| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
J Biol Chem, Vol. 275, Issue 18, 13343-13348, May 5, 2000
From the Mutations in either KCNQ2 or KCNQ3 underlie
benign familial neonatal convulsions (BFNC), an inherited epilepsy. The
corresponding proteins are co-expressed in broad regions of the brain
and associate to heteromeric K+ channels. These
channels mediate M-type currents that regulate neuronal excitability.
We investigated the basis for the increase in currents seen after
co-expressing these subunits in Xenopus oocytes. Noise
analysis and single channel recordings revealed a conductance of
Surface Expression and Single Channel Properties of KCNQ2/KCNQ3,
M-type K+ Channels Involved in Epilepsy*
§¶,
,, and**
Zentrum für Molekulare Neurobiologie
Hamburg (ZMNH), Universität Hamburg, Martinistrasse 85, D-20246
Hamburg, Germany and the Istituto di Cibernetica e Biofisica,
Via de Marini 6, I-16149 Genova, Italy
18 pS for KCNQ2 and 7 pS for KCNQ3. Different conductance levels
(ranging from 8 to 22 pS) were seen upon co-expression. Their weighted
average is close to that obtained by noise analysis (16 pS). The open
probability of heteromeric channels was not increased significantly.
Co-expression of both subunits increased the surface expression of
KCNQ2 and KCNQ3 by factors of 5 and >10, respectively. A KCNQ2 mutant
associated with BFNC that has a truncated cytoplasmic carboxyl terminus
did not reach the surface and failed to stimulate KCNQ3 surface
expression. By contrast, several BFNC-associated missense mutations in
KCNQ2 or KCNQ3 did not alter their surface expression. Thus, the
increase in currents seen upon co-expressing KCNQ2 and KCNQ3 is
predominantly due to an increase in surface expression, which is
dependent on an intact carboxyl terminus.
*
This work was supported by grants from the Deutsche
Forschungsgemeinschaft and the Fonds der Chemischen Industrie (to
T. J. J.) and by Telethon Italy Grant 1079 (to M. P.).The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  A. Etxeberria, P. Aivar, J. A. Rodriguez-Alfaro, A. Alaimo, P. Villace, J. C. Gomez-Posada, P. Areso, and A. Villarroel Calmodulin regulates the trafficking of KCNQ2 potassium channels FASEB J, April 1, 2008; 22(4): 1135 - 1143. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. H. Hansen, O. Waroux, V. Seutin, T. J. Jentsch, S. Aznar, and J. D. Mikkelsen Kv7 channels: interaction with dopaminergic and serotonergic neurotransmission in the CNS J. Physiol., April 1, 2008; 586(7): 1823 - 1832. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Maljevic, T. V. Wuttke, and H. Lerche Nervous system KV7 disorders: breakdown of a subthreshold brake J. Physiol., April 1, 2008; 586(7): 1791 - 1801. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. B. Rasmussen, C. Frokjaer-Jensen, C. S. Jensen, H. S. Jensen, N. K. Jorgensen, H. Misonou, J. S. Trimmer, S.-P. Olesen, and N. Schmitt Requirement of subunit co-assembly and ankyrin-G for M-channel localization at the axon initial segment J. Cell Sci., March 15, 2007; 120(6): 953 - 963. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Xicluna, B. Lacombe, I. Dreyer, C. Alcon, L. Jeanguenin, H. Sentenac, J.-B. Thibaud, and I. Cherel Increased Functional Diversity of Plant K+ Channels by Preferential Heteromerization of the Shaker-like Subunits AKT2 and KAT2 J. Biol. Chem., January 5, 2007; 282(1): 486 - 494. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Dahimene, S. Alcolea, P. Naud, P. Jourdon, D. Escande, R. Brasseur, A. Thomas, I. Baro, and J. Merot The N-Terminal Juxtamembranous Domain of KCNQ1 Is Critical for Channel Surface Expression: Implications in the Romano-Ward LQT1 Syndrome Circ. Res., November 10, 2006; 99(10): 1076 - 1083. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. J. Chung, Y. N. Jan, and L. Y. Jan Polarized axonal surface expression of neuronal KCNQ channels is mediated by multiple signals in the KCNQ2 and KCNQ3 C-terminal domains PNAS, June 6, 2006; 103(23): 8870 - 8875. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Schwake, D. Athanasiadu, C. Beimgraben, J. Blanz, C. Beck, T. J. Jentsch, P. Saftig, and T. Friedrich Structural determinants of M-type KCNQ (Kv7) K+ channel assembly. J. Neurosci., April 5, 2006; 26(14): 3757 - 3766. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Z. Pan, T. Kao, Z. Horvath, J. Lemos, J.-Y. Sul, S. D. Cranstoun, V. Bennett, S. S. Scherer, and E. C. Cooper A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axon. J. Neurosci., March 8, 2006; 26(10): 2599 - 2613. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. V. Soldovieri, P. Castaldo, L. Iodice, F. Miceli, V. Barrese, G. Bellini, E. M. del Giudice, A. Pascotto, S. Bonatti, L. Annunziato, et al. Decreased Subunit Stability as a Novel Mechanism for Potassium Current Impairment by a KCNQ2 C Terminus Mutation Causing Benign Familial Neonatal Convulsions J. Biol. Chem., January 6, 2006; 281(1): 418 - 428. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. S. Surti, L. Huang, Y. N. Jan, L. Y. Jan, and E. C. Cooper Identification by mass spectrometry and functional characterization of two phosphorylation sites of KCNQ2/KCNQ3 channels PNAS, December 6, 2005; 102(49): 17828 - 17833. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Jespersen, M. Grunnet, and S.