Kcnq2

Summary

Gene Symbol: Kcnq2
Description: potassium voltage-gated channel, subfamily Q, member 2
Alias: HNSPC, KQT2, Nmf134, potassium voltage-gated channel subfamily KQT member 2, KQT-like 2, potassium channel subunit alpha KvLQT2, voltage-gated potassium channel subunit Kv7.2
Species: mouse

Top Publications

  1. ncbi KQT2, a new putative potassium channel family produced by alternative splicing. Isolation, genomic structure, and alternative splicing of the putative potassium channels
    M Nakamura
    Pharmaceutical Basic Research Laboratories Aobadai, Japan Tobacco Inc, Kanagawa, Japan
    Receptors Channels 5:255-71. 1998
  2. ncbi Disruption of the epilepsy KCNQ2 gene results in neural hyperexcitability
    H Watanabe
    Pharmaceutical Frontier Research Laboratories, Japan Tobacco, Yokohama Department of Neurology, School of Medicine, Keio University, Tokyo, Japan
    J Neurochem 75:28-33. 2000
  3. pmc Activity-dependent transcriptional regulation of M-Type (Kv7) K(+) channels by AKAP79/150-mediated NFAT actions
    Jie Zhang
    Department of Physiology, MS 7756, University of Texas Health Science Center, San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229, USA
    Neuron 76:1133-46. 2012
  4. ncbi KCNQ2 is a nodal K+ channel
    Jérôme J Devaux
    Department of Neurology, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104 6077, USA
    J Neurosci 24:1236-44. 2004
  5. pmc Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganization
    Nanda A Singh
    Department of Human Genetics, University of Utah, Salt Lake City, UT 84112, USA
    J Physiol 586:3405-23. 2008
  6. ncbi A spontaneous mutation involving Kcnq2 (Kv7.2) reduces M-current density and spike frequency adaptation in mouse CA1 neurons
    James F Otto
    Anticonvulsant Drug Development Program, Department of Pharmacology and Toxicology, University of Utah, Salt Lake City, Utah 84112, USA
    J Neurosci 26:2053-9. 2006
  7. ncbi A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axon
    Zongming Pan
    Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 26:2599-613. 2006
  8. pmc Epilepsy-Associated KCNQ2 Channels Regulate Multiple Intrinsic Properties of Layer 2/3 Pyramidal Neurons
    Zachary Niday
    Department of Physiology and Neurobiology, University of Connecticut, Storrs, Connecticut 06269
    J Neurosci 37:576-586. 2017
  9. pmc Transcompartmental reversal of single fibre hyperexcitability in juxtaparanodal Kv1.1-deficient vagus nerve axons by activation of nodal KCNQ channels
    Edward Glasscock
    J L Noebels Department of Neurology, Baylor College of Medicine, One Baylor Plaza, NB220, Houston, TX 77030, USA
    J Physiol 590:3913-26. 2012
  10. pmc Effects of KCNQ2 gene truncation on M-type Kv7 potassium currents
    Jon Robbins
    Wolfson Centre for Age Related Disease, King s College London, London, United Kingdom
    PLoS ONE 8:e71809. 2013

