{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T18:12:58Z","timestamp":1791051178667,"version":"4.1.0"},"reference-count":133,"publisher":"Frontiers Media SA","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Front. Pharmacol."],"DOI":"10.3389\/fphar.2014.00053","type":"journal-article","created":{"date-parts":[[2014,4,3]],"date-time":"2014-04-03T07:56:07Z","timestamp":1396511767000},"source":"Crossref","is-referenced-by-count":81,"title":["Biophysics, pathophysiology, and pharmacology of ion channel gating pores"],"prefix":"10.3389","volume":"5","author":[{"given":"Adrien","family":"Moreau","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Gosselin-Badaroudine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Chahine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2014,4,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1007\/s00424-009-0761-0","article-title":"Cardiac sodium channelopathies","volume":"460","author":"Amin","year":"2010","journal-title":"Pflugers Arch"},{"key":"B2","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1038\/242459a0","article-title":"Currents related to movement of the gating particles of the sodium channels","volume":"242","author":"Armstrong","year":"1973","journal-title":"Nature"},{"key":"B3","doi-asserted-by":"publisher","first-page":"8464","DOI":"10.1021\/bi00194a010","article-title":"Isoform specific interactions of troponin I and troponin C determine pH sensitivity of myofibrillar Ca2+ activation","volume":"33","author":"Ball","year":"1994","journal-title":"Biochemistry"},{"key":"B4","doi-asserted-by":"publisher","first-page":"1019","DOI":"10.1172\/JCI200318062","article-title":"Congenital sick sinus syndrome caused by recessive mutations in the cardiac sodium channel gene (SCN5A)","volume":"112","author":"Benson","year":"2003","journal-title":"J. Clin. Invest"},{"key":"B5","doi-asserted-by":"publisher","first-page":"991","DOI":"10.1016\/j.neuron.2011.11.014","article-title":"The pore of the voltage-gated proton channel","volume":"72","author":"Berger","year":"2011","journal-title":"Neuron"},{"key":"B6","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1161\/01.RES.0000052672.97759.36","article-title":"Compound heterozygosity for mutations (W156X and R225W) in SCN5A associated with severe cardiac conduction disturbances and degenerative changes in the conduction system","volume":"92","author":"Bezzina","year":"2003","journal-title":"Circ. Res"},{"key":"B7","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1016\/S0006-3495(01)75687-1","article-title":"Gating properties of gap junction channels assembled from connexin43 and connexin43 fused with green fluorescent protein","volume":"81","author":"Bukauskas","year":"2001","journal-title":"Biophys. J"},{"key":"B8","doi-asserted-by":"publisher","first-page":"7904","DOI":"10.1073\/pnas.0702638104","article-title":"Two atomic constraints unambiguously position the S4 segment relative to S1 and S2 segments in the closed state of Shaker K channel","volume":"104","author":"Campos","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B9","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1146\/annurev.neuro.29.051605.112815","article-title":"Pathomechanisms in channelopathies of skeletal muscle and brain","volume":"29","author":"Cannon","year":"2006","journal-title":"Annu. Rev. Neurosci"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1887","DOI":"10.1113\/jphysiol.2010.186874","article-title":"Voltage-sensor mutations in channelopathies of skeletal muscle","volume":"588","author":"Cannon","year":"2010","journal-title":"J. Physiol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1016\/j.tcb.2010.09.006","article-title":"pH regulation and beyond: unanticipated functions for the voltage-gated proton channel, HVCN1","volume":"21","author":"Capasso","year":"2011","journal-title":"Trends Cell Biol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"953","DOI":"10.1146\/annurev.bi.55.070186.004513","article-title":"Molecular properties of voltage-sensitive sodium channels","volume":"55","author":"Catterall","year":"1986","journal-title":"Annu. Rev. Biochem"},{"key":"B13","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1016\/j.toxicon.2006.09.022","article-title":"Voltage-gated ion channels and gating modifier toxins","volume":"49","author":"Catterall","year":"2007","journal-title":"Toxicon"},{"key":"B14","doi-asserted-by":"publisher","first-page":"883","DOI":"10.1016\/S0300-9084(00)01174-3","article-title":"Molecular mechanisms of neurotoxin action on voltage-gated sodium channels","volume":"82","author":"Cestele","year":"2000","journal-title":"Biochimie"},{"key":"B15","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1007\/BF00585952","article-title":"Functional expression and properties of the human skeletal muscle sodium channel","volume":"427","author":"Chahine","year":"1994a","journal-title":"Pflugers