroshi Gohma
    Institute of Laboratory Animals, Graduate School of Medicine, Kyoto University, Kyoto, Japan
    Physiol Genomics 24:198-206. 2006
    ..In the colon and small intestine, KCNQ1 is coassembled with KCNE3 to play an important role in transepithelial cAMP-stimulated Cl- secretion...
  87. pmc The role of S4 charges in voltage-dependent and voltage-independent KCNQ1 potassium channel complexes
    Gianina Panaghie
    Greenberg Division of Cardiology, Department of Medicine, Cornell University, Weill Medical College, New York, NY 10021, USA
    J Gen Physiol 129:121-33. 2007
    ..formed with KCNQ1 alpha subunits to generate the voltage-dependent I(Ks) channel in human heart; MiRP1 and MiRP2 remove the voltage dependence of KCNQ1 to generate potassium "leak" currents in gastrointestinal epithelia...
  88. ncbi Localization of a novel autosomal recessive non-syndromic hearing impairment locus DFNB63 to chromosome 11q13.3-q13.4
    A Tlili
    Laboratoire de Génétique Moléculaire Humaine, Faculte de Medecine de Sfax, Tunisie
    Ann Hum Genet 71:271-5. 2007
    ..We positionally excluded MYO7A from being the DFNB63-causative gene. In addition, the screening of two candidate genes, SHANK2 and KCNE3, failed to reveal any disease-causing mutations.
  89. ncbi KCNE3 is an inhibitory subunit of the Kv4.3 potassium channel
    Alicia Lundby
    The Danish National Research Foundation Centre for Cardiac Arrhythmia, Department of Medical Physiology, University of Copenhagen, The Panum Institute, 3 Blegdamsvej, DK 2200 Copenhagen N, Denmark
    Biochem Biophys Res Commun 346:958-67. 2006
    ..Here we show that the KCNE3 beta-subunit has a strong inhibitory effect on current conducted by heterologously expressed Kv4.3 channels...
  90. ncbi Phosphorylation and protonation of neighboring MiRP2 sites: function and pathophysiology of MiRP2-Kv3.4 potassium channels in periodic paralysis
    Geoffrey W Abbott
    Greenberg Division of Cardiology, Department of Medicine and Department of Pharmacology, Cornell University, Weill Medical College, New York, New York, USA
    FASEB J 20:293-301. 2006
    MinK-related peptide 2 (MiRP2) and Kv3.4 subunits assemble in skeletal muscle to create subthreshold, voltage-gated potassium channels. MiRP2 acts on Kv3...
  91. ncbi Protein distribution of Kcnq1, Kcnh2, and Kcne3 potassium channel subunits during mouse embryonic development
    María Pilar de Castro
    Cardiovascular Development Group, Department of Experimental Biology, University of Jaen, Jaen, Spain
    Anat Rec A Discov Mol Cell Evol Biol 288:304-15. 2006
    ..In this study, we report a detailed analysis of Kcnq1, Kcnh2, and Kcne3 protein expression during mouse embryogenesis. Our results demonstrate that Kcnq1 and Kcnh2 are widely distributed...
  92. pmc Ancillary subunits and stimulation frequency determine the potency of chromanol 293B block of the KCNQ1 potassium channel
    Glenna C L Bett
    Department of Gynecology and Obstetrics, Department of Physiology and Biophysics, 124 Sherman Hall, State University of NY at Buffalo, Buffalo, NY 14214, USA
    J Physiol 576:755-67. 2006
    ..In epithelia, KCNQ1 co-assembles with KCNE3 (Mirp2) producing a constitutively open channel. Chromanol 293B is a selective KCNQ1 blocker...
  93. ncbi [R1239H mutation of CACNA1S gene in a Chinese family with hypokalaemic periodic paralysis]
    Qing Ke
    Department of Neurology, General Hospital of PLA, Beijing, 100853 PR China
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 23:272-4. 2006
    Mutation screening was performed to a Chinese family with hypokalaemic periodic paraiysis(HOKPP) for locating the corresponding mutations of gene and for specifying the clinical features associated with mutations.