Scientific Experts

  • Jeffrey Noebels
  • Jérôme J Devaux
  • Jennifer A Kearney
  • Zhe Jin
  • M Nakamura
  • Nanda A Singh
  • David Kapfhamer
  • Shuang Li
  • Heun Soh
  • Zachary Niday
  • Sebastian Schütze
  • Anastasios V Tzingounis
  • Wayne N Frankel
  • WenJing Liu
  • Yuko Tomonoh
  • Chih H King
  • Thanos Tzounopoulos
  • Jon Robbins
  • Pingzheng Zhou
  • James F Otto
  • Jie Zhang
  • Edward Glasscock
  • Laura A McCallum
  • Carolina Roza
  • Xin Zhou
  • Yan Yang
  • X Zhou
  • Nicole A Hawkins
  • Steven S Scherer
  • Anastassios V Tzingounis
  • Yvonne G Weber
  • Zongming Pan
  • H Christian Peters
  • H Steve White
  • Virginia E Hawkins
  • Daniel K Mulkey
  • Ian J Orozco
  • Thomas J Jentsch
  • Bopanna I Kalappa
  • Sayaka Yamashita
  • Joseph J LoTurco
  • Masanobu Deshimaru
  • Hiroki Kitaura
  • Christoph Lossin
  • Junko Yamada
  • Kimi Araki
  • Rima Pant
  • Haruna Kitamura
  • Fumiaki Mori
  • Ryo Saito
  • Hua Wen
  • Taku Uchida
  • Shinichi Hirose
  • Eric Lancaster
  • Elior Peles
  • Yasuyoshi Tanaka
  • Akiyoshi Kakita
  • Masayuki Itoh
  • Keisuke Migita
  • Yasuhiro Miyazaki
  • Shinya Ueno
  • Koichi Wakabayashi
  • Yukio Takano
  • Tomoko Arasaki
  • Daniela Salomon
  • Min Li
  • Min Gu
  • Fa Jun Nan
  • Zhaobing Gao
  • Veronica Choi
  • David A Brown
  • Joanne M Reilly
  • Gayle M Passmore
  • Haibo Yu
  • Fe C Abogadie
  • Jing Qian
  • Mark S Shapiro
  • Matthew J Kole
  • Shan Ping Yu
  • S P Yu
  • Andrew Escayg
  • Stephanie L Pierce
  • Dongdong Chen
  • Jose A Lopez-Garcia
  • J Wei
  • Melinda S Martin
  • K Francis
  • Ling Wei
  • Sol Castillejo
  • Sarah K England