-P. Olesen The KCNQ1 Potassium Channel: From Gene to Physiological Function Physiology, December 1, 2005; 20(6): 408 - 416. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y. Li, N. Gamper, D. W. Hilgemann, and M. S. Shapiro Regulation of Kv7 (KCNQ) K+ Channel Open Probability by Phosphatidylinositol 4,5-Bisphosphate J. Neurosci., October 26, 2005; 25(43): 9825 - 9835. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Schenzer, T. Friedrich, M. Pusch, P. Saftig, T. J. Jentsch, J. Grotzinger, and M. Schwake Molecular Determinants of KCNQ (Kv7) K+ Channel Sensitivity to the Anticonvulsant Retigabine J. Neurosci., May 18, 2005; 25(20): 5051 - 5060. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. R.F. Claes, B. Ceulemans, D. Audenaert, L. Deprez, A. Jansen, D. Hasaerts, S. Weckx, K. G. Claeys, J. Del-Favero, C. Van Broeckhoven, et al. De novo KCNQ2 mutations in patients with benign neonatal seizures Neurology, December 14, 2004; 63(11): 2155 - 2158. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Etxeberria, I. Santana-Castro, M. P. Regalado, P. Aivar, and A. Villarroel Three Mechanisms Underlie KCNQ2/3 Heteromeric Potassium M-Channel Potentiation J. Neurosci., October 13, 2004; 24(41): 9146 - 9152. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. Estevez, M. Pusch, C. Ferrer-Costa, M. Orozco, and T. J. Jentsch Functional and structural conservation of CBS domains from CLC chloride channels J. Physiol., June 1, 2004; 557(2): 363 - 378. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M C Richards, S E Heron, H E Spendlove, I E Scheffer, B Grinton, S F Berkovic, J C Mulley, and A Davy Novel mutations in the KCNQ2 gene link epilepsy to a dysfunction of the KCNQ2-calmodulin interaction J. Med. Genet., March 1, 2004; 41(3): e35 - 35. [Full Text] [PDF]
|
|
|
|
 |
|
 N. A. Singh, P. Westenskow, C. Charlier, C. Pappas, J. Leslie, J. Dillon, V. E. Anderson, M. C. Sanguinetti, and M. F. Leppert KCNQ2 and KCNQ3 potassium channel genes in benign familial neonatal convulsions: expansion of the functional and mutation spectrum Brain, December 1, 2003; 126(12): 2726 - 2737. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. L. Prole, P. A. Lima, and N. V. Marrion Mechanisms Underlying Modulation of Neuronal KCNQ2/KCNQ3 Potassium Channels by Extracellular Protons J. Gen. Physiol., November 24, 2003; 122(6): 775 - 793. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. M. Passmore, A. A. Selyanko, M. Mistry, M. Al-Qatari, S. J. Marsh, E. A. Matthews, A. H. Dickenson, T. A. Brown, S. A. Burbidge, M. Main, et al. KCNQ/M Currents in Sensory Neurons: Significance for Pain Therapy J. Neurosci., August 6, 2003; 23(18): 7227 - 7236. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. K. Hadley, G. M. Passmore, L. Tatulian, M. Al-Qatari, F. Ye, A. D. Wickenden, and D. A. Brown Stoichiometry of Expressed KCNQ2/KCNQ3 Potassium Channels and Subunit Composition of Native Ganglionic M Channels Deduced from Block by Tetraethylammonium J. Neurosci., June 15, 2003; 23(12): 5012 - 5019. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Wen and I. B. Levitan Calmodulin Is an Auxiliary Subunit of KCNQ2/3 Potassium Channels J. Neurosci., September 15, 2002; 22(18): 7991 - 8001. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Altier, S. J. Dubel, C. Barrere, S. E. Jarvis, S. C. Stotz, R. L. Spaetgens, J. D. Scott, V. Cornet, M. De Waard, G. W. Zamponi, et al. Trafficking of L-type Calcium Channels Mediated by the Postsynaptic Scaffolding Protein AKAP79 J. Biol. Chem., September 6, 2002; 277(37): 33598 - 33603. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Yus-Najera, I. Santana-Castro, and A. Villarroel The Identification and Characterization of a Noncontinuous Calmodulin-binding Site in Noninactivating Voltage-dependent KCNQ Potassium Channels J. Biol. Chem., August 2, 2002; 277(32): 28545 - 28553. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Dedek, B. Kunath, C. Kananura, U. Reuner, T. J. Jentsch, and O. K. Steinlein Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel PNAS, September 19, 2001; (2001) 211431298. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. S. Smith, C. A. Iannotti, P. Dargis, E. P. Christian, and J. Aiyar Differential Expression of KCNQ2 Splice Variants: Implications to M Current Function during Neuronal Development J. Neurosci., February 15, 2001; 21(4): 1096 - 1103. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. C. Schroeder, M. Hechenberger, F. Weinreich, C. Kubisch, and T. J. Jentsch KCNQ5, a Novel Potassium Channel Broadly Expressed in Brain, Mediates M-type Currents J. Biol. Chem., July 28, 2000; 275(31): 24089 - 24095. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Dedek, B. Kunath, C. Kananura, U. Reuner, T. J. Jentsch, and O. K. Steinlein Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel PNAS, October 9, 2001; 98(21): 12272 - 12277. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Castaldo, E. M. del Giudice, G. Coppola, A. Pascotto, L. Annunziato, and M. Taglialatela Benign Familial Neonatal Convulsions Caused by Altered Gating of KCNQ2/KCNQ3 Potassium Channels J. Neurosci., January 15, 2002; 22(2): RC199 - RC199. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| All ASBMB Journals | Molecular and Cellular Proteomics |
| Journal of Lipid Research | ASBMB Today |
