References and models cited by this paper

References and models that cite this paper

Aggarwal SK, MacKinnon R (1996) Contribution of the S4 segment to gating charge in the Shaker K+ channel. Neuron 16:1169-77 [PubMed]

Biervert C, Schroeder BC, Kubisch C, Berkovic SF, Propping P, Jentsch TJ, Steinlein OK (1998) A potassium channel mutation in neonatal human epilepsy. Science 279:403-6 [PubMed]

Borgatti R, Zucca C, Cavallini A, Ferrario M, Panzeri C, Castaldo P, Soldovieri MV, Baschirotto C, Bresolin N, Dalla Bernardina B, Taglialatela M, Bassi MT (2004) A novel mutation in KCNQ2 associated with BFNC, drug resistant epilepsy, and mental retardation. Neurology 63:57-65 [PubMed]

Brown DA, Adams PR (1980) Muscarinic suppression of a novel voltage-sensitive K+ current in a vertebrate neurone. Nature 283:673-6 [PubMed]

Burley SK, Petsko GA (1985) Aromatic-aromatic interaction: a mechanism of protein structure stabilization. Science 229:23-8 [PubMed]

Castaldo P, del Giudice EM, Coppola G, Pascotto A, Annunziato L, Taglialatela M (2002) Benign familial neonatal convulsions caused by altered gating of KCNQ2/KCNQ3 potassium channels. J Neurosci 22:RC199 [PubMed]

Chapman KE, Raol YH, Brooks-Kayal A (2012) Neonatal seizures: controversies and challenges in translating new therapies from the lab to the isolette. Eur J Neurosci 35:1857-65 [Journal] [PubMed]

Charlier C, Singh NA, Ryan SG, Lewis TB, Reus BE, Leach RJ, Leppert M (1998) A pore mutation in a novel KQT-like potassium channel gene in an idiopathic epilepsy family. Nat Genet 18:53-5 [Journal] [PubMed]

Chung HJ, Jan YN, Jan LY (2006) Polarized axonal surface expression of neuronal KCNQ channels is mediated by multiple signals in the KCNQ2 and KCNQ3 C-terminal domains. Proc Natl Acad Sci U S A 103:8870-5 [Journal] [PubMed]

Cooper EC, Harrington E, Jan YN, Jan LY (2001) M channel KCNQ2 subunits are localized to key sites for control of neuronal network oscillations and synchronization in mouse brain. J Neurosci 21:9529-40 [PubMed]

Dedek K, Fusco L, Teloy N, Steinlein OK (2003) Neonatal convulsions and epileptic encephalopathy in an Italian family with a missense mutation in the fifth transmembrane region of KCNQ2. Epilepsy Res 54:21-7

Dedek K, Kunath B, Kananura C, Reuner U, Jentsch TJ, Steinlein OK (2001) Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel. Proc Natl Acad Sci U S A 98:12272-7 [Journal] [PubMed]

Devaux JJ, Kleopa KA, Cooper EC, Scherer SS (2004) KCNQ2 is a nodal K+ channel. J Neurosci 24:1236-44 [Journal] [PubMed]

Glass HC, Glidden D, Jeremy RJ, Barkovich AJ, Ferriero DM, Miller SP (2009) Clinical Neonatal Seizures are Independently Associated with Outcome in Infants at Risk for Hypoxic-Ischemic Brain Injury. J Pediatr 155:318-23 [Journal] [PubMed]

Gunthorpe MJ, Large CH, Sankar R (2012) The mechanism of action of retigabine (ezogabine), a first-in-class K+ channel opener for the treatment of epilepsy. Epilepsia 53:412-24 [Journal] [PubMed]

Hadley JK, Passmore GM, Tatulian L, Al-Qatari M, Ye F, Wickenden AD, Brown DA (2003) Stoichiometry of expressed KCNQ2/KCNQ3 potassium channels and subunit composition of native ganglionic M channels deduced from block by tetraethylammonium. J Neurosci 23:5012-9 [PubMed]

Hermann R, Ferron GM, Erb K, Knebel N, Ruus P, Paul J, Richards L, Cnota HP, Troy S (2003) Effects of age and sex on the disposition of retigabine. Clin Pharmacol Ther 73:61-70 [Journal] [PubMed]

Hines ML, Carnevale NT (1997) The NEURON simulation environment. Neural Comput 9:1179-209 [PubMed]

Long SB, Campbell EB, Mackinnon R (2005) Voltage sensor of Kv1.2: structural basis of electromechanical coupling. Science 309:903-8 [Journal] [PubMed]

