Genomes and Genes
Gene Symbol: KCNE3
Description: potassium channel, voltage gated subfamily E regulatory beta subunit 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, potassium voltage-gated channel, Isk-related family, member 3, voltage-gated K+ channel subunit MIRP2
- A mutation in the KCNE3 potassium channel gene is associated with susceptibility to thyrotoxic hypokalemic periodic paralysisMagnus 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)...
- Protease-activated receptor regulation of Cl- secretion in Calu-3 cells requires prostaglandin release and CFTR activationMelissa 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...
- 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)...
- Molecular determinants of cardiac transient outward potassium current (I(to)) expression and regulationNoriko 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...
- Severe respiratory phenotype caused by a de novo Arg528Gly mutation in the CACNA1S gene in a patient with hypokalemic periodic paralysisTae 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. 2010Hypokalemic periodic paralysis (HOKPP) is a rare disorder characterized by episodic muscle weakness with hypokalemia...
- Functional effects of KCNE3 mutation and its role in the development of Brugada syndromeEva 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...
- 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 deathA 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...
- Novel KCNE3 mutation reduces repolarizing potassium current and associated with long QT syndromeSeiko 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...
- Ménière's disease is associated with single nucleotide polymorphisms in the human potassium channel genes, KCNE1 and KCNE3Katsumi 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...
- MiRP2 forms potassium channels in skeletal muscle with Kv3.4 and is associated with periodic paralysisG 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..A missense mutation is identified in the gene for MiRP2 (KCNE3) in two families with periodic paralysis and found to segregate with the disease. Mutant MiRP2-Kv3...
- Lack of association of the potassium channel-associated peptide MiRP2-R83H variant with periodic paralysisD 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...
- Polymorphisms in KCNE1 or KCNE3 are not associated with Ménière disease in the Caucasian populationColleen 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...
- Periodic paralysis mutation MiRP2-R83H in controls: Interpretations and general recommendationKarin Jurkat-Rott
Department of Applied Physiology, Ulm University, Germany
Neurology 62:1012-5. 2004An 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...
- KCNE3 mutation V17M identified in a patient with lone atrial fibrillationAlicia 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.
- The alpha(1S) subunit of the L-type calcium channel is not a predisposition gene for thyrotoxic periodic paralysisNelson 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)...
- Genotype-phenotype correlation and therapeutic rationale in hyperkalemic periodic paralysisKarin 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.
- Regulation and properties of KCNQ1 (K(V)LQT1) and impact of the cystic fibrosis transmembrane conductance regulatorA 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...
- The cardiac K+ channel KCNQ1 is essential for gastric acid secretionF 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...
- KCNE2 modulates current amplitudes and activation kinetics of HCN4: influence of KCNE family members on HCN4 currentsNiels 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...
- KCNE4 domains required for inhibition of KCNQ1Lauren 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...
- 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...
- The small conductance K+ channel, KCNQ1: expression, function, and subunit composition in murine tracheaF 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...
- Divergent expression of delayed rectifier K(+) channel subunits during mouse heart developmentD 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-..
- An arrhythmia susceptibility gene in Caenorhabditis elegansKi 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...
- KCNE1 constrains the voltage sensor of Kv7.1 K+ channelsLiora 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...
- Cell cycle-dependent expression of Kv3.4 channels modulates proliferation of human uterine artery smooth muscle cellsEduardo 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...
- Electrophysiological and molecular identification of hepatocellular volume-activated K+ channelsW 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...
- [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. 2006Mutation screening was performed on two Chinese families with HOKPP to locat the corresponding mutations and to specify the clinical features associated with the mutation.
- [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
- MinK, MiRP1, and MiRP2 diversify Kv3.1 and Kv3.2 potassium channel gatingAnthony 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...
- Dynamic subunit stoichiometry confers a progressive continuum of pharmacological sensitivity by KCNQ potassium channelsHaibo 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...
- P2Y6 receptor mediates colonic NaCl secretion via differential activation of cAMP-mediated transportMichael 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...
- Genetic and clinical aspects of Brugada syndrome: an updateGiuseppe 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...
- Electrolyte transport in the mouse trachea: no evidence for a contribution of luminal K(+) conductanceR 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 ..
- KCNE3 T4A as the genetic basis of Brugada-pattern electrocardiogramTadashi 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...
- West Nile virus protease activity in detergent solutions and application for affinity tag removalQiwei 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...
- Oestrogen promotes KCNQ1 potassium channel endocytosis and postendocytic trafficking in colonic epitheliumRaphaël Rapetti-Mauss
Department of Molecular Medicine, RCSI ERC, Beaumont Hospital, PO Box 9063, Dublin 9, Ireland
J Physiol 591:2813-31. 2013The 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...
- A single transmembrane site in the KCNE-encoded proteins controls the specificity of KvLQT1 channel gatingYonathan 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 ..
