Article
A Na+ channel mutation linked to hypokalemic periodic paralysis exposes a proton-selective gating pore.
The Journal of general physiology - 1 Jul 2007
Struyk Arie F, Cannon Stephen C
Abstract excerpt
The heritable muscle disorder hypokalemic periodic paralysis (HypoPP) is characterized by attacks of flaccid weakness, brought on by sustained sarcolemmal depolarization. HypoPP is genetically linked to missense mutations at charged residues in the S4 voltage-sensing segments of either CaV1.1 (the skeletal muscle L-type Ca(2+) channel) or NaV1.4 (the skeletal muscle voltage-gated Na(+) channel). Although these...
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