Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis
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KCNC3: phenotype, mutations, channel biophysics-a study of 260 familial ataxia patientsFunctionality of the voltage-gated proton channel truncated in S4Physiological and Pathophysiological Insights of Nav1.4 and Nav1.5 ComparisonThe Physiology, Pathology, and Pharmacology of Voltage-Gated Calcium Channels and Their Future Therapeutic PotentialCa(V)1.1: The atypical prototypical voltage-gated Ca²⁺ channelChannelopathies of skeletal muscle excitabilityDisrupted coupling of gating charge displacement to Na+ current activation for DIIS4 mutations in hypokalemic periodic paralysisIon permeation and block of the gating pore in the voltage sensor of NaV1.4 channels with hypokalemic periodic paralysis mutationsLeaky sodium channels from voltage sensor mutations in periodic paralysis, but not paramyotoniaA sodium channel knockin mutant (NaV1.4-R669H) mouse model of hypokalemic periodic paralysis.The pore of the voltage-gated proton channelPathophysiological role of omega pore current in channelopathiesLeaky channels make weak muscles.A calcium channel mutant mouse model of hypokalemic periodic paralysisA novel NaV1.5 voltage sensor mutation associated with severe atrial and ventricular arrhythmias.Mice with an NaV1.4 sodium channel null allele have latent myasthenia, without susceptibility to periodic paralysis.Beneficial effects of bumetanide in a CaV1.1-R528H mouse model of hypokalaemic periodic paralysis.Transient compartment-like syndrome and normokalaemic periodic paralysis due to a Ca(v)1.1 mutation.Skeletal muscle channelopathies: new insights into the periodic paralyses and nondystrophic myotonias.Architecture and gating of Hv1 proton channels.NaV1.4 mutations cause hypokalaemic periodic paralysis by disrupting IIIS4 movement during recovery.Muscle channelopathies: does the predicted channel gating pore offer new treatment insights for hypokalaemic periodic paralysis?Voltage-sensor mutations in channelopathies of skeletal muscle.Biophysics, pathophysiology, and pharmacology of ion channel gating pores.Cardiac arrhythmias in hypokalemic periodic paralysis: Hypokalemia as only cause?Molecular biology and biophysical properties of ion channel gating pores.Skeletal muscle na channel disorders.Domain III S4 in closed-state fast inactivation: insights from a periodic paralysis mutationPhospholemman, a major regulator of skeletal muscle Na+/K+-ATPase, is not mutated in probands with hypokalemic periodic paralysis.Gender differences in penetrance and phenotype in hypokalemic periodic paralysis.Atomistic Modeling of Ion Conduction through the Voltage-Sensing Domain of the Shaker K+ Ion Channel.An atypical CaV1.1 mutation reveals a common mechanism for hypokalemic periodic paralysis.Fluorescent Visualization of Cellular Proton Fluxes.N1366S mutation of human skeletal muscle sodium channel causes paramyotonia congenita.Stac3 enhances expression of human CaV1.1 in Xenopus oocytes and reveals gating pore currents in HypoPP mutant channels.Sodium Channelopathies of Skeletal Muscle.Spider toxin inhibits gating pore currents underlying periodic paralysis.Hypokalaemic periodic paralysis and myotonia in a patient with homozygous mutation p.R1451L in NaV1.4.A New Cardiac Channelopathy: From Clinical Phenotypes to Molecular Mechanisms Associated With Na1.5 Gating PoresGating Pore Currents in Sodium Channels
P2860
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P2860
Gating pore currents in DIIS4 mutations of NaV1.4 associated with periodic paralysis: saturation of ion flux and implications for disease pathogenesis
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Gating pore currents in DIIS4 ...... tions for disease pathogenesis
@en
type
label
Gating pore currents in DIIS4 ...... tions for disease pathogenesis
@en
prefLabel
Gating pore currents in DIIS4 ...... tions for disease pathogenesis
@en
P2093
P2860
P356
P1476
Gating pore currents in DIIS4 ...... tions for disease pathogenesis
@en
P2093
Arie F Struyk
David Francis
Stephen C Cannon
Vladislav S Markin
P2860
P304
P356
10.1085/JGP.200809967
P577
2008-10-01T00:00:00Z