- A replication study on proposed candidate genes in Ménière's disease, and a review of the current status of genetic studiesElina 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.
- Molecular architecture of the human sinus node: insights into the function of the cardiac pacemakerNatalie 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...
- Hypokalemic periodic paralysis; two different genes responsible for similar clinical manifestationsHunmin Kim
Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea
Korean J Pediatr 54:473-6. 2011Primary 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...
- The genetic basis of Brugada syndrome: a mutation updatePaula 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...
- Functional delivery of a membrane protein into oocyte membranes using bicellesCongbao Kang
Department of Biochemistry, Center for Structural Biology, Vanderbilt University School of Medicine, Nashville, Tennessee 37232 8725, USA
Biochemistry 49:653-5. 2010Voltage-gated potassium channel modulatory membrane protein KCNE3 was overexpressed and purified into both micelles and bicelles...
- Thyrotoxic periodic paralysis in an Italian man: clinical manifestation and genetic analysisFrancesco 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...
- KCNE1 and KCNE3 beta-subunits regulate membrane surface expression of Kv12.2 K(+) channels in vitro and form a tripartite complex in vivoSinead 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...
- Autonomously functioning thyroid nodule associated with thyrotoxic periodic paralysisHiroshi 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...
- Sexual dimorphism and oestrogen regulation of KCNE3 expression modulates the functional properties of KCNQ1 K⁺ channelsRodrigo 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 ..
- Serum- and glucocorticoid-inducible kinases (SGK) regulate KCNQ1/KCNE potassium channelsNathalie 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-..
- Deep resequencing of the voltage-gated potassium channel subunit KCNE3 gene in chronic tinnitusPhilipp 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...
- Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transportPatricia Preston
Leibniz Institut für Molekulare Pharmakologie and Max Delbrück Centrum für Molekulare Medizin, 13125 Berlin, Germany
J Biol Chem 285:7165-75. 2010The KCNE3 beta-subunit constitutively opens outwardly rectifying KCNQ1 (Kv7.1) K(+) channels by abolishing their voltage-dependent gating...
- A shared mechanism for lipid- and beta-subunit-coordinated stabilization of the activated K+ channel voltage sensorEun 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)...
- Bacterial synthesis, purification, and solubilization of membrane protein KCNE3, a regulator of voltage-gated potassium channelsS A Goncharuk
Shemyakin and Ovchinnikov Institute of Bioorganic Chemistry, Russian Academy of Sciences, Moscow, 117997, Russia
Biochemistry (Mosc) 74:1344-9. 2009An 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...
- Functional implications of KCNE subunit expression for the Kv7.5 (KCNQ5) channelMeritxell 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...
- Impact of KCNE subunits on KCNQ1 (Kv7.1) channel membrane surface targetingMeritxell 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...
- Genetic dissection reveals unexpected influence of beta subunits on KCNQ1 K+ channel polarized trafficking in vivoTorsten 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...
- Novel female sex-dependent actions of oestrogen in the intestineFiona 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...
- Distinct subdomains of the KCNQ1 S6 segment determine channel modulation by different KCNE subunitsCarlos 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 ..
- Extracellular potassium inhibits Kv7.1 potassium channels by stabilizing an inactivated stateAnders 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...
- Endocannabinoids and cannabinoid analogues block human cardiac Kv4.3 channels in a receptor-independent mannerIrene 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...
- KCNE peptides differently affect voltage sensor equilibrium and equilibration rates in KCNQ1 K+ channelsJessica 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...
- KCNQ1 mutation Q147R is associated with atrial fibrillation and prolonged QT intervalAlicia 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...
- In vitro molecular interactions and distribution of KCNE family with KCNQ1 in the human heartSaid 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...
- K+ channel KVLQT1 located in the basolateral membrane of distal colonic epithelium is not essential for activating Cl- secretionTianjiang 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). Immunofluorescence labeling for Kcnq1 was most prominent in the lateral membrane of crypt cells in rat colon...
- Manifestation, management and molecular analysis of candidate genes in two rare cases of thyrotoxic hypokalemic periodic paralysisCamilla 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...
- Correlating phenotype and genotype in the periodic paralysesT 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...
- Identification of a KCNE2 gain-of-function mutation in patients with familial atrial fibrillationYiqing 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)...
- No mutation in the KCNE3 potassium channel gene in Chinese thyrotoxic hypokalaemic periodic paralysis patientsNelson L S Tang
Department of Chemical Pathology, Faculty of Medicine, The Chinese University of Hong Kong, Kong Kong
Clin Endocrinol (Oxf) 61:109-12. 2004Mutation in KCNE3 gene (Isk-related family potassium voltage-gated channel member 3 gene) was recently associated with the aetiology of thyrotoxic periodic paralysis (TPP)...
- Mechanisms of anion secretion in Calu-3 human airway epithelial cells by 7,8-benzoquinolineA 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...
- KCNQ1 gain-of-function mutation in familial atrial fibrillationYi 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...
