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
Products:     Kcnq2

Top Publications

  1. Nakamura M, Watanabe H, Kubo Y, Yokoyama M, Matsumoto T, Sasai H, et al. KQT2, a new putative potassium channel family produced by alternative splicing. Isolation, genomic structure, and alternative splicing of the putative potassium channels. Receptors Channels. 1998;5:255-71 pubmed
    ..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. Devaux J, Kleopa K, Cooper E, Scherer S. KCNQ2 is a nodal K+ channel. J Neurosci. 2004;24:1236-44 pubmed
    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...
  3. Singh N, Otto J, Dahle E, Pappas C, Leslie J, Vilaythong A, et al. Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganization. J Physiol. 2008;586:3405-23 pubmed publisher
    ..weeks of onset and a favourable prognosis, sparing cognitive abilities despite persistent expression of the mutant KCNQ2 or KCNQ3 potassium channels throughout adulthood...
  4. Watanabe H, Nagata E, Kosakai A, Nakamura M, Yokoyama M, Tanaka K, et al. Disruption of the epilepsy KCNQ2 gene results in neural hyperexcitability. J Neurochem. 2000;75:28-33 pubmed
    ..Recently, two novel voltage-dependent potassium channel genes, KCNQ2 and KCNQ3, were identified by positional cloning as being responsible for BFNC...
  5. Otto J, Yang Y, Frankel W, White H, Wilcox K. A spontaneous mutation involving Kcnq2 (Kv7.2) reduces M-current density and spike frequency adaptation in mouse CA1 neurons. J Neurosci. 2006;26:2053-9 pubmed
    ..Mutations in two subunits (KCNQ2 and KCNQ3; Kv7.2 and Kv7...
  6. Pan Z, Kao T, Horvath Z, Lemos J, Sul J, Cranstoun S, et al. A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axon. J Neurosci. 2006;26:2599-613 pubmed
    ..Here, antibodies against four different KCNQ2 and KCNQ3 polypeptide epitopes show these subunits concentrated at the axonal initial segment (AIS) and node of ..
  7. Zhang J, Shapiro M. Activity-dependent transcriptional regulation of M-Type (Kv7) K(+) channels by AKAP79/150-mediated NFAT actions. Neuron. 2012;76:1133-46 pubmed publisher
    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...
  8. Maljevic S, Reid C, Petrou S. Models for discovery of targeted therapy in genetic epileptic encephalopathies. J Neurochem. 2017;143:30-48 pubmed publisher
  9. Wen H, Levitan I. Calmodulin is an auxiliary subunit of KCNQ2/3 potassium channels. J Neurosci. 2002;22:7991-8001 pubmed
    Calmodulin (CaM) was identified as a KCNQ2 and KCNQ3 potassium channel-binding protein, using a yeast two-hybrid screen...
  10. Yang Y, Beyer B, Otto J, O Brien T, Letts V, White H, et al. Spontaneous deletion of epilepsy gene orthologs in a mutant mouse with a low electroconvulsive threshold. Hum Mol Genet. 2003;12:975-84 pubmed
    ..Two of these genes, Kcnq2 and Chrna4, are known to be mutated in human epilepsy families...

Detail Information

Publications38

  1. Nakamura M, Watanabe H, Kubo Y, Yokoyama M, Matsumoto T, Sasai H, et al. KQT2, a new putative potassium channel family produced by alternative splicing. Isolation, genomic structure, and alternative splicing of the putative potassium channels. Receptors Channels. 1998;5:255-71 pubmed
    ..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. Devaux J, Kleopa K, Cooper E, Scherer S. KCNQ2 is a nodal K+ channel. J Neurosci. 2004;24:1236-44 pubmed
    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...
  3. Singh N, Otto J, Dahle E, Pappas C, Leslie J, Vilaythong A, et al. Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganization. J Physiol. 2008;586:3405-23 pubmed publisher
    ..weeks of onset and a favourable prognosis, sparing cognitive abilities despite persistent expression of the mutant KCNQ2 or KCNQ3 potassium channels throughout adulthood...