Detail Information

Publications33

  1. ncbi KQT2, a new putative potassium channel family produced by alternative splicing. Isolation, genomic structure, and alternative splicing of the putative potassium channels
    M Nakamura
    Pharmaceutical Basic Research Laboratories Aobadai, Japan Tobacco Inc, Kanagawa, Japan
    Receptors Channels 5:255-71. 1998
    ..Eleven mouse cDNA clones homologous to the new human putative K+ channel (designated HNSPC, which we recently reported) were isolated from the brain cDNA libraries...
  2. ncbi Disruption of the epilepsy KCNQ2 gene results in neural hyperexcitability
    H Watanabe
    Pharmaceutical Frontier Research Laboratories, Japan Tobacco, Yokohama Department of Neurology, School of Medicine, Keio University, Tokyo, Japan
    J Neurochem 75:28-33. 2000
    ..Recently, two novel voltage-dependent potassium channel genes, KCNQ2 and KCNQ3, were identified by positional cloning as being responsible for BFNC...
  3. pmc Activity-dependent transcriptional regulation of M-Type (Kv7) K(+) channels by AKAP79/150-mediated NFAT actions
    Jie Zhang
    Department of Physiology, MS 7756, University of Texas Health Science Center, San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78229, USA
    Neuron 76:1133-46. 2012
    M-type K(+) channels, encoded by KCNQ2-KCNQ5 genes, play key roles in regulation of neuronal excitability; however, less is known about the mechanisms controlling their transcriptional expression...
  4. ncbi KCNQ2 is a nodal K+ channel
    Jérôme J Devaux
    Department of Neurology, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104 6077, USA
    J Neurosci 24:1236-44. 2004
    Mutations in the gene encoding the K+ channel KCNQ2 cause neonatal epilepsy and myokymia, indicating that KCNQ2 regulates the excitability of CNS neurons and motor axons, respectively...
  5. pmc Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganization
    Nanda A Singh
    Department of Human Genetics, University of Utah, Salt Lake City, UT 84112, USA
    J Physiol 586:3405-23. 2008
    ..weeks of onset and a favourable prognosis, sparing cognitive abilities despite persistent expression of the mutant KCNQ2 or KCNQ3 potassium channels throughout adulthood...
  6. ncbi A spontaneous mutation involving Kcnq2 (Kv7.2) reduces M-current density and spike frequency adaptation in mouse CA1 neurons
    James F Otto
    Anticonvulsant Drug Development Program, Department of Pharmacology and Toxicology, University of Utah, Salt Lake City, Utah 84112, USA
    J Neurosci 26:2053-9. 2006
    ..Mutations in two subunits (KCNQ2 and KCNQ3; Kv7.2 and Kv7...
  7. ncbi A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axon
    Zongming Pan
    Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 26:2599-613. 2006
    ..Here, antibodies against four different KCNQ2 and KCNQ3 polypeptide epitopes show these subunits concentrated at the axonal initial segment (AIS) and node of ..
  8. pmc Epilepsy-Associated KCNQ2 Channels Regulate Multiple Intrinsic Properties of Layer 2/3 Pyramidal Neurons
    Zachary Niday
    Department of Physiology and Neurobiology, University of Connecticut, Storrs, Connecticut 06269
    J Neurosci 37:576-586. 2017
    b>KCNQ2 potassium channels are critical for normal brain function, as both loss-of-function and gain-of-function KCNQ2 variants can lead to various forms of neonatal epilepsy...
  9. pmc Transcompartmental reversal of single fibre hyperexcitability in juxtaparanodal Kv1.1-deficient vagus nerve axons by activation of nodal KCNQ channels
    Edward Glasscock
    J L Noebels Department of Neurology, Baylor College of Medicine, One Baylor Plaza, NB220, Houston, TX 77030, USA
    J Physiol 590:3913-26. 2012
    ....
  10. pmc Effects of KCNQ2 gene truncation on M-type Kv7 potassium currents
    Jon Robbins
    Wolfson Centre for Age Related Disease, King s College London, London, United Kingdom
    PLoS ONE 8:e71809. 2013
    The KCNQ2 gene product, Kv7.2, is a subunit of the M-channel, a low-threshold voltage-gated K(+) channel that regulates mammalian and human neuronal excitability...
  11. doi Calmodulin orchestrates the heteromeric assembly and the trafficking of KCNQ2/3 (Kv7.2/3) channels in neurons
    WenJing Liu
    Aix Marseille Universite, CNRS, CRN2M UMR7286, 13344 Marseille Cedex 15, Marseille, France
    Mol Cell Neurosci 58:40-52. 2014
