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钠离子通道失控:神经元和肌肉通道病的再生电流。

Sodium channels gone wild: resurgent current from neuronal and muscle channelopathies.

机构信息

Department of Neurology, University of Texas Southwestern Medical Center, 5223 Harry Hines Blvd., Dallas, Texas 75390-8813, USA.

出版信息

J Clin Invest. 2010 Jan;120(1):80-3. doi: 10.1172/JCI41340. Epub 2009 Dec 28.

Abstract

Voltage-dependent sodium channels are the central players in the excitability of neurons, cardiac muscle, and skeletal muscle. Hundreds of mutations in sodium channels have been associated with human disease, particularly genetic forms of epilepsy, arrhythmias, myotonia, and periodic paralysis. In this issue of the JCI, Jarecki and colleagues present evidence suggesting that many such mutations alter the gating of sodium channels to produce resurgent sodium current, an unusual form of gating in which sodium channels reopen following an action potential, thus promoting the firing of another action potential (see the related article beginning on page 369). The results of this study suggest a widespread pathophysiological role for this mechanism, previously described to occur normally in only a few types of neurons.

摘要

电压门控钠离子通道是神经元、心肌和平滑肌兴奋性的核心。数百种钠离子通道突变与人类疾病有关,特别是遗传性癫痫、心律失常、肌强直和周期性瘫痪。在本期 JCI 中,Jarecki 及其同事提供的证据表明,许多此类突变改变了钠离子通道的门控,产生了钠电流的折返,这是一种异常的门控形式,即在动作电位后钠离子通道重新开放,从而促进了另一个动作电位的发放(见第 369 页开始的相关文章)。这项研究的结果表明,这种机制在以前仅在少数几种神经元中正常发生的情况下,具有广泛的病理生理学作用。

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