  4. Watanabe H, Nagata E, Kosakai A, Nakamura M, Yokoyama M, Tanaka K, et al. Disruption of the epilepsy KCNQ2 gene results in neural hyperexcitability. J Neurochem. 2000;75:28-33 pubmed
    ..Recently, two novel voltage-dependent potassium channel genes, KCNQ2 and KCNQ3, were identified by positional cloning as being responsible for BFNC...
  5. Otto J, Yang Y, Frankel W, White H, Wilcox K. A spontaneous mutation involving Kcnq2 (Kv7.2) reduces M-current density and spike frequency adaptation in mouse CA1 neurons. J Neurosci. 2006;26:2053-9 pubmed
    ..Mutations in two subunits (KCNQ2 and KCNQ3; Kv7.2 and Kv7...
  6. Pan Z, Kao T, Horvath Z, Lemos J, Sul J, Cranstoun S, et al. A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axon. J Neurosci. 2006;26:2599-613 pubmed
    ..Here, antibodies against four different KCNQ2 and KCNQ3 polypeptide epitopes show these subunits concentrated at the axonal initial segment (AIS) and node of ..
  7. Zhang J, Shapiro M. Activity-dependent transcriptional regulation of M-Type (Kv7) K(+) channels by AKAP79/150-mediated NFAT actions. Neuron. 2012;76:1133-46 pubmed publisher
    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...
  8. Maljevic S, Reid C, Petrou S. Models for discovery of targeted therapy in genetic epileptic encephalopathies. J Neurochem. 2017;143:30-48 pubmed publisher
  9. Wen H, Levitan I. Calmodulin is an auxiliary subunit of KCNQ2/3 potassium channels. J Neurosci. 2002;22:7991-8001 pubmed
    Calmodulin (CaM) was identified as a KCNQ2 and KCNQ3 potassium channel-binding protein, using a yeast two-hybrid screen...
  10. Yang Y, Beyer B, Otto J, O Brien T, Letts V, White H, et al. Spontaneous deletion of epilepsy gene orthologs in a mutant mouse with a low electroconvulsive threshold. Hum Mol Genet. 2003;12:975-84 pubmed
    ..Two of these genes, Kcnq2 and Chrna4, are known to be mutated in human epilepsy families...
  11. Peters H, Hu H, Pongs O, Storm J, Isbrandt D. Conditional transgenic suppression of M channels in mouse brain reveals functions in neuronal excitability, resonance and behavior. Nat Neurosci. 2005;8:51-60 pubmed
    In humans, mutations in the KCNQ2 or KCNQ3 potassium-channel genes are associated with an inherited epilepsy syndrome...
  12. Kearney J, Yang Y, Beyer B, Bergren S, Claes L, Dejonghe P, et al. Severe epilepsy resulting from genetic interaction between Scn2a and Kcnq2. Hum Mol Genet. 2006;15:1043-8 pubmed
    ..The voltage-gated potassium channel Kcnq2 is responsible for generating M current (I(KM)) that is thought to control excitability and limit repetitive ..
  13. McCallum L, Pierce S, England S, Greenwood I, Tribe R. The contribution of Kv7 channels to pregnant mouse and human myometrial contractility. J Cell Mol Med. 2011;15:577-86 pubmed publisher
    ..Consequently, activation of the encoded channels represents a novel mechanism for treatment of preterm labour...
  14. Roza C, Castillejo S, Lopez Garcia J. Accumulation of Kv7.2 channels in putative ectopic transduction zones of mice nerve-end neuromas. Mol Pain. 2011;7:58 pubmed publisher
    ..2 channels after axotomy may represent a homeostatic compensation to over excitability in axotomized fibers, opening a window for a peripheral action of M-current modulators under conditions of neuropathy. ..
  15. Zhou X, Song M, Chen D, Wei L, Yu S. Potential role of KCNQ/M-channels in regulating neuronal differentiation in mouse hippocampal and embryonic stem cell-derived neuronal cultures. Exp Neurol. 2011;229:471-83 pubmed publisher
    ..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...