- Ectopic expression of KCNE3 accelerates cardiac repolarization and abbreviates the QT intervalReza Mazhari
Department of Medicine, Institute of Molecular Cardiobiology, Johns Hopkins University, 720 Rutland Avenue, Baltimore, MD 21205, USA
J Clin Invest 109:1083-90. 2002Regulatory subunit KCNE3 (E3) interacts with KCNQ1 (Q1) in epithelia, regulating its activation kinetics and augmenting current density...
- Characterization of basolateral K+ channels underlying anion secretion in the human airway cell line Calu-3Elizabeth 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-..
- KCNQ1 K+ channels are involved in lipopolysaccharide-induced apoptosis of distal kidney cellsChristophe 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...
- KCNQ potassium channels: physiology, pathophysiology, and pharmacologyJ 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...
- Structural determinants of KvLQT1 control by the KCNE family of proteinsY 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...
- KCNE2 confers background current characteristics to the cardiac KCNQ1 potassium channelN 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...
- The very small-conductance K+ channel KvLQT1 and epithelial functionM Bleich
Physiologisches Institut, Freiburg, Germany
Pflugers Arch 440:202-6. 2000..biophysical and pharmacological properties are determined by the regulatory subunits IsK (KCNE1) and MiRP2 (KCNE3)...
- A constitutively open potassium channel formed by KCNQ1 and KCNE3B 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...
- Expression and transcriptional control of human KCNE genesAndrew 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...
- A novel sodium channel mutation in a family with hypokalemic periodic paralysisD E Bulman
Division of Neurology, Ottawa General Hospital, Ottawa Hospital Research Institute, Ontario, Canada
Neurology 53:1932-6. 1999To 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).
- Toxic thyroid adenoma presenting as hypokalemic periodic paralysisTetsuya 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...
- Fenofibrate inhibits intestinal Cl- secretion by blocking basolateral KCNQ1 K+ channelsPoonam 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...
- KCNE1 and KCNE3 stabilize and/or slow voltage sensing S4 segment of KCNQ1 channelKoichi 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 ..
- Contribution of KCNQ1 to the regulatory volume decrease in the human mammary epithelial cell line MCF-7Brenna 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...
- The MiRP2-Kv3.4 potassium channel: muscling in on Alzheimer's diseaseEun 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...
- Abolition of Ca2+-mediated intestinal anion secretion and increased stool dehydration in mice lacking the intermediate conductance Ca2+-dependent K+ channel Kcnn4Carlos 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...
- WTC deafness Kyoto (dfk): a rat model for extensive investigations of Kcnq1 functionsHiroshi 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...
- The role of S4 charges in voltage-dependent and voltage-independent KCNQ1 potassium channel complexesGianina 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...
- Localization of a novel autosomal recessive non-syndromic hearing impairment locus DFNB63 to chromosome 11q13.3-q13.4A 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.
- KCNE3 is an inhibitory subunit of the Kv4.3 potassium channelAlicia 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...
- Phosphorylation and protonation of neighboring MiRP2 sites: function and pathophysiology of MiRP2-Kv3.4 potassium channels in periodic paralysisGeoffrey 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. 2006MinK-related peptide 2 (MiRP2) and Kv3.4 subunits assemble in skeletal muscle to create subthreshold, voltage-gated potassium channels. MiRP2 acts on Kv3...
- Protein distribution of Kcnq1, Kcnh2, and Kcne3 potassium channel subunits during mouse embryonic developmentMaría 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...
- Ancillary subunits and stimulation frequency determine the potency of chromanol 293B block of the KCNQ1 potassium channelGlenna 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...
- [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. 2006Mutation 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.
- Colocalization of KCNQ1/KCNE channel subunits in the mouse gastrointestinal tractK 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...
- KCNE variants reveal a critical role of the beta subunit carboxyl terminus in PKA-dependent regulation of the IKs potassium channelJunko 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...
- Disease-associated mutations in KCNE potassium channel subunits (MiRPs) reveal promiscuous disruption of multiple currents and conservation of mechanismGeoffrey 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...
- MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmiaG 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...
- Interaction of KCNE subunits with the KCNQ1 K+ channel poreGianina 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...
- MinK-related peptides(MiRPs): structure and functionSteve Goldstein; Fiscal Year: 2004..This allowed isolation of the genes for MiRP1, MiRP2 and MIRP3...
- Regulation of Kv3.1 by MiRPs in Auditory NeuronsGEOFFREY ABBOTT; Fiscal Year: 2006..We recently found that Kv3.1 forms complexes with MiRP2, a single transmembrane domain channel ancillary subunit, in mammalian brain...
- Molecular Basis of Automaticity in the MyometriumGlenna Bett; Fiscal Year: 2008..Identification of an ion channel which is the uterine pacemaker offers the opportunity for designing interventions to prevent and delay preterm labor. [unreadable] [unreadable] [unreadable] [unreadable]..