Arch"},{"key":"B16","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1016\/0896-6273(94)90271-2","article-title":"Sodium channel mutations in paramyotonia congenita uncouple inactivation from activation","volume":"12","author":"Chahine","year":"1994b","journal-title":"Neuron"},{"key":"B17","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1016\/S0006-3495(98)77510-1","article-title":"Extrapore residues of the S5-S6 loop of domain 2 of the voltage-gated skeletal muscle sodium channel (rSkM1) contribute to the mu-conotoxin GIIIA binding site","volume":"75","author":"Chahine","year":"1998","journal-title":"Biophys. J"},{"key":"B18","doi-asserted-by":"publisher","first-page":"629","DOI":"10.1085\/jgp.20028679","article-title":"Tracking voltage-dependent conformational changes in skeletal muscle sodium channel during activation","volume":"120","author":"Chanda","year":"2002","journal-title":"J. Gen. Physiol"},{"key":"B19","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1111\/j.1752-8062.2010.00249.x","article-title":"SCN5A rare variants in familial dilated cardiomyopathy decrease peak sodium current depending on the common polymorphism H558R and common splice variant Q1077del","volume":"3","author":"Cheng","year":"2010","journal-title":"Clin. Trans. Sci"},{"key":"B20","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1085\/jgp.200308813","article-title":"Properties of single voltage-gated proton channels in human eosinophils estimated by noise analysis and by direct measurement","volume":"121","author":"Cherny","year":"2003","journal-title":"J. Gen. Physiol"},{"key":"B21","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1016\/S0014-5793(03)00666-5","article-title":"Distinct primary structures of the major peptide toxins from the venom of the spider Macrothele gigas that bind to sites 3 and 4 in the sodium channel","volume":"547","author":"Corzo","year":"2003","journal-title":"FEBS Lett"},{"key":"B22","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1152\/physrev.00028.2002","article-title":"Voltage-gated proton channels and other proton transfer pathways","volume":"83","author":"Decoursey","year":"2003","journal-title":"Physiol. Rev"},{"key":"B23","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1152\/physrev.00011.2012","article-title":"Voltage-gated proton channels: molecular biology, physiology, and pathophysiology of the HV family","volume":"93","author":"Decoursey","year":"2013","journal-title":"Physiol. Rev"},{"key":"B24","doi-asserted-by":"publisher","first-page":"12272","DOI":"10.1073\/pnas.211431298","article-title":"Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel","volume":"98","author":"Dedek","year":"2001","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B25","doi-asserted-by":"publisher","first-page":"6109","DOI":"10.1073\/pnas.1102724108","article-title":"Intermediate states of the Kv1.2 voltage sensor from atomistic molecular dynamics simulations","volume":"108","author":"Delemotte","year":"2011","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B26","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1126\/science.280.5360.69","article-title":"The structure of the potassium channel: molecular basis of K+ conduction and selectivity","volume":"280","author":"Doyle","year":"1998","journal-title":"Science"},{"key":"B27","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1016\/S0006-3495(62)86959-8","article-title":"Cation selective glass electrodes and their mode of operation","volume":"2","author":"Eisenman","year":"1962","journal-title":"Biophys. J"},{"key":"B28","doi-asserted-by":"publisher","first-page":"512","DOI":"10.1016\/j.bbrc.2005.12.038","article-title":"A voltage-activated proton current in human cardiac fibroblasts","volume":"340","author":"El Chemaly","year":"2006","journal-title":"Biochem. Biophys. Res. Commun"},{"key":"B29","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1113\/jphysiol.1978.sp012231","article-title":"Effects of pH on the myofilaments and the sarcoplasmic reticulum of skinned cells from cardiace and skeletal muscles","volume":"276","author":"Fabiato","year":"1978","journal-title":"J. Physiol"},{"key":"B30","doi-asserted-by":"publisher","first-page":"3775","DOI":"10.1093\/brain\/awt300","article-title":"Transient compartment-like syndrome and normokalaemic periodic paralysis due to a Cav1.1 mutation","volume":"136","author":"Fan","year":"2013","journal-title":"Brain"},{"key":"B31","doi-asserted-by":"publisher","first-page":"1635","DOI":"10.1212\/WNL.0b013e318219fb57","article-title":"Leaky sodium channels from voltage sensor mutations in periodic paralysis, but not paramyotonia","volume":"76","author":"Francis","year":"2011","journal-title":"Neurology"},{"key":"B32","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1093\/europace\/eum053","article-title":"Homozygous SCN5A mutation in