  16. Tomonoh Y, Deshimaru M, Araki K, Miyazaki Y, Arasaki T, Tanaka Y, et al. The kick-in system: a novel rapid knock-in strategy. PLoS ONE. 2014;9:e88549 pubmed publisher
    ..2 (Kcnq2): p.Tyr284Cys (Y284C) and p...
  17. Li S, Kalappa B, Tzounopoulos T. Noise-induced plasticity of KCNQ2/3 and HCN channels underlies vulnerability and resilience to tinnitus. elife. 2015;4: pubmed publisher
    ..This hyperactivity is caused, at least in part, by decreased Kv7.2/3 (KCNQ2/3) potassium currents...
  18. Schütze S, Orozco I, Jentsch T. KCNQ Potassium Channels Modulate Sensitivity of Skin Down-hair (D-hair) Mechanoreceptors. J Biol Chem. 2016;291:5566-75 pubmed publisher
    ..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...
  19. Anta B, Martín Rodríguez C, Gomis Pèrez C, Calvo L, López Benito S, Calderón García A, et al. Ubiquitin-specific Protease 36 (USP36) Controls Neuronal Precursor Cell-expressed Developmentally Down-regulated 4-2 (Nedd4-2) Actions over the Neurotrophin Receptor TrkA and Potassium Voltage-gated Channels 7.2/3 (Kv7.2/3). J Biol Chem. 2016;291:19132-45 pubmed publisher
    ..2/3 channel regulation. Our results demonstrate that USP36 binds to and regulates the actions of Nedd4-2 over different substrates affecting their expression and functions. ..
  20. Uchida T, Lossin C, Ihara Y, Deshimaru M, Yanagawa Y, Koyama S, et al. Abnormal ?-aminobutyric acid neurotransmission in a Kcnq2 model of early onset epilepsy. Epilepsia. 2017;58:1430-1439 pubmed publisher
    Mutations of the KCNQ2 gene, which encodes the Kv 7.2 subunit of voltage-gated M-type potassium channels, have been associated with epilepsy in the neonatal period...
  21. Hawkins N, Martin M, Frankel W, Kearney J, Escayg A. Neuronal voltage-gated ion channels are genetic modifiers of generalized epilepsy with febrile seizures plus. Neurobiol Dis. 2011;41:655-60 pubmed publisher
    ..GEFS+, we used mouse models to study the effect of combining the human GEFS+ mutation SCN1A-R1648H with SCN2A, KCNQ2, and SCN8A mutations...
  22. Devaux J. The C-terminal domain of ßIV-spectrin is crucial for KCNQ2 aggregation and excitability at nodes of Ranvier. J Physiol. 2010;588:4719-30 pubmed publisher
    ..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...
  23. McCormack T, Rudy B, Seldin M. Chromosomal mapping of the potassium channel genes Kcnq2 and Kcnq3 in mouse. Genomics. 1999;56:360-1 pubmed
  24. Weber Y, Geiger J, Kämpchen K, Landwehrmeyer B, Sommer C, Lerche H. Immunohistochemical analysis of KCNQ2 potassium channels in adult and developing mouse brain. Brain Res. 2006;1077:1-6 pubmed
    ..BFNC is caused by loss-of-function mutations in the potassium channels KCNQ2 and KCNQ3 which can well explain the resulting neuronal hyperexcitability...
  25. Jin Z, Liang G, Cooper E, Jarlebark L. Expression and localization of K channels KCNQ2 and KCNQ3 in the mammalian cochlea. Audiol Neurootol. 2009;14:98-105 pubmed publisher
    ..Here, we describe Kcnq2/3 gene expression and distribution of M channel subunits KCNQ2 and 3 in the cochlea...
  26. Kapfhamer D, Berger K, Hopf F, Seif T, Kharazia V, Bonci A, et al. 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. J Neurosci. 2010;30:8830-40 pubmed publisher
    ..The M-type potassium channel subunit, KCNQ2, is a putative GSK3beta substrate...
  27. Robbins J, Passmore G, Abogadie F, Reilly J, Brown D. Effects of KCNQ2 gene truncation on M-type Kv7 potassium currents. PLoS ONE. 2013;8:e71809 pubmed publisher
    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...
