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Gene Review

KCNQ3  -  potassium channel, voltage gated KQT-like...

Homo sapiens

Synonyms: BFNC2, EBN2, KQT-like 3, KV7.3, Kv7.3, ...
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Disease relevance of KCNQ3


High impact information on KCNQ3


Biological context of KCNQ3

  • Regulation of KCNQ2/KCNQ3 current by G protein cycling: the kinetics of receptor-mediated signaling by Gq [8].
  • Identification by mass spectrometry and functional characterization of two phosphorylation sites of KCNQ2/KCNQ3 channels [7].
  • Immunoreactivity for KCNQ2, but not KCNQ3, is also prominent in some terminal fields, suggesting a presynaptic role for a distinct subgroup of M-channels in the regulation of action potential propagation and neurotransmitter release [9].
  • By contrast, several BFNC-associated missense mutations in KCNQ2 or KCNQ3 did not alter their surface expression [10].
  • We have tested the association between JME phenotype and an intragenic marker in KCNQ3 by using the transmission disequilibrium test in 119 probands and their parents [11].

Anatomical context of KCNQ3

  • The pharmacological and biophysical properties of the K+ currents observed in the coinjected oocytes differ somewhat from those observed after injecting either KCNQ2 or KCNQ3 by itself [12].
  • The recently identified KCNQ5 gene is expressed in brain and skeletal muscle, and can co-assemble with KCNQ3, suggesting it may also play a role in the M-current heterogeneity [13].
  • To probe if the KCNQ2 and KCNQ3 subtypes underlie the M current of rat superior cervical ganglia (SCG) neurons and of hippocampus, we raised specific antibodies against them and also used the cysteine-alkylating agent N-ethylmaleimide (NEM) as an additional probe of subunit composition [14].
  • We conclude that coexpressed KCNQ2 plus KCNQ3 cDNAs generate channels with 1:1 (KCNQ2:KCNQ3) stoichiometry in CHO cells and that native M channels in SCG neurons adopt the same conformation during development, assisted by the increased expression of KCNQ3 mRNA and protein [15].
  • Finally, Ala 315 of KCNQ3, a residue located in the inner vestibule after the selectivity filter, plays a critical role in preventing current flow in KCNQ3 homomeric channels, whereas it is permissive in heteromers in combination with Thr at the equivalent 276 position of KCNQ2 [16].

Associations of KCNQ3 with chemical compounds

  • Analysis of KCNQ3/4 chimeras determined the C terminus to be responsible for the differential maximal P(o), channel expression, and NEM action between the two channels [17].
  • Using the differential sensitivity of KCNQ3 and KCNQ1 to retigabine, we constructed chimeras to identify minimal segments required for sensitivity to the drug [1].
  • Antibodies and a cysteine-modifying reagent show correspondence of M current in neurons to KCNQ2 and KCNQ3 K+ channels [14].
  • Stoichiometry of expressed KCNQ2/KCNQ3 potassium channels and subunit composition of native ganglionic M channels deduced from block by tetraethylammonium [15].
  • Further, KCNQ2 and KCNQ3 are coassociated with tubulin and protein kinase A within a Triton X-100-insoluble protein complex [9].

Regulatory relationships of KCNQ3

  • KCNQ5 yields currents that activate slowly with depolarization and can form heteromeric channels with KCNQ3 [18].
  • During brain development, KCNQ3 is expressed later than KCNQ2 [19].

Other interactions of KCNQ3

  • Chimeras constructed from different lengths of the KCNQ4 carboxy terminal and the rest KCNQ3 localized a region that confers sensitivity to Ca2+/CaM [20].
  • Using a chimaeric strategy, we show that a cytoplasmic carboxy-terminal subunit interaction domain (sid) suffices to transfer assembly properties between KCNQ3 and KCNQ1 [21].
  • The functional interaction between KCNQ2 and KCNQ3 provides a framework for understanding how mutations in either channel can cause a form of idiopathic generalized epilepsy [12].
  • We demonstrate that KCNE2 associates with KCNQ2 and/or KCNQ3 subunits [22].
  • A short motif, common to KCNQ2 and KCNQ3, mediates both in vivo ankyrin-G interaction and retention of the subunits at the AIS [23].