  94. ncbi Colocalization of KCNQ1/KCNE channel subunits in the mouse gastrointestinal tract
    K Dedek
    Zentrum für Molekulare Neurobiologie Hamburg ZMNH, Hamburg University, Germany
    Pflugers Arch 442:896-902. 2001
    ..in the mouse gastrointestinal tract KCNQ1 is prominently expressed in stomach, small intestine and colon, while KCNE3 is expressed in the colon and to a lesser extent in small intestine...
  95. pmc KCNE variants reveal a critical role of the beta subunit carboxyl terminus in PKA-dependent regulation of the IKs potassium channel
    Junko Kurokawa
    Department of Bio Informational Pharmacology, Medical Research Institute, Tokyo Medical and Dental University, Tokyo, Japan
    Channels (Austin) 3:16-24. 2009
    ..when co-assembled with KCNE1 and channels that favor the open state when co-assembled with either KCNE2 or KCNE3. In the heart, stimulation of the sympathetic nervous system enhances IKs...
  96. ncbi Disease-associated mutations in KCNE potassium channel subunits (MiRPs) reveal promiscuous disruption of multiple currents and conservation of mechanism
    Geoffrey W Abbott
    Department of Pediatrics, Boyer Center for Molecular Medicine, Yale University School of Medicine, New Haven, Connecticut 06536 USA
    FASEB J 16:390-400. 2002
    ..To investigate whether MinK, MiRP1, and MiRP2 operate similarly with their known native alpha subunit partners (KCNQ1, HERG, and Kv3...
  97. 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
    ..A mechanism for acquired arrhythmia is revealed: genetically based reduction in potassium currents that remains clinically silent until combined with additional stressors...
  98. pmc Interaction of KCNE subunits with the KCNQ1 K+ channel pore
    Gianina Panaghie
    Greenberg Division of Cardiology, Department of Medicine, Weill Medical College of Cornell University, 520 East 70th Street, New York, NY 10021, USA
    J Physiol 570:455-67. 2006
    ..alpha subunits form functionally distinct potassium channels by coassembling with KCNE ancillary subunits MinK and MiRP2. MinK-KCNQ1 channels generate the slowly activating, voltage-dependent cardiac IKs current...

Research Grants19

  1. Structural Basis for KCNE Modulation of the KCNQ1 Channel
    Carlos G Vanoye; Fiscal Year: 2010
    ..Aim 3. Determine the 3-D structure of KCNE3 in bilayers and formulate a working model for how KCNE3 rapidly activates Q1 channel function. Aim 4...
  2. MinK-related peptides(MiRPs): structure and function
    Steve Goldstein; Fiscal Year: 2004
    ..This allowed isolation of the genes for MiRP1, MiRP2 and MIRP3...
  3. Molecular Physiology of KCNE Potassium Channel Subunits
    ALFRED GEORGE; Fiscal Year: 2007
    ..Co-expression of KCNQ1 with KCNE3, KCNE4 or KCNE5 is particularly interesting with functional effects ranging from constitutive activation (KCNE3) ..
  4. Modulation of cardiac K+ channels by drugs
    Michael Sanguinetti; Fiscal Year: 2004
    ..the inactivated state in drug block of HERG channels, and how binding of accessory beta-subunits (minK, MiRP1 and MiRP2) enhances drug block of HERG and KvLQT1 channels...
  5. Regulation of Kv3.1 by MiRPs in Auditory Neurons
    GEOFFREY ABBOTT; Fiscal Year: 2006
    ..We recently found that Kv3.1 forms complexes with MiRP2, a single transmembrane domain channel ancillary subunit, in mammalian brain...
  6. Molecular Basis of Automaticity in the Myometrium
    Glenna Bett; Fiscal Year: 2007
    ..Currently, there are no effective ways to prevent preterm birth. Identification of an ion channel which is the uterine pacemaker offers the opportunity for designing interventions to prevent and delay preterm labor. ..