Long SB, Tao X, Campbell EB, MacKinnon R (2007) Atomic structure of a voltage-dependent K+ channel in a lipid membrane-like environment. Nature 450:376-82 [Journal] [PubMed]

Maljevic S, Wuttke TV, Lerche H (2008) Nervous system KV7 disorders: breakdown of a subthreshold brake. J Physiol 586:1791-801 [Journal] [PubMed]

Martire M, Castaldo P, D'Amico M, Preziosi P, Annunziato L, Taglialatela M (2004) M channels containing KCNQ2 subunits modulate norepinephrine, aspartate, and GABA release from hippocampal nerve terminals. J Neurosci 24:592-7 [Journal] [PubMed]

Miceli F, Soldovieri MV, Hernandez CC, Shapiro MS, Annunziato L, Taglialatela M (2008) Gating consequences of charge neutralization of arginine residues in the S4 segment of K(v)7.2, an epilepsy-linked K+ channel subunit. Biophys J 95:2254-64 [Journal] [PubMed]

Miceli F, Soldovieri MV, Lugli L, Bellini G, Ambrosino P, Migliore M, del Giudice EM, Ferrari F, Pascotto A, Taglialatela M (2009) Neutralization of a unique, negatively-charged residue in the voltage sensor of K V 7.2 subunits in a sporadic case of benign familial neonatal seizures. Neurobiol Dis 34:501-10 [Journal] [PubMed]

Miceli F, Vargas E, Bezanilla F, Taglialatela M (2012) Gating currents from Kv7 channels carrying neuronal hyperexcitability mutations in the voltage-sensing domain. Biophys J 102:1372-82 [Journal] [PubMed]

Migliore M, Morse TM, Davison AP, Marenco L, Shepherd GM, Hines ML (2003) ModelDB: making models publicly accessible to support computational neuroscience. Neuroinformatics 1:135-9 [Journal] [PubMed]

Nabbout R, Dulac O (2011) Epilepsy. Genetics of early-onset epilepsy with encephalopathy. Nat Rev Neurol 8:129-30

Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, Bellini G, Miceli F, Soldovieri MV, Miragli (1993) Benign Familial Neonatal Seizures J Physiol 586:1791-801

Painter MJ, Scher MS, Stein AD, Armatti S, Wang Z, Gardiner JC, Paneth N, Minnigh B, Alvin J (1999) Phenobarbital compared with phenytoin for the treatment of neonatal seizures. N Engl J Med 341:485-9 [Journal] [PubMed]

Papazian DM, Shao XM, Seoh SA, Mock AF, Huang Y, Wainstock DH (1995) Electrostatic interactions of S4 voltage sensor in Shaker K+ channel. Neuron 14:1293-301 [PubMed]

Peretz A, Pell L, Gofman Y, Haitin Y, Shamgar L, Patrich E, Kornilov P, Gourgy-Hacohen O, Ben-Tal N, Attali B (2010) Targeting the voltage sensor of Kv7.2 voltage-gated K+ channels with a new gating-modifier. Proc Natl Acad Sci U S A 107:15637-42 [Journal] [PubMed]

Peters HC, Hu H, Pongs O, Storm JF, Isbrandt D (2005) Conditional transgenic suppression of M channels in mouse brain reveals functions in neuronal excitability, resonance and behavior. Nat Neurosci 8:51-60 [Journal] [PubMed]

Raol YH, Lapides DA, Keating JG, Brooks-Kayal AR, Cooper EC (2009) A KCNQ channel opener for experimental neonatal seizures and status epilepticus. Ann Neurol 65:326-36 [Journal] [PubMed]

Sadewa AH, Sasongko TH, Gunadi , Lee MJ, Daikoku K, Yamamoto A, Yamasaki T, Tanaka S, Matsuo M, Nishio H (2008) Germ-line mutation of KCNQ2, p.R213W, in a Japanese family with benign familial neonatal convulsion. Pediatr Int 50:167-71 [Journal] [PubMed]

Saitsu H, Kato M, Koide A, Goto T, Fujita T, Nishiyama K, Tsurusaki Y, Doi H, Miyake N, Hayasaka K, Matsumoto N (2012) Whole exome sequencing identifies KCNQ2 mutations in Ohtahara syndrome. Ann Neurol 72:298-300 [Journal] [PubMed]

Sankar R, Painter MJ (2005) Neonatal seizures: after all these years we still love what doesn't work. Neurology 64:776-7 [Journal] [PubMed]