Analytical, diagnostic and therapeutic context of KCNQ3


  1. Molecular determinants of KCNQ (Kv7) K+ channel sensitivity to the anticonvulsant retigabine. Schenzer, A., Friedrich, T., Pusch, M., Saftig, P., Jentsch, T.J., Grötzinger, J., Schwake, M. J. Neurosci. (2005) [Pubmed]
  2. Conditional transgenic suppression of M channels in mouse brain reveals functions in neuronal excitability, resonance and behavior. Peters, H.C., Hu, H., Pongs, O., Storm, J.F., Isbrandt, D. Nat. Neurosci. (2005) [Pubmed]
  3. Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel. Dedek, K., Kunath, B., Kananura, C., Reuner, U., Jentsch, T.J., Steinlein, O.K. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  4. Localization of a gene for benign adult familial myoclonic epilepsy to chromosome 8q23.3-q24.1. Mikami, M., Yasuda, T., Terao, A., Nakamura, M., Ueno, S., Tanabe, H., Tanaka, T., Onuma, T., Goto, Y., Kaneko, S., Sano, A. Am. J. Hum. Genet. (1999) [Pubmed]
  5. A pore mutation in a novel KQT-like potassium channel gene in an idiopathic epilepsy family. Charlier, C., Singh, N.A., Ryan, S.G., Lewis, T.B., Reus, B.E., Leach, R.J., Leppert, M. Nat. Genet. (1998) [Pubmed]
  6. Moderate loss of function of cyclic-AMP-modulated KCNQ2/KCNQ3 K+ channels causes epilepsy. Schroeder, B.C., Kubisch, C., Stein, V., Jentsch, T.J. Nature (1998) [Pubmed]
  7. Identification by mass spectrometry and functional characterization of two phosphorylation sites of KCNQ2/KCNQ3 channels. Surti, T.S., Huang, L., Jan, Y.N., Jan, L.Y., Cooper, E.C. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  8. Regulation of KCNQ2/KCNQ3 current by G protein cycling: the kinetics of receptor-mediated signaling by Gq. Suh, B.C., Horowitz, L.F., Hirdes, W., Mackie, K., Hille, B. J. Gen. Physiol. (2004) [Pubmed]
  9. Colocalization and coassembly of two human brain M-type potassium channel subunits that are mutated in epilepsy. Cooper, E.C., Aldape, K.D., Abosch, A., Barbaro, N.M., Berger, M.S., Peacock, W.S., Jan, Y.N., Jan, L.Y. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  10. Surface expression and single channel properties of KCNQ2/KCNQ3, M-type K+ channels involved in epilepsy. Schwake, M., Pusch, M., Kharkovets, T., Jentsch, T.J. J. Biol. Chem. (2000) [Pubmed]
  11. Genetic association analysis of KCNQ3 and juvenile myoclonic epilepsy in a South Indian population. Vijai, J., Kapoor, A., Ravishankar, H.M., Cherian, P.J., Girija, A.S., Rajendran, B., Rangan, G., Jayalakshmi, S., Mohandas, S., Radhakrishnan, K., Anand, A. Hum. Genet. (2003) [Pubmed]
  12. Functional expression of two KvLQT1-related potassium channels responsible for an inherited idiopathic epilepsy. Yang, W.P., Levesque, P.C., Little, W.A., Conder, M.L., Ramakrishnan, P., Neubauer, M.G., Blanar, M.A. J. Biol. Chem. (1998) [Pubmed]
  13. KCNQ potassium channels: physiology, pathophysiology, and pharmacology. Robbins, J. Pharmacol. Ther. (2001) [Pubmed]
  14. Antibodies and a cysteine-modifying reagent show correspondence of M current in neurons to KCNQ2 and KCNQ3 K+ channels. Roche, J.P., Westenbroek, R., Sorom, A.J., Hille, B., Mackie, K., Shapiro, M.S. Br. J. Pharmacol. (2002) [Pubmed]
  15. Stoichiometry of expressed KCNQ2/KCNQ3 potassium channels and subunit composition of native ganglionic M channels deduced from block by tetraethylammonium. Hadley, J.K., Passmore, G.M., Tatulian, L., Al-Qatari, M., Ye, F., Wickenden, A.D., Brown, D.A. J. Neurosci. (2003) [Pubmed]
  16. Three mechanisms underlie KCNQ2/3 heteromeric potassium M-channel potentiation. Etxeberria, A., Santana-Castro, I., Regalado, M.P., Aivar, P., Villarroel, A. J. Neurosci. (2004) [Pubmed]
  17. Single-channel analysis of KCNQ K+ channels reveals the mechanism of augmentation by a cysteine-modifying reagent. Li, Y., Gamper, N., Shapiro, M.S. J. Neurosci. (2004) [Pubmed]
  18. KCNQ5, a novel potassium channel broadly expressed in brain, mediates M-type currents. Schroeder, B.C., Hechenberger, M., Weinreich, F., Kubisch, C., Jentsch, T.J. J. Biol. Chem. (2000) [Pubmed]
  19. The KCNQ2 potassium channel: splice variants, functional and developmental expression. Brain localization and comparison with KCNQ3. Tinel, N., Lauritzen, I., Chouabe, C., Lazdunski, M., Borsotto, M. FEBS Lett. (1998) [Pubmed]
  20. Structural requirements for differential sensitivity of KCNQ K+ channels to modulation by Ca2+/calmodulin. Gamper, N., Li, Y., Shapiro, M.S. Mol. Biol. Cell (2005) [Pubmed]
  21. A carboxy-terminal domain determines the subunit specificity of KCNQ K+ channel assembly. Schwake, M., Jentsch, T.J., Friedrich, T. EMBO Rep. (2003) [Pubmed]
  22. M-type KCNQ2-KCNQ3 potassium channels are modulated by the KCNE2 subunit. Tinel, N., Diochot, S., Lauritzen, I., Barhanin, J., Lazdunski, M., Borsotto, M. FEBS Lett. (2000) [Pubmed]
  23. A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axon. Pan, Z., Kao, T., Horvath, Z., Lemos, J., Sul, J.Y., Cranstoun, S.D., Bennett, V., Scherer, S.S., Cooper, E.C. J. Neurosci. (2006) [Pubmed]
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