Brugada syndrome with monomorphic ventricular tachycardia and structural heart abnormalities","volume":"9","author":"Frigo","year":"2007","journal-title":"Europace"},{"key":"B33","doi-asserted-by":"publisher","first-page":"93","DOI":"10.4161\/chan.4.2.10672","article-title":"Double gaps along Shaker S4 demonstrate omega currents at three different closed states","volume":"4","author":"Gamal El-Din","year":"2010","journal-title":"Channels (Austin)"},{"key":"B34","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1161\/CIRCEP.107.750752","article-title":"Molecular and clinical characterization of a novel SCN5A mutation associated with atrioventricular block and dilated cardiomyopathy","volume":"1","author":"Ge","year":"2008","journal-title":"Circ. Arrhythm. Electrophysiol"},{"key":"B35","doi-asserted-by":"publisher","first-page":"554","DOI":"10.1073\/pnas.89.2.554","article-title":"Primary structure and functional expression of the human cardiac tetrodotoxin-insensitive voltage-dependent sodium channel","volume":"89","author":"Gellens","year":"1992","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B36","doi-asserted-by":"publisher","first-page":"19250","DOI":"10.1073\/pnas.1217990109","article-title":"Gating pore currents and the resting state of Nav1.4 voltage sensor domains","volume":"109","author":"Gosselin-Badaroudine","year":"2012a","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B37","doi-asserted-by":"publisher","first-page":"e38331","DOI":"10.1371\/journal.pone.0038331","article-title":"A proton leak current through the cardiac sodium channel is linked to mixed arrhythmia and the dilated cardiomyopathy phenotype","volume":"7","author":"Gosselin-Badaroudine","year":"2012b","journal-title":"PLoS ONE"},{"key":"B38","doi-asserted-by":"publisher","first-page":"8","DOI":"10.4161\/chan.27179","article-title":"Na 1.5 mutations linked to dilated cardiomyopathy phenotypes: is the gating pore current the missing link?","author":"Gosselin-Badaroudine","year":"2013","journal-title":"Channels (Austin)"},{"key":"B39","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1161\/01.RES.0000050585.07097.D7","article-title":"A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill","volume":"92","author":"Groenewegen","year":"2003","journal-title":"Circ. Res"},{"key":"B40","doi-asserted-by":"publisher","first-page":"998","DOI":"10.1093\/brain\/awu015","article-title":"NaV1.4 mutations cause hypokalaemic periodic paralysis by disrupting IIIS4 movement during recovery","volume":"137","author":"Groome","year":"2014","journal-title":"Brain"},{"key":"B41","doi-asserted-by":"publisher","first-page":"e10985","DOI":"10.1371\/journal.pone.0010985","article-title":"Multiple loss-of-function mechanisms contribute to SCN5A-related familial sick sinus syndrome","volume":"5","author":"Gui","year":"2010a","journal-title":"PLoS ONE"},{"key":"B42","doi-asserted-by":"publisher","first-page":"564","DOI":"10.1111\/j.1540-8167.2010.01762.x","article-title":"Mutation-specific effects of polymorphism H558R in SCN5A-related sick sinus syndrome","volume":"21","author":"Gui","year":"2010b","journal-title":"J. Cardiovasc. Electrophysiol"},{"key":"B43","doi-asserted-by":"publisher","first-page":"508","DOI":"10.1073\/pnas.83.2.508","article-title":"Molecular model of the action potential sodium channel","volume":"83","author":"Guy","year":"1986","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B44","doi-asserted-by":"publisher","first-page":"1486","DOI":"10.1002\/humu.21106","article-title":"The genetic basis of long QT and short QT syndromes: a mutation update","volume":"30","author":"Hedley","year":"2009","journal-title":"Hum. Mutat"},{"key":"B45","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1038\/356441a0","article-title":"Calcium channel characteristics conferred on the sodium channel by single mutations","volume":"356","author":"Heinemann","year":"1992","journal-title":"Nature"},{"key":"B46","doi-asserted-by":"publisher","first-page":"1811","DOI":"10.1113\/jphysiol.2007.148304","article-title":"Regulation of neural KCNQ channels: signalling pathways, structural motifs and functional implications","volume":"586","author":"Hernandez","year":"2008","journal-title":"J. Physiol"},{"key":"B47","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1111\/j.1752-8062.2008.00017.x","article-title":"Coding sequence mutations identified in MYH7, TNNT2, SCN5A, CSRP3, LBD3, and TCAP from 313 patients with familial or idiopathic dilated cardiomyopathy","volume":"1","author":"Hershberger","year":"2008","journal-title":"Clin. Trans. Sci"},{"key":"B48","doi-asserted-by":"publisher","first-page":"1641","DOI":"10.1016\/j.jacc.2011.01.015","article-title":"Update 2011: clinical and genetic issues in familial dilated cardiomyopathy","volume":"57","author":"Hershberger","year":"2011","journal-title":"J. Am. Coll. Cardiol"},{"key":"B49","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1152\/physrev.00021.2009","article-title":"Inwardly