| scholarly article | Q13442814 |
| P2093 | author name string | Stephen C Cannon | |
| Arie F Struyk | |||
| P2860 | cites work | The production, buffering and efflux of protons in human skeletal muscle during exercise and recovery | Q70621778 |
| Expression and functional characterization of the cardiac L-type calcium channel carrying a skeletal muscle DHP-receptor mutation causing hypokalaemic periodic paralysis | Q70958741 | ||
| Gating defects of a novel Na+ channel mutant causing hypokalemic periodic paralysis | Q80062919 | ||
| Gating of the L-type Ca channel in human skeletal myotubes: an activation defect caused by the hypokalemic periodic paralysis mutation R528H | Q22008539 | ||
| Charge movement associated with the opening and closing of the activation gates of the Na channels | Q24649965 | ||
| A calcium channel mutation causing hypokalemic periodic paralysis | Q28242375 | ||
| Dihydropyridine receptor mutations cause hypokalemic periodic paralysis | Q28243593 | ||
| Pathomechanisms in channelopathies of skeletal muscle and brain | Q28246271 | ||
| Cable parameters, sodium, potassium, chloride, and water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita | Q34064967 | ||
| Experimental generation and computational modeling of intracellular pH gradients in cardiac myocytes | Q34189895 | ||
| The resting membrane parameters of human intercostal muscle at low, normal, and high extracellular potassium | Q34266808 | ||
| Structure and function of voltage-gated ion channels | Q34296742 | ||
| The cloning and expression of a sodium channel beta 1-subunit cDNA from human brain | Q34356775 | ||
| Voltage-sensor sodium channel mutations cause hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced current | Q35208537 | ||
| INTRACELLULAR ACID-BASE REGULATION. I. THE RESPONSE OF MUSCLE CELLS TO CHANGES IN CO2 TENSION OR EXTRACELLULAR BICARBONATE CONCENTRATION. | Q35570752 | ||
| Histidine scanning mutagenesis of basic residues of the S4 segment of the shaker k+ channel | Q36444662 | ||
| Enhanced inactivation and pH sensitivity of Na(+) channel mutations causing hypokalaemic periodic paralysis type II. | Q40742646 | ||
| The human skeletal muscle Na channel mutation R669H associated with hypokalemic periodic paralysis enhances slow inactivation. | Q40907023 | ||
| Electrophysiological properties of the hypokalaemic periodic paralysis mutation (R528H) of the skeletal muscle alpha 1s subunit as expressed in mouse L cells. | Q41215041 | ||
| Voltage sensors in domains III and IV, but not I and II, are immobilized by Na+ channel fast inactivation | Q41608443 | ||
| Primary structure and functional expression of a mammalian skeletal muscle sodium channel | Q42189395 | ||
| Subunit stoichiometry of a mammalian K+ channel determined by construction of multimeric cDNAs. | Q42604432 | ||
| Hypokalaemic periodic paralysis type 2 caused by mutations at codon 672 in the muscle sodium channel gene SCN4A. | Q43608348 | ||
| A proton pore in a potassium channel voltage sensor reveals a focused electric field | Q44757373 | ||
| Voltage-sensing arginines in a potassium channel permeate and occlude cation-selective pores | Q45252796 | ||
| Voltage-dependent proton transport by the voltage sensor of the Shaker K+ channel. | Q46015095 | ||
| Ion permeation through a voltage- sensitive gating pore in brain sodium channels having voltage sensor mutations | Q46615032 | ||
| Gating pore current in an inherited ion channelopathy | Q48799799 | ||
| Saxitoxin binding to sodium channels of rat skeletal muscles. | Q51877540 | ||
| A novel sodium channel mutation in a family with hypokalemic periodic paralysis | Q55670198 | ||
| P433 | issue | 1 | |
| P921 | main subject | hypokalemic periodic paralysis | Q622828 |
| P304 | page(s) | 11-20 | |
| P577 | publication date | 2007-07-01 | |
| P1433 | published in | The Journal of General Physiology | Q1092259 |
| P1476 | title | A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating pore | |
| P478 | volume | 130 |
| Q57816587 | A New Cardiac Channelopathy: From Clinical Phenotypes to Molecular Mechanisms Associated With Na1.5 Gating Pores |
| Q36498120 | A calcium channel mutant mouse model of hypokalemic periodic paralysis |
| Q58740889 | A leaky voltage sensor domain of cardiac sodium channels causes arrhythmias associated with dilated cardiomyopathy |
| Q59809830 | A novel ATP1A2 mutation in a patient with hypokalaemic periodic paralysis and CNS symptoms |
| Q36680153 | A novel NaV1.5 voltage sensor mutation associated with severe atrial and ventricular arrhythmias |
| Q34295483 | A proton leak current through the cardiac sodium channel is linked to mixed arrhythmia and the dilated cardiomyopathy phenotype |
| Q35370222 | A sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis |
| Q35606217 | Acetazolamide efficacy in hypokalemic periodic paralysis and the predictive role of genotype |
| Q48656390 | Altered Kv3.3 channel gating in early-onset spinocerebellar ataxia type 13. |
| Q46154188 | An atypical CaV1.1 mutation reveals a common mechanism for hypokalemic periodic paralysis. |
| Q46026379 | Atomistic Modeling of Ion Conduction through the Voltage-Sensing Domain of the Shaker K+ Ion Channel. |
| Q37383463 | Beneficial effects of bumetanide in a CaV1.1-R528H mouse model of hypokalaemic periodic paralysis |
| Q38207583 | Biophysics, pathophysiology, and pharmacology of ion channel gating pores |
| Q36870057 | Bumetanide prevents transient decreases in muscle force in murine hypokalemic periodic paralysis. |
| Q30379553 | COG lobe B sub-complex engages v-SNARE GS15 and functions via regulated interaction with lobe A sub-complex. |
| Q26866428 | Ca(V)1.1: The atypical prototypical voltage-gated Ca²⁺ channel |
| Q38236485 | Cardiac arrhythmias in hypokalemic periodic paralysis: Hypokalemia as only cause? |
| Q90289933 | Cell-Free Expression of Sodium Channel Domains for Pharmacology Studies. Noncanonical Spider Toxin Binding Site in the Second Voltage-Sensing Domain of Human Nav1.4 Channel |
| Q28082428 | Channelopathies of skeletal muscle excitability |
| Q37401733 | Conduits of life's spark: a perspective on ion channel research since the birth of neuron |
| Q89994678 | Conservation of the Ca2+-permeability through the voltage sensor domain of mammalian CatSper subunit |
| Q36715293 | Construction and validation of a homology model of the human voltage-gated proton channel hHV1 |
| Q37018940 | Depolarization-activated gating pore current conducted by mutant sodium channels in potassium-sensitive normokalemic periodic paralysis |
| Q33807683 | Diagnostics and therapy of muscle channelopathies--Guidelines of the Ulm Muscle Centre. |
| Q33964031 | Disrupted coupling of gating charge displacement to Na+ current activation for DIIS4 mutations in hypokalemic periodic paralysis |
| Q42868384 | Do hyperpolarization-induced proton currents contribute to the pathogenesis of hypokalemic periodic paralysis, a voltage sensor channelopathy? |
| Q42394799 | Effect of sensor domain mutations on the properties of voltage-gated ion channels: molecular dynamics studies of the potassium channel Kv1.2. |
| Q38600648 | Elevated resting H(+) current in the R1239H type 1 Hypokalemic Periodic Paralysis mutated Ca(2+) channel. |
| Q88922983 | Evaluation of mutant muscle Ca2+ channel properties using two different expression systems |
| Q58327238 | Gating Pore Currents in Sodium Channels |
| Q35843838 | Gating currents from Kv7 channels carrying neuronal hyperexcitability mutations in the voltage-sensing domain. |
| Q36436974 | Gating pore currents and the resting state of Nav1.4 voltage sensor domains |
| Q35014373 | Gating pore currents are defects in common with two Nav1.5 mutations in patients with mixed arrhythmias and dilated cardiomyopathy |
| Q36908941 | Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis |
| Q41277802 | Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy. |
| Q35787126 | Gating transitions in the selectivity filter region of a sodium channel are coupled to the domain IV voltage sensor |
| Q34024636 | Gating-pore currents demonstrate selective and specific modulation of individual sodium channel voltage-sensors by biological toxins |
| Q45366706 | Gender differences in penetrance and phenotype in hypokalemic periodic paralysis |
| Q26777559 | Genetic neurological channelopathies: molecular genetics and clinical phenotypes |
| Q37786254 | Hereditary Channelopathies in Neurology |
| Q92964252 | Hydrophobic gasket mutation produces gating pore currents in closed human voltage-gated proton channels |
| Q55512224 | Hypokalaemic periodic paralysis and myotonia in a patient with homozygous mutation p.R1451L in NaV1.4. |
| Q35144896 | Identification and functional characterization of Kir2.6 mutations associated with non-familial hypokalemic periodic paralysis. |
| Q87841107 | Improving the characterization of calcium channel gating pore currents with Stac3 |
| Q34139997 | Ion Channel Voltage Sensors: Structure, Function, and Pathophysiology |
| Q34028248 | Ion permeation and block of the gating pore in the voltage sensor of NaV1.4 channels with hypokalemic periodic paralysis mutations |
| Q37102151 | K+-dependent paradoxical membrane depolarization and Na+ overload, major and reversible contributors to weakness by ion channel leaks |
| Q36439881 | Leaky channels make weak muscles |
| Q34999202 | Leaky sodium channels from voltage sensor mutations in periodic paralysis, but not paramyotonia |
| Q47131974 | Mechanisms Responsible for ω-Pore Currents in Cav Calcium Channel Voltage-Sensing Domains. |