  28. Li S, Choi V, Tzounopoulos T. Pathogenic plasticity of Kv7.2/3 channel activity is essential for the induction of tinnitus. Proc Natl Acad Sci U S A. 2013;110:9980-5 pubmed publisher
    ..Moreover, our findings point to previously unknown biological targets for designing therapeutic drugs that may prevent the development of tinnitus in humans...
  29. Liu W, Devaux J. Calmodulin orchestrates the heteromeric assembly and the trafficking of KCNQ2/3 (Kv7.2/3) channels in neurons. Mol Cell Neurosci. 2014;58:40-52 pubmed publisher
    Mutations in KCNQ2 and KCNQ3 genes are responsible for benign familial neonatal seizures and epileptic encephalopathies...
  30. Zhou X, Wei J, Song M, Francis K, Yu S. Novel role of KCNQ2/3 channels in regulating neuronal cell viability. Cell Death Differ. 2011;18:493-505 pubmed publisher
    ..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...
  31. King C, Lancaster E, Salomon D, Peles E, Scherer S. Kv7.2 regulates the function of peripheral sensory neurons. J Comp Neurol. 2014;522:3262-80 pubmed publisher
    ..The functional role of Kv7.2 has been hampered by the lack of a viable Kcnq2-null animal model...
  32. Soh H, Pant R, LoTurco J, Tzingounis A. Conditional deletions of epilepsy-associated KCNQ2 and KCNQ3 channels from cerebral cortex cause differential effects on neuronal excitability. J Neurosci. 2014;34:5311-21 pubmed publisher
    b>KCNQ2 and KCNQ3 potassium channels have emerged as central regulators of pyramidal neuron excitability and spiking behavior...
  33. Tzingounis A, Nicoll R. Contribution of KCNQ2 and KCNQ3 to the medium and slow afterhyperpolarization currents. Proc Natl Acad Sci U S A. 2008;105:19974-9 pubmed publisher
    ..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)...
  34. Ihara Y, Tomonoh Y, Deshimaru M, Zhang B, Uchida T, Ishii A, et al. Retigabine, a Kv7.2/Kv7.3-Channel Opener, Attenuates Drug-Induced Seizures in Knock-In Mice Harboring Kcnq2 Mutations. PLoS ONE. 2016;11:e0150095 pubmed publisher
    The hetero-tetrameric voltage-gated potassium channel Kv7.2/Kv7.3, which is encoded by KCNQ2 and KCNQ3, plays an important role in limiting network excitability in the neonatal brain. Kv7.2/Kv7...
  35. Martin P, Cifuentes Diaz C, Devaux J, Garcia M, Bureau J, Thomasseau S, et al. Schwannomin-interacting Protein 1 Isoform IQCJ-SCHIP1 Is a Multipartner Ankyrin- and Spectrin-binding Protein Involved in the Organization of Nodes of Ranvier. J Biol Chem. 2017;292:2441-2456 pubmed publisher
    ..Our work reveals that IQCJ-SCHIP1 interacts with several major nodal proteins, and we suggest that it contributes to a higher organizational level of the AnkG/?IV-spectrin network critical for node integrity. ..
  36. Niday Z, Hawkins V, Soh H, Mulkey D, Tzingounis A. Epilepsy-Associated KCNQ2 Channels Regulate Multiple Intrinsic Properties of Layer 2/3 Pyramidal Neurons. J Neurosci. 2017;37:576-586 pubmed publisher
    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...
  37. Zhou P, Yu H, Gu M, Nan F, Gao Z, Li M. Phosphatidylinositol 4,5-bisphosphate alters pharmacological selectivity for epilepsy-causing KCNQ potassium channels. Proc Natl Acad Sci U S A. 2013;110:8726-31 pubmed publisher
    ..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...
  38. Glasscock E, Qian J, Kole M, Noebels J. Transcompartmental reversal of single fibre hyperexcitability in juxtaparanodal Kv1.1-deficient vagus nerve axons by activation of nodal KCNQ channels. J Physiol. 2012;590:3913-26 pubmed publisher