rectifying potassium channels: their structure, function, and physiological roles","volume":"90","author":"Hibino","year":"2010","journal-title":"Physiol. Rev"},{"key":"B50","doi-asserted-by":"publisher","first-page":"S47","DOI":"10.1111\/j.1540-8167.2006.00383.x","article-title":"Molecular control of cardiac sodium homeostasis in health and disease","volume":"17","author":"Hilgemann","year":"2006","journal-title":"J Cardiovasc. Electrophysiol"},{"key":"B51","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1113\/jphysiol.1952.sp004764","article-title":"A quantitative description of membrane current and its application to conduction and excitation in nerve","volume":"117","author":"Hodgkin","year":"1952","journal-title":"J. Physiol"},{"key":"B52","doi-asserted-by":"publisher","first-page":"7970","DOI":"10.1073\/pnas.0803936105","article-title":"A voltage-sensing phosphatase, Ci-VSP, which shares sequence identity with PTEN, dephosphorylates phosphatidylinositol 4,5-bisphosphate","volume":"105","author":"Iwasaki","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B53","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1038\/nature01580","article-title":"X-ray structure of a voltage-dependent K+ channel","volume":"423","author":"Jiang","year":"2003","journal-title":"Nature"},{"issue":"112","key":"B54","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2012.00112","article-title":"Pathophysiological role of omega pore current in channelopathies","volume":"3","author":"Jurkat-Rott","year":"2012","journal-title":"Front. Pharmacol"},{"key":"B55","doi-asserted-by":"publisher","first-page":"305","DOI":"10.1007\/978-90-481-9485-8_18","article-title":"Hereditary channelopathies in neurology","volume":"686","author":"Jurkat-Rott","year":"2010","journal-title":"Adv. Exp. Med. Biol"},{"key":"B56","doi-asserted-by":"publisher","first-page":"9549","DOI":"10.1073\/pnas.97.17.9549","article-title":"Voltage-sensor sodium channel mutations cause hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced current","volume":"97","author":"Jurkat-Rott","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B57","doi-asserted-by":"publisher","first-page":"4036","DOI":"10.1073\/pnas.0811277106","article-title":"K+-dependent paradoxical membrane depolarization and Na+ overload, major and reversible contributors to weakness by ion channel leaks","volume":"106","author":"Jurkat-Rott","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B58","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1016\/j.bpj.2011.10.057","article-title":"Molecular dynamics investigation of the omega-current in the Kv1.2 voltage sensor domains","volume":"102","author":"Khalili-Araghi","year":"2012","journal-title":"Biophys. J"},{"key":"B59","doi-asserted-by":"publisher","first-page":"3035","DOI":"10.1152\/jn.00858.2005","article-title":"Atypical phenotypes from flatworm Kv3 channels","volume":"95","author":"Klassen","year":"2006","journal-title":"J. Neurophysiol"},{"key":"B60","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1186\/1471-2202-9-52","article-title":"A naturally occurring omega current in a Kv3 family potassium channel from a platyhelminth","volume":"9","author":"Klassen","year":"2008","journal-title":"BMC Neurosci"},{"key":"B61","doi-asserted-by":"publisher","first-page":"1606","DOI":"10.1161\/CIRCULATIONAHA.109.914911","article-title":"Elevated cytosolic Na+ increases mitochondrial formation of reactive oxygen species in failing cardiac myocytes","volume":"121","author":"Kohlhaas","year":"2010","journal-title":"Circulation"},{"key":"B62","doi-asserted-by":"publisher","first-page":"1623","DOI":"10.1096\/fj.02-0238hyp","article-title":"Family ties of gated pores: evolution of the sensor module","volume":"16","author":"Kumanovics","year":"2002","journal-title":"FASEB J"},{"key":"B63","doi-asserted-by":"publisher","first-page":"480","DOI":"10.1111\/j.1540-8167.2006.00411.x","article-title":"SCN5A mutation associated with cardiac conduction defect and atrial arrhythmias","volume":"17","author":"Laitinen-Forsblom","year":"2006","journal-title":"J. Cardiovasc. Electrophysiol"},{"key":"B64","doi-asserted-by":"publisher","first-page":"244","DOI":"10.1016\/0014-5793(96)00173-1","article-title":"Electrophysiological properties of the hypokalaemic periodic paralysis mutation (R528H) of the skeletal muscle alpha 1s subunit as expressed in mouse L cells","volume":"382","author":"Lapie","year":"1996","journal-title":"FEBS Lett"},{"key":"B65","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1016\/j.jacc.2012.02.052","article-title":"Multifocal ectopic Purkinje-related premature contractions: a new SCN5A-related cardiac channelopathy","volume":"60","author":"Laurent","year":"2012","journal-title":"J. Am. Coll. Cardiol"},{"key":"B66","doi-asserted-by":"publisher","first-page":"186","DOI":"10.3988\/jcn.2009.5.4.186","article-title":"Clinical