| Q36967712 | Mice with an NaV1.4 sodium channel null allele have latent myasthenia, without susceptibility to periodic paralysis |
| Q38266367 | Molecular biology and biophysical properties of ion channel gating pores. |
| Q35990363 | Molecular dynamics simulations of voltage-gated cation channels: insights on voltage-sensor domain function and modulation |
| Q26800090 | Molecular pathophysiology and pharmacology of the voltage-sensing module of neuronal ion channels |
| Q37685726 | Muscle channelopathies: does the predicted channel gating pore offer new treatment insights for hypokalaemic periodic paralysis? |
| Q43110938 | Mutations in the Voltage Sensors of Domains I and II of Nav1.5 that are Associated with Arrhythmias and Dilated Cardiomyopathy Generate Gating Pore Currents. |
| Q55401194 | Mutations in the voltage-sensing domain affect the alternative ion permeation pathway in the TRPM3 channel. |
| Q47644010 | N1366S mutation of human skeletal muscle sodium channel causes paramyotonia congenita. |
| Q47637076 | Na leak with gating pore properties in hypokalemic periodic paralysis V876E mutant muscle Ca channel. |
| Q89508197 | NaV1.4 DI-S4 periodic paralysis mutation R222W enhances inactivation and promotes leak current to attenuate action potentials and depolarize muscle fibers |
| Q37647775 | NaV1.4 mutations cause hypokalaemic periodic paralysis by disrupting IIIS4 movement during recovery |
| Q40402516 | Nav 1.5 mutations linked to dilated cardiomyopathy phenotypes: Is the gating pore current the missing link? |
| Q26784147 | Omega pore, an alternative ion channel permeation pathway involved in the development of several channelopathies |
| Q36022793 | Pathophysiological role of omega pore current in channelopathies |
| Q28660353 | Philosophy of voltage-gated proton channels |
| Q41623786 | Phospholemman, a major regulator of skeletal muscle Na+/K+-ATPase, is not mutated in probands with hypokalemic periodic paralysis |
| Q30392343 | Proton currents constrain structural models of voltage sensor activation |
| Q35025840 | Recent advances in the pathogenesis and drug action in periodic paralyses and related channelopathies |
| Q90316166 | Resurgent and Gating Pore Currents Induced by De Novo SCN2A Epilepsy Mutations |
| Q90722875 | Skeletal muscle CaV1.1 channelopathies |
| Q37390029 | Skeletal muscle channelopathies: new insights into the periodic paralyses and nondystrophic myotonias |
| Q38698910 | Skeletal muscle na channel disorders |
| Q49908047 | Sodium Channelopathies of Skeletal Muscle. |
| Q41661778 | Sodium channel NaV1.9 mutations associated with insensitivity to pain dampen neuronal excitability |
| Q37711046 | Sodium channelopathies of skeletal muscle result from gain or loss of function |
| Q52325902 | Spider toxin inhibits gating pore currents underlying periodic paralysis. |
| Q48131423 | Stac3 enhances expression of human CaV1.1 in Xenopus oocytes and reveals gating pore currents in HypoPP mutant channels. |
| Q42610360 | State of the art in hereditary muscle channelopathies |
| Q64039626 | Strength and muscle structure preserved during long-term therapy in a patient with hypokalemic periodic paralysis (Cav1.1-R1239G) |
| Q59065909 | Structural basis for gating pore current in periodic paralysis |
| Q26744730 | Therapeutic Approaches to Genetic Ion Channelopathies and Perspectives in Drug Discovery |
| Q33964075 | Tracking S4 movement by gating pore currents in the bacterial sodium channel NaChBac |
| Q37383483 | Transient compartment-like syndrome and normokalaemic periodic paralysis due to a Ca(v)1.1 mutation |
| Q55236601 | Voltage and pH sensing by the voltage-gated proton channel, HV1. |
| Q33763782 | Voltage sensor charge loss accounts for most cases of hypokalemic periodic paralysis |
| Q38571185 | Voltage-gated proton (H(v)1) channels, a singular voltage sensing domain. |
| Q34050134 | Voltage-gated proton channel in a dinoflagellate |
| Q28681630 | Voltage-gated proton channels: molecular biology, physiology, and pathophysiology of the H(V) family |
| Q24657828 | Voltage-gated proton channels: what's next? |
| Q34265707 | Voltage-gated sodium channels at 60: structure, function and pathophysiology |
| Q24629456 | Voltage-sensing domain of voltage-gated proton channel Hv1 shares mechanism of block with pore domains |
| Q37692699 | Voltage-sensor mutations in channelopathies of skeletal muscle |
| Q24618339 | Water wires in atomistic models of the Hv1 proton channel |
| Q37188305 | When all is lost…a severe myopathy with hypotonia from sodium channel mutations |
| Q52343820 | When muscle Ca2+ channels carry monovalent cations through gating pores: insights into the pathophysiology of type 1 hypokalaemic periodic paralysis. |
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