    Mutations in KCNQ2 and KCNQ3 genes are responsible for benign familial neonatal seizures and epileptic encephalopathies...
  12. pmc The kick-in system: a novel rapid knock-in strategy
    Yuko Tomonoh
    Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan
    PLoS ONE 9:e88549. 2014
    ..2 (Kcnq2): p.Tyr284Cys (Y284C) and p...
  13. pmc Kv7.2 regulates the function of peripheral sensory neurons
    Chih H King
    Department of Neuroscience, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, 19104
    J Comp Neurol 522:3262-80. 2014
    ..The functional role of Kv7.2 has been hampered by the lack of a viable Kcnq2-null animal model...
  14. pmc Conditional deletions of epilepsy-associated KCNQ2 and KCNQ3 channels from cerebral cortex cause differential effects on neuronal excitability
    Heun Soh
    Department of Physiology and Neurobiology, University of Connecticut, Storrs, Connecticut 06269
    J Neurosci 34:5311-21. 2014
    b>KCNQ2 and KCNQ3 potassium channels have emerged as central regulators of pyramidal neuron excitability and spiking behavior...
  15. pmc Noise-induced plasticity of KCNQ2/3 and HCN channels underlies vulnerability and resilience to tinnitus
    Shuang Li
    Departments of Otolaryngology and Neurobiology, University of Pittsburgh School of Medicine, Pittsburgh, United States
    elife 4:. 2015
    ..This hyperactivity is caused, at least in part, by decreased Kv7.2/3 (KCNQ2/3) potassium currents...
  16. pmc KCNQ Potassium Channels Modulate Sensitivity of Skin Down-hair (D-hair) Mechanoreceptors
    Sebastian Schütze
    From the Leibniz Institut für Molekulare Pharmakologie FMP and Max Delbrück Centrum für Molekulare Medizin MDC, Robert Rossle Strasse 10, 13125 Berlin and
    J Biol Chem 291:5566-75. 2016
    ..channels play an important role in regulating the excitability of neuronal cells, as highlighted by mutations in Kcnq2 and Kcnq3 that underlie certain forms of epilepsy...
  17. pmc Pathogenic plasticity of Kv7.2/3 channel activity is essential for the induction of tinnitus
    Shuang Li
    Department of Otolaryngology and Neurobiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA
    Proc Natl Acad Sci U S A 110:9980-5. 2013
    ..Moreover, our findings point to previously unknown biological targets for designing therapeutic drugs that may prevent the development of tinnitus in humans...
  18. pmc Phosphatidylinositol 4,5-bisphosphate alters pharmacological selectivity for epilepsy-causing KCNQ potassium channels
    Pingzheng Zhou
    State Key Laboratory of Drug Research, Shanghai Institute of Materia Medica, Chinese Academy of Sciences, Shanghai 201203, China
    Proc Natl Acad Sci U S A 110:8726-31. 2013
    ..Whereas all five potassium channel subtypes (KCNQ1-KCNQ5) are found in the nervous system, KCNQ2 and KCNQ3 are the primary players that mediate M currents...
  19. pmc Novel role of KCNQ2/3 channels in regulating neuronal cell viability
    X Zhou
    Department of Pharmaceutical and Biomedical Sciences, Medical University of South Carolina, Charleston, SC 29425, USA
    Cell Death Differ 18:493-505. 2011
    ..The present investigation examined a possible role of the KCNQ2/3 channel or M-channel (also named Kv7.2/7.3 channels) in the pro-apoptotic process...
  20. pmc Accumulation of Kv7.2 channels in putative ectopic transduction zones of mice nerve-end neuromas
    Carolina Roza
    Dpto, Fisiologia, Edificio de Medicina Universidad de Alcalá, Madrid, Spain
    Mol Pain 7:58. 2011
    ..We hypothesized that after nerve damage, accumulation of Kv7 channels in afferent fibers may increase M-type currents which then acquired a more important role at regulating fiber excitability...
  21. pmc Potential role of KCNQ/M-channels in regulating neuronal differentiation in mouse hippocampal and embryonic stem cell-derived neuronal cultures
    Xin Zhou
    Department of Pharmaceutical and Biomedical Sciences, Medical University of South Carolina, Charleston, SC 29425, USA
    Exp Neurol 229:471-83. 2011
    ..It is composed of the molecular counterparts KCNQ2 and KCNQ3 (also named Kv7.2 and Kv7.3) channels and expressed in the soma and dendrites of neurons...
  22. pmc Neuronal voltage-gated ion channels are genetic modifiers of generalized epilepsy with febrile seizures plus
    Nicole A Hawkins
    Neuroscience Graduate Program, Vanderbilt University Medical Center, Nashville, TN 37232, USA
    Neurobiol Dis 41:655-60. 2011