diversity of SCN4A-mutation-associated skeletal muscle sodium channelopathy","volume":"5","author":"Lee","year":"2009","journal-title":"J. Clin. Neurol"},{"key":"B67","doi-asserted-by":"crossref","first-page":"253","DOI":"10.4161\/chan.4847","article-title":"muO conotoxins inhibit NaV channels by interfering with their voltage sensors in domain-2","volume":"1","author":"Leipold","year":"2007","journal-title":"Channels (Austin)"},{"key":"B68","doi-asserted-by":"publisher","first-page":"2737","DOI":"10.2174\/1381612054546815","article-title":"Ion channel defects in idiopathic epilepsies","volume":"11","author":"Lerche","year":"2005","journal-title":"Curr. Pharm. Des"},{"key":"B69","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1085\/jgp.201110642","article-title":"R1 in the Shaker S4 occupies the gating charge transfer center in the resting state","volume":"138","author":"Lin","year":"2011","journal-title":"J. Gen. Physiol"},{"key":"B70","doi-asserted-by":"publisher","first-page":"e38259","DOI":"10.1371\/journal.pone.0038259","article-title":"Disease-related cardiac troponins alter thin filament ca(2+) association and dissociation rates","volume":"7","author":"Liu","year":"2012","journal-title":"PLoS ONE"},{"key":"B71","doi-asserted-by":"publisher","first-page":"897","DOI":"10.1126\/science.1116269","article-title":"Crystal structure of a mammalian voltage-dependent Shaker family K+ channel","volume":"309","author":"Long","year":"2005","journal-title":"Science"},{"key":"B72","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1038\/nature06265","article-title":"Atomic structure of a voltage-dependent K+ channel in a lipid membrane-like environment","volume":"450","author":"Long","year":"2007","journal-title":"Nature"},{"key":"B73","doi-asserted-by":"publisher","first-page":"172","DOI":"10.1161\/01.RES.0000232546.92777.05","article-title":"Elevated cytosolic Na+ decreases mitochondrial Ca2+ uptake during excitation-contraction coupling and impairs energetic adaptation in cardiac myocytes","volume":"99","author":"Maack","year":"2006","journal-title":"Circ. Res"},{"key":"B74","doi-asserted-by":"publisher","first-page":"1566","DOI":"10.1016\/j.jacc.2012.05.050","article-title":"R222Q SCN5A mutation is associated with reversible ventricular ectopy and dilated cardiomyopathy","volume":"60","author":"Mann","year":"2012","journal-title":"J. Am. Coll. Cardiol"},{"key":"B75","doi-asserted-by":"crossref","first-page":"4452","DOI":"10.1016\/S0021-9258(18)61214-1","article-title":"Purification and chemical and biological characterizations of seven toxins from the Mexican scorpion, Centruroides suffusus suffusus","volume":"262","author":"Martin","year":"1987","journal-title":"J. Biol. Chem"},{"key":"B76","doi-asserted-by":"publisher","first-page":"1102","DOI":"10.1038\/ncomms2077","article-title":"Structure of a bacterial voltage-gated sodium channel pore reveals mechanisms of opening and closing","volume":"3","author":"McCusker","year":"2012","journal-title":"Nat. Commun"},{"key":"B77","doi-asserted-by":"publisher","first-page":"2163","DOI":"10.1161\/01.CIR.0000144458.58660.BB","article-title":"SCN5A mutation associated with dilated cardiomyopathy, conduction disorder, and arrhythmia","volume":"110","author":"McNair","year":"2004","journal-title":"Circulation"},{"key":"B78","doi-asserted-by":"publisher","first-page":"2160","DOI":"10.1016\/j.jacc.2010.09.084","article-title":"SCN5A mutations associate with arrhythmic dilated cardiomyopathy and commonly localize to the voltage-sensing mechanism","volume":"57","author":"McNair","year":"2011","journal-title":"J. Am. Coll. Cardiol"},{"key":"B79","doi-asserted-by":"crossref","first-page":"10320","DOI":"10.1523\/JNEUROSCI.18-24-10320.1998","article-title":"Gating of the L-type Ca channel in human skeletal myotubes: an activation defect caused by the hypokalemic periodic paralysis mutation R528H","volume":"18","author":"Morrill","year":"1998","journal-title":"J. Neurosci"},{"key":"B80","doi-asserted-by":"publisher","first-page":"1239","DOI":"10.1038\/nature03650","article-title":"Phosphoinositide phosphatase activity coupled to an intrinsic voltage sensor","volume":"435","author":"Murata","year":"2005","journal-title":"Nature"},{"key":"B81","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1038\/nature10557","article-title":"Aspartate 112 is the selectivity filter of the human voltage-gated proton channel","volume":"480","author":"Musset","year":"2011","journal-title":"Nature"},{"key":"B82","doi-asserted-by":"publisher","first-page":"298","DOI":"10.1073\/pnas.75.1.298","article-title":"Molecular mechanisms for proton transport in membranes","volume":"75","author":"Nagle","year":"1978","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B83","doi-asserted-by":"publisher","first-page":"1681","DOI":"10.1016\/j.hrthm.2012.06.029","article-title":"Escape