Schwarz JR, Glassmeier G, Cooper EC, Kao TC, Nodera H, Tabuena D, Kaji R, Bostock H (2006) KCNQ channels mediate IKs, a slow K+ current regulating excitability in the rat node of Ranvier. J Physiol 573:17-34 [Journal] [PubMed]

Schwede T, Kopp J, Guex N, Peitsch MC (2003) SWISS-MODEL: An automated protein homology-modeling server. Nucleic Acids Res 31:3381-5 [PubMed]

Shah MM, Migliore M, Valencia I, Cooper EC, Brown DA (2008) Functional significance of axonal Kv7 channels in hippocampal pyramidal neurons. Proc Natl Acad Sci U S A 105:7869-74 [Journal] [PubMed]

Singh NA, Charlier C, Stauffer D, DuPont BR, Leach RJ, Melis R, Ronen GM, Bjerre I, Quattlebaum T, Murphy JV, McHarg ML, Gagnon D, Rosales TO, Peiffer A, Anderson VE, Leppert M (1998) A novel potassium channel gene, KCNQ2, is mutated in an inherited epilepsy of newborns. Nat Genet 18:25-9 [Journal] [PubMed]

Soldovieri MV, Castaldo P, Iodice L, Miceli F, Barrese V, Bellini G, Miraglia del Giudice E, Pascotto A, Bonatti S, Annunziato L, Taglialatela M (2006) 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 281:418-28 [Journal] [PubMed]

Soldovieri MV, Miceli F, Taglialatela M (2011) Driving with no brakes: molecular pathophysiology of Kv7 potassium channels. Physiology (Bethesda) 26:365-76 [Journal] [PubMed]

Steinlein OK, Conrad C, Weidner B (2007) Benign familial neonatal convulsions: always benign? Epilepsy Res 73:245-9 [Journal] [PubMed]

Stewart AP, Gómez-Posada JC, McGeorge J, Rouhani MJ, Villarroel A, Murrell-Lagnado RD, Edwardson JM (2012) The Kv7.2/Kv7.3 heterotetramer assembles with a random subunit arrangement. J Biol Chem 287:11870-7 [Journal] [PubMed]

van Rooij LG, Toet MC, van Huffelen AC, Groenendaal F, Laan W, Zecic A, de Haan T, van Straaten IL, Vrancken S, van Wezel G, van der Sluijs J, Ter Horst H, Gavilanes D, Laroche S, Naulaers G, de Vries LS (2010) Effect of treatment of subclinical neonatal seizures detected with aEEG: randomized, controlled trial. Pediatrics 125:e358-66 [Journal] [PubMed]

Wang HS, Pan Z, Shi W, Brown BS, Wymore RS, Cohen IS, Dixon JE, McKinnon D (1998) KCNQ2 and KCNQ3 potassium channel subunits: molecular correlates of the M-channel. Science 282:1890-3 [PubMed]

Weckhuysen S, Mandelstam S, Suls A, Audenaert D, Deconinck T, Claes LR, Deprez L, Smets K, Hristova D, Yordanova I, Jordanova A, Ceulemans B, Jansen A, Hasaerts D, Roelens F, Lagae L, Yendle S, Stanley T, Heron SE, Mulley JC, Berkovic SF, Scheffer IE, de Jonghe P (2012) KCNQ2 encephalopathy: emerging phenotype of a neonatal epileptic encephalopathy. Ann Neurol 71:15-25 [Journal] [PubMed]

Wuttke TV, Penzien J, Fauler M, Seebohm G, Lehmann-Horn F, Lerche H, Jurkat-Rott K (2008) Neutralization of a negative charge in the S1-S2 region of the KV7.2 (KCNQ2) channel affects voltage-dependent activation in neonatal epilepsy. J Physiol 586:545-55 [Journal] [PubMed]

Cavarretta F, Carnevale NT, Tegolo D, Migliore M (2014) Effects of low frequency electric fields on synaptic integration in hippocampal CA1 pyramidal neurons: implications for power line emissions. Front Cell Neurosci 8:310 [Journal] [PubMed]

Miceli F, Soldovieri MV, Ambrosino P, De Maria M, Migliore M, Migliore R, Taglialatela M (2015) Early-onset epileptic encephalopathy caused by gain-of-function mutations in the voltage sensor of Kv7.2 and Kv7.3 potassium channel subunits. J Neurosci 35:3782-93 [Journal] [PubMed]