    ..GEFS+, we used mouse models to study the effect of combining the human GEFS+ mutation SCN1A-R1648H with SCN2A, KCNQ2, and SCN8A mutations...
  23. pmc The C-terminal domain of ßIV-spectrin is crucial for KCNQ2 aggregation and excitability at nodes of Ranvier
    Jérôme J Devaux
    Département Signalisation Neuronale, CRN2M, UMR 6231, CNRS, Faculte de Medecine Secteur Nord, Université de la Méditerranée Université Paul Cézanne, CS80011, Bd Pierre Dramard, 13344 Marseille Cedex 15, France
    J Physiol 588:4719-30. 2010
    ..Axon and myelin structure in the PNS were unaffected in quivering-3J mice. Of interest, KCNQ2 subunit aggregates were undetectable at PNS and CNS nodes, whereas Nav and Kv1.1/Kv1...
  24. pmc Protein Phosphatase 2a and glycogen synthase kinase 3 signaling modulate prepulse inhibition of the acoustic startle response by altering cortical M-Type potassium channel activity
    David Kapfhamer
    Ernest Gallo Clinic and Research Center, Emeryville, California 94608, USA
    J Neurosci 30:8830-40. 2010
    ..The M-type potassium channel subunit, KCNQ2, is a putative GSK3beta substrate...
  25. pmc The contribution of Kv7 channels to pregnant mouse and human myometrial contractility
    Laura A McCallum
    Maternal and Fetal Research Unit, Division of Reproduction and Endocrinology, King s College London, St Thomas Hospital Campus, London, UK
    J Cell Mol Med 15:577-86. 2011
    ..Consequently, activation of the encoded channels represents a novel mechanism for treatment of preterm labour...
  26. pmc Contribution of KCNQ2 and KCNQ3 to the medium and slow afterhyperpolarization currents
    Anastassios V Tzingounis
    Department of Physiology and Neurobiology, University of Connecticut, Storrs, CT 06269, USA
    Proc Natl Acad Sci U S A 105:19974-9. 2008
    ..familial neonatal convulsion (BNFC) is a neurological disorder caused by mutations in the potassium channel genes KCNQ2 and KCNQ3, which are thought to contribute to the medium afterhyperpolarization (mAHP)...
  27. pmc Expression and localization of K channels KCNQ2 and KCNQ3 in the mammalian cochlea
    Zhe Jin
    Center for Hearing and Communication Research, Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden
    Audiol Neurootol 14:98-105. 2009
    ..Here, we describe Kcnq2/3 gene expression and distribution of M channel subunits KCNQ2 and 3 in the cochlea...
  28. ncbi Immunohistochemical analysis of KCNQ2 potassium channels in adult and developing mouse brain
    Yvonne G Weber
    Department of Neurology of the University of Ulm, Germany
    Brain Res 1077:1-6. 2006
    ..BFNC is caused by loss-of-function mutations in the potassium channels KCNQ2 and KCNQ3 which can well explain the resulting neuronal hyperexcitability...
  29. ncbi Severe epilepsy resulting from genetic interaction between Scn2a and Kcnq2
    Jennifer A Kearney
    Department of Human Genetics, 4909 Buhl Building 0618, 1241 E Catherine Street, Ann Arbor, MI 48109 0618, USA
    Hum Mol Genet 15:1043-8. 2006
    ..The voltage-gated potassium channel Kcnq2 is responsible for generating M current (I(KM)) that is thought to control excitability and limit repetitive ..
  30. ncbi Conditional transgenic suppression of M channels in mouse brain reveals functions in neuronal excitability, resonance and behavior
    H Christian Peters
    Institut für Neurale Signalverarbeitung, Zentrum fur Molekulare Neurobiologie Hamburg, Martinistrasse 52, 20246 Hamburg, Germany
    Nat Neurosci 8:51-60. 2005
    In humans, mutations in the KCNQ2 or KCNQ3 potassium-channel genes are associated with an inherited epilepsy syndrome...
  31. ncbi Spontaneous deletion of epilepsy gene orthologs in a mutant mouse with a low electroconvulsive threshold
    Yan Yang
    The Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609, USA
    Hum Mol Genet 12:975-84. 2003
    ..Two of these genes, Kcnq2 and Chrna4, are known to be mutated in human epilepsy families...
  32. ncbi Calmodulin is an auxiliary subunit of KCNQ2/3 potassium channels
    Hua Wen
    Department of Neuroscience, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 22:7991-8001. 2002
    Calmodulin (CaM) was identified as a KCNQ2 and KCNQ3 potassium channel-binding protein, using a yeast two-hybrid screen...
  33. ncbi Chromosomal mapping of the potassium channel genes Kcnq2 and Kcnq3 in mouse
    T McCormack
    Department of Physiology, New York University Medical Center, New York, New York 10016, USA
    Genomics 56:360-1. 1999