capture bigeminy: phenotypic marker of cardiac sodium channel voltage sensor mutation R222Q","volume":"9","author":"Nair","year":"2012","journal-title":"Heart Rhythm"},{"key":"B84","doi-asserted-by":"publisher","first-page":"364","DOI":"10.1161\/CIRCRESAHA.107.164673","article-title":"Divergent biophysical defects caused by mutant sodium channels in dilated cardiomyopathy with arrhythmia","volume":"102","author":"Nguyen","year":"2008","journal-title":"Circ. Res"},{"key":"B85","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1038\/312121a0","article-title":"Primary structure of Electrophorus electricus sodium channel deduced from cDNA sequence","volume":"312","author":"Noda","year":"1984","journal-title":"Nature"},{"key":"B86","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1161\/CIRCGENETICS.111.962597","article-title":"High prevalence of long QT syndrome-associated SCN5A variants in patients with early-onset lone atrial fibrillation","volume":"5","author":"Olesen","year":"2012","journal-title":"Circ. Cardiovasc. Genet"},{"key":"B87","doi-asserted-by":"publisher","first-page":"447","DOI":"10.1001\/jama.293.4.447","article-title":"Sodium channel mutations and susceptibility to heart failure and atrial fibrillation","volume":"293","author":"Olson","year":"2005","journal-title":"JAMA"},{"key":"B88","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1113\/jphysiol.1994.sp020339","article-title":"The role of troponin C in modulating the Ca2+ sensitivity of mammalian skinned cardiac and skeletal muscle fibres","volume":"480(Pt 1)","author":"Palmer","year":"1994","journal-title":"J. Physiol"},{"key":"B89","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1023\/A:1018623604365","article-title":"The effect of pH on the Ca2+ affinity of the Ca2+ regulatory sites of skeletal and cardiac troponin C in skinned muscle fibres","volume":"18","author":"Parsons","year":"1997","journal-title":"J. Muscle Res. Cell Motil"},{"key":"B90","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1038\/nature11077","article-title":"Crystal structure of a voltage-gated sodium channel in two potentially inactivated states","volume":"486","author":"Payandeh","year":"2012","journal-title":"Nature"},{"key":"B91","doi-asserted-by":"publisher","first-page":"e1332","DOI":"10.1371\/journal.pone.0001332","article-title":"A tale of switched functions: from cyclooxygenase inhibition to M-channel modulation in new diphenylamine derivatives","volume":"2","author":"Peretz","year":"2007","journal-title":"PLoS ONE"},{"key":"B92","doi-asserted-by":"publisher","first-page":"15637","DOI":"10.1073\/pnas.0911294107","article-title":"Targeting the voltage sensor of Kv7.2 voltage-gated K+ channels with a new gating-modifier","volume":"107","author":"Peretz","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B93","doi-asserted-by":"publisher","first-page":"871","DOI":"10.1016\/S0008-6363(02)00849-0","article-title":"Sodium and the heart: a hidden key factor in cardiac regulation","volume":"57","author":"Pieske","year":"2003","journal-title":"Cardiovasc. Res"},{"key":"B94","doi-asserted-by":"publisher","first-page":"617","DOI":"10.1038\/nchembio.622","article-title":"Contributions of counter-charge in a potassium channel voltage-sensor domain","volume":"7","author":"Pless","year":"2011","journal-title":"Nat. Chem. Biol"},{"key":"B95","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1046\/j.1432-1327.1999.00625.x","article-title":"Scorpion toxins specific for Na+-channels","volume":"264","author":"Possani","year":"1999","journal-title":"Eur. J. Biochem"},{"key":"B96","doi-asserted-by":"publisher","first-page":"1213","DOI":"10.1038\/nature04700","article-title":"A voltage-gated proton-selective channel lacking the pore domain","volume":"440","author":"Ramsey","year":"2006","journal-title":"Nature"},{"key":"B97","doi-asserted-by":"publisher","first-page":"841","DOI":"10.1161\/01.CIR.93.5.841","article-title":"Report of the 1995 World Health Organization\/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies","volume":"93","author":"Richardson","year":"1996","journal-title":"Circulation"},{"key":"B98","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1002\/mus.880070205","article-title":"Hypokalemic periodic paralysis: in vitro investigation of muscle fiber membrane parameters","volume":"7","author":"Rudel","year":"1984","journal-title":"Muscle Nerve"},{"key":"B99","doi-asserted-by":"publisher","first-page":"589","DOI":"10.1126\/science.1122352","article-title":"A voltage sensor-domain protein is a voltage-gated proton channel","volume":"312","author":"Sasaki","year":"2006","journal-title":"Science"},{"key":"B100","doi-asserted-by":"publisher","first-page":"10100","DOI":"10.1523\/JNEUROSCI.2476-06.2006","article-title":"Effects in neocortical