Article
Gating of myotonic Na channel mutants defines the response to mexiletine and a potent derivative.
Neurology - 27 Nov 2001
Desaphy J F, De Luca A, Tortorella P, De Vito D, George A L, Conte Camerino D
Abstract excerpt
BACKGROUND: Myotonia and periodic paralysis caused by sodium channel mutations show variable responses to the anti-myotonic drug mexiletine. OBJECTIVE: To investigate whether variability among sodium channel mutants results from differences in drug binding affinity or in channel gating. METHODS: Whole-cell sodium currents (I(Na)) were recorded in tsA201 cells expressing human wild-type (WT) and mutant skeletal...
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