Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis

scientific article published on October 2008

Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1085/JGP.200809967
P932PMC publication ID2553391
P698PubMed publication ID18824591
P5875ResearchGate publication ID23287978

P2093author name stringStephen C Cannon
David Francis
Vladislav S Markin
Arie F Struyk
P2860cites workA proton pore in a potassium channel voltage sensor reveals a focused electric fieldQ44757373
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Voltage sensor of Kv1.2: structural basis of electromechanical couplingQ46588794
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Calibrated measurement of gating-charge arginine displacement in the KvAP voltage-dependent K+ channelQ46790301
Gating pore current in an inherited ion channelopathyQ48799799
Effects of mutations causing hypokalaemic periodic paralysis on the skeletal muscle L-type Ca2+ channel expressed in Xenopus laevis oocytesQ48907500
Sodium influx during action potential in innervated and denervated rat skeletal muscles.Q51353268
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Osmolality influences bistability of membrane potential under hypokalemic conditions in mouse skeletal muscle: an experimental and theoretical study.Q52043914
Induction and removal of inward-going rectification in sheep cardiac Purkinje fibresQ52220938
Paradoxical depolarization of BA2+- treated muscle exposed to low extracellular K+: insights into resting potential abnormalities in hypokalemic paralysis.Q53556880
A novel sodium channel mutation in a family with hypokalemic periodic paralysisQ55670198
Expression and functional characterization of the cardiac L-type calcium channel carrying a skeletal muscle DHP-receptor mutation causing hypokalaemic periodic paralysisQ70958741
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Insulin acts in hypokalemic periodic paralysis by reducing inward rectifier K+ currentQ73114370
The effect of internal and external potassium concentration on the membrane potential of frog muscleQ74258618
CLINICAL AND ELECTROMYOGRAPHIC STUDIES IN A PATIENT WITH PRIMARY HYPOKALEMIC PERIODIC PARALYSISQ78278026
The influence of potassium and chloride ions on the membrane potential of single muscle fibresQ78767148
Gating of the L-type Ca channel in human skeletal myotubes: an activation defect caused by the hypokalemic periodic paralysis mutation R528HQ22008539
Charge movement associated with the opening and closing of the activation gates of the Na channelsQ24649965
A calcium channel mutation causing hypokalemic periodic paralysisQ28242375
Dihydropyridine receptor mutations cause hypokalemic periodic paralysisQ28243593
Pathomechanisms in channelopathies of skeletal muscle and brainQ28246271
Hypokalemic periodic paralysis: In vitro investigation of muscle fiber membrane parametersQ28265960
Interface connections of a transmembrane voltage sensorQ30476262
Muscle Na+ channelopathies: MRI detects intracellular 23Na accumulation during episodic weaknessQ31056809
The cloning and expression of a sodium channel beta 1-subunit cDNA from human brainQ34356775
A voltage-sensor water poreQ35129025
Voltage-sensor sodium channel mutations cause hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced currentQ35208537
A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating poreQ36299707
Independent versus coupled inactivation in sodium channels. Role of the domain 2 S4 segmentQ36411959
Specificity of charge-carrying residues in the voltage sensor of potassium channelsQ36412550
Transfer of twelve charges is needed to open skeletal muscle Na+ channelsQ36435658
Histidine scanning mutagenesis of basic residues of the S4 segment of the shaker k+ channelQ36444662
Label-free biosensing with functionalized nanopipette probesQ37118884
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
Molecular basis of charge movement in voltage-gated sodium channelsQ41254973
Primary structure and functional expression of a mammalian skeletal muscle sodium channelQ42189395
The principle of gating charge movement in a voltage-dependent K+ channelQ42598185
Subunit stoichiometry of a mammalian K+ channel determined by construction of multimeric cDNAs.Q42604432
Closing in on the resting state of the Shaker K(+) channel.Q42633636
Do hyperpolarization-induced proton currents contribute to the pathogenesis of hypokalemic periodic paralysis, a voltage sensor channelopathy?Q42868384
Hypokalaemic periodic paralysis type 2 caused by mutations at codon 672 in the muscle sodium channel gene SCN4A.Q43608348
Effects of chloride transport on bistable behaviour of the membrane potential in mouse skeletal muscleQ44049308
Role of the anomalous rectifier in determining membrane potentials of mouse muscle fibres at low extracellular K+.Q44666322
In skeletal muscle the relaxation of the resting membrane potential induced by K(+) permeability changes depends on Cl(-) transportQ44675448
P433issue4
P921main subjectperiodic paralysisQ1788314
P304page(s)447-464
P577publication date2008-10-01
P1433published inThe Journal of General PhysiologyQ1092259
P1476titleGating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis
P478volume132

Reverse relations

cites work (P2860)
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