neurons of mutations of the Na(v)1.2 Na+ channel causing benign familial neonatal-infantile seizures","volume":"26","author":"Scalmani","year":"2006","journal-title":"J. Neurosci"},{"key":"B101","doi-asserted-by":"publisher","first-page":"687","DOI":"10.1038\/25367","article-title":"Moderate loss of function of cyclic-AMP-modulated KCNQ2\/KCNQ3 K+ channels causes epilepsy","volume":"396","author":"Schroeder","year":"1998","journal-title":"Nature"},{"key":"B102","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1038\/msb.2011.75","article-title":"Fast, scalable generation of high-quality protein multiple sequence alignments using Clustal Omega","volume":"7","author":"Sievers","year":"2011","journal-title":"Mol. Syst. Biol"},{"key":"B103","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1113\/jphysiol.1980.sp013426","article-title":"The variance of sodium current fluctuations at the node of Ranvier","volume":"307","author":"Sigworth","year":"1980","journal-title":"J. Physiol"},{"key":"B104","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1038\/nature05598","article-title":"Gating pore current in an inherited ion channelopathy","volume":"446","author":"Sokolov","year":"2007","journal-title":"Nature"},{"key":"B105","doi-asserted-by":"publisher","first-page":"19980","DOI":"10.1073\/pnas.0810562105","article-title":"Depolarization-activated gating pore current conducted by mutant sodium channels in potassium-sensitive normokalemic periodic paralysis","volume":"105","author":"Sokolov","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A"},{"key":"B106","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1085\/jgp.201010414","article-title":"Ion permeation and block of the gating pore in the voltage sensor of NaV1.4 channels with hypokalemic periodic paralysis mutations","volume":"136","author":"Sokolov","year":"2010","journal-title":"J. Gen. Physiol"},{"key":"B107","doi-asserted-by":"publisher","first-page":"469","DOI":"10.1085\/jgp.117.5.469","article-title":"Histidine scanning mutagenesis of basic residues of the S4 segment of the shaker k+ channel","volume":"117","author":"Starace","year":"2001","journal-title":"J. Gen. Physiol"},{"key":"B108","doi-asserted-by":"publisher","first-page":"548","DOI":"10.1038\/nature02270","article-title":"A proton pore in a potassium channel voltage sensor reveals a focused electric field","volume":"427","author":"Starace","year":"2004","journal-title":"Nature"},{"key":"B109","doi-asserted-by":"publisher","first-page":"1091","DOI":"10.1093\/brain\/124.6.1091","article-title":"Hypokalaemic periodic paralysis type 2 caused by mutations at codon 672 in the muscle sodium channel gene SCN4A","volume":"124","author":"Sternberg","year":"2001","journal-title":"Brain"},{"issue":"71","key":"B110","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2011.00071","article-title":"Neurotoxins and their binding areas on voltage-gated sodium channels","volume":"2","author":"Stevens","year":"2011","journal-title":"Front. Pharmacol"},{"key":"B111","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1007\/s00424-010-0800-x","article-title":"Channelopathies in Cav1.1, Cav1.3, and Cav1.4 voltage-gated L-type Ca2+ channels","volume":"460","author":"Striessnig","year":"2010","journal-title":"Pflugers Arch"},{"key":"B112","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1085\/jgp.200709755","article-title":"A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating pore","volume":"130","author":"Struyk","year":"2007","journal-title":"J. Gen. Physiol"},{"key":"B113","doi-asserted-by":"publisher","first-page":"447","DOI":"10.1085\/jgp.200809967","article-title":"Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis","volume":"132","author":"Struyk","year":"2008","journal-title":"J. Gen. Physiol"},{"key":"B114","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1097\/MAJ.0b013e31822b430c","article-title":"Genotype and phenotype analysis of patients with sporadic periodic paralysis","volume":"343","author":"Sung","year":"2012","journal-title":"Am. J. Med. Sci"},{"key":"B115","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1126\/science.1185954","article-title":"A gating charge transfer center in voltage sensors","volume":"328","author":"Tao","year":"2010","journal-title":"Science"},{"key":"B116","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1186\/1750-1172-1-27","article-title":"Cardiomyopathy, familial dilated","volume":"1","author":"Taylor","year":"2006","journal-title":"Orphanet. J. Rare Dis"},{"key":"B117","doi-asserted-by":"publisher","first-page":"546","DOI":"10.1038\/nature05396","article-title":"The twisted ion-permeation pathway of a resting voltage-sensing domain","volume":"445","author":"Tombola","year":"2007","journal-title":"Nature"},{"key":"B118","doi-asserted-by":"publisher","first-page":"379","DOI":"10.1016\/j.neuron.2004.12.047","article-title":"Voltage-sensing