A calcium channel mutant mouse model of hypokalemic periodic paralysis
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ChannelopathiesThe Physiology, Pathology, and Pharmacology of Voltage-Gated Calcium Channels and Their Future Therapeutic PotentialOmega pore, an alternative ion channel permeation pathway involved in the development of several channelopathiesAlternating bipolar field stimulation identifies muscle fibers with defective excitability but maintained local Ca(2+) signals and contraction.Channelopathies of skeletal muscle excitabilityNovel mutations in human and mouse SCN4A implicate AMPK in myotonia and periodic paralysis.Disrupted coupling of gating charge displacement to Na+ current activation for DIIS4 mutations in hypokalemic periodic paralysisMuscle channelopathies: the nondystrophic myotonias and periodic paralyses.Gating pore currents are defects in common with two Nav1.5 mutations in patients with mixed arrhythmias and dilated cardiomyopathy.Rem uncouples excitation-contraction coupling in adult skeletal muscle fibers.Leaky channels make weak muscles.A 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.Biophysics, pathophysiology, and pharmacology of ion channel gating pores.Mutational consequences of aberrant ion channels in neurological disorders.Phosphoinositides in Ca(2+) signaling and excitation-contraction coupling in skeletal muscle: an old player and newcomers.Elevated resting H(+) current in the R1239H type 1 Hypokalemic Periodic Paralysis mutated Ca(2+) channel.Impaired calcium signaling in muscle fibers from intercostal and foot skeletal muscle in a cigarette smoke-induced mouse model of COPD.Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy.Phospholemman, a major regulator of skeletal muscle Na+/K+-ATPase, is not mutated in probands with hypokalemic periodic paralysis.The large-conductance calcium-activated potassium channel holds the key to the conundrum of familial hypokalemic periodic paralysis.An atypical CaV1.1 mutation reveals a common mechanism for hypokalemic periodic paralysis.Mechanisms Responsible for ω-Pore Currents in Cav Calcium Channel Voltage-Sensing Domains.Na leak with gating pore properties in hypokalemic periodic paralysis V876E mutant muscle Ca channel.Stac3 enhances expression of human CaV1.1 in Xenopus oocytes and reveals gating pore currents in HypoPP mutant channels.Sodium Channelopathies of Skeletal Muscle.When muscle Ca2+ channels carry monovalent cations through gating pores: insights into the pathophysiology of type 1 hypokalaemic periodic paralysis.Mutations in the voltage-sensing domain affect the alternative ion permeation pathway in the TRPM3 channel.Genetic Evidence Supporting the Role of the Calcium Channel, CACNA1S, in Tooth Cusp and Root PatterningA leaky voltage sensor domain of cardiac sodium channels causes arrhythmias associated with dilated cardiomyopathy
P2860
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P2860
A calcium channel mutant mouse model of hypokalemic periodic paralysis
description
2012 nî lūn-bûn
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2012年の論文
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2012年学术文章
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name
A calcium channel mutant mouse model of hypokalemic periodic paralysis
@ast
A calcium channel mutant mouse model of hypokalemic periodic paralysis
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type
label
A calcium channel mutant mouse model of hypokalemic periodic paralysis
@ast
A calcium channel mutant mouse model of hypokalemic periodic paralysis
@en
prefLabel
A calcium channel mutant mouse model of hypokalemic periodic paralysis
@ast
A calcium channel mutant mouse model of hypokalemic periodic paralysis
@en
P2093
P2860
P356
P1476
A calcium channel mutant mouse model of hypokalemic periodic paralysis
@en
P2093
Arie F Struyk
Dennis K Burns
Erick O Hernández-Ochoa
Hillery F Gray
Martin F Schneider
Stephen C Cannon
P2860
P304
P356
10.1172/JCI66091
P407
P577
2012-11-26T00:00:00Z