CareLit Fachartikel
The pH-dependent effect of mexiletine on IVS4 sodium channel mutants R1448H/C
B Mohammadi, K Jurkat-Rott, A Alekov, F Lehmann-Horn, R Dengler, J Bufler · Aktuelle Neurologie · 2005 · Heft 10
Dokument
965111
CareLit-ID
Jahr
2005
Publikation
PDF
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Metadaten
DOI
ja
zitierfähig
Bibliografische Angaben
Zusammenfassung
The effects of extracellular pH (6.2, 7.4 and 8.2) and 0.1mM mexiletine, a channel blocker of the lidocaine type, are studied on two sodium channel mutations of the fourth voltage sensor, R1448H/C. The fast inactivated channel state to which mexiletine preferentially binds is destabilized by the mutations. In contrast to the expected low response of R1448H/C carriers, mexiletine is particularly effective in preventing exercise-induced stiffness and paralysis from which these patients suffer. Our measurements performed in the whole-cell mode on stably transfected HEK cells show for the first time that the mutatio…
Schlagworte
Mexiletine
Channel
Inactivation
R1448H
Mutations
Potential
Block
R1448C
Recovery
Inactivated
State
Which
Aktuelle