arginines in a potassium channel permeate and occlude cation-selective pores","volume":"45","author":"Tombola","year":"2005","journal-title":"Neuron"},{"issue":"8","key":"B119","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2011.00008","article-title":"Recent advances in the pathogenesis and drug action in periodic paralyses and related channelopathies","volume":"2","author":"Tricarico","year":"2011","journal-title":"Front. Pharmacol"},{"key":"B120","doi-asserted-by":"publisher","first-page":"11591","DOI":"10.1021\/ja0631955","article-title":"Structural and functional consequences of an amide-to-ester substitution in the selectivity filter of a potassium channel","volume":"128","author":"Valiyaveetil","year":"2006","journal-title":"J. Am. Chem. Soc"},{"key":"B121","doi-asserted-by":"publisher","first-page":"2120","DOI":"10.1212\/01.WNL.0000145768.09934.EC","article-title":"New mutations of SCN4A cause a potassium-sensitive normokalemic periodic paralysis","volume":"63","author":"Vicart","year":"2004","journal-title":"Neurology"},{"key":"B122","doi-asserted-by":"publisher","first-page":"368","DOI":"10.1161\/CIRCULATIONAHA.106.646513","article-title":"Cardiac sodium channel dysfunction in sudden infant death syndrome","volume":"115","author":"Wang","year":"2007","journal-title":"Circulation"},{"key":"B123","doi-asserted-by":"publisher","first-page":"874","DOI":"10.1161\/CIRCEP.111.963983","article-title":"Electrocardiographic characteristics and SCN5A mutations in idiopathic ventricular fibrillation associated with early repolarization","volume":"4","author":"Watanabe","year":"2011","journal-title":"Circ. Arrhythm. Electrophysiol"},{"key":"B124","doi-asserted-by":"publisher","first-page":"4082","DOI":"10.1172\/JCI57398","article-title":"A sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis","volume":"121","author":"Wu","year":"2011","journal-title":"J. Clin. Invest"},{"key":"B125","doi-asserted-by":"publisher","first-page":"4580","DOI":"10.1172\/JCI66091","article-title":"A calcium channel mutant mouse model of hypokalemic periodic paralysis","volume":"122","author":"Wu","year":"2012","journal-title":"J. Clin. Invest"},{"key":"B126","doi-asserted-by":"publisher","first-page":"2045","DOI":"10.1212\/01.wnl.0000275523.95103.36","article-title":"Peripheral nerve hyperexcitability due to dominant-negative KCNQ2 mutations","volume":"69","author":"Wuttke","year":"2007","journal-title":"Neurology"},{"key":"B127","doi-asserted-by":"publisher","first-page":"27300","DOI":"10.1074\/jbc.M708447200","article-title":"Tarantula huwentoxin-IV inhibits neuronal sodium channels by binding to receptor site 4 and trapping the domain ii voltage sensor in the closed configuration","volume":"283","author":"Xiao","year":"2008","journal-title":"J. Biol. Chem"},{"key":"B128","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1016\/S0896-6273(00)80028-8","article-title":"Molecular basis of charge movement in voltage-gated sodium channels","volume":"16","author":"Yang","year":"1996","journal-title":"Neuron"},{"key":"B129","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1016\/0896-6273(95)90078-0","article-title":"Evidence for voltage-dependent S4 movement in sodium channels","volume":"15","author":"Yang","year":"1995","journal-title":"Neuron"},{"key":"B130","doi-asserted-by":"publisher","DOI":"10.1126\/stke.2532004re15","article-title":"The VGL-chanome: a protein superfamily specialized for electrical signaling and ionic homeostasis","author":"Yu","year":"2004","journal-title":"Sci. STKE"},{"key":"B131","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1124\/pr.57.4.13","article-title":"Overview of molecular relationships in the voltage-gated ion channel superfamily","volume":"57","author":"Yu","year":"2005","journal-title":"Pharmacol. Rev"},{"key":"B132","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1038\/nature11054","article-title":"Crystal structure of an orthologue of the NaChBac voltage-gated sodium channel","volume":"486","author":"Zhang","year":"2012","journal-title":"Nature"},{"key":"B133","doi-asserted-by":"publisher","first-page":"957","DOI":"10.1016\/j.ajhg.2013.09.016","article-title":"Gain-of-function mutations in SCN11A cause familial episodic pain","volume":"93","author":"Zhang","year":"2013","journal-title":"Am. J. Hum. Genet"}],"container-title":["Frontiers in Pharmacology"],"original-title":[],"deposited":{"date-parts":[[2022,3,31]],"date-time":"2022-03-31T18:04:19Z","timestamp":1648749859000},"score":1,"resource":{"primary":{"URL":"http:\/\/journal.frontiersin.org\/article\/10.3389\/fphar.2014.00053\/abstract"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,4,3]]},"references-count":133,"URL":"https:\/\/doi.org\/10.3389\/fphar.2014.00053","relation":{},"ISSN":["1663-9812"],"issn-type":[{"value":"1663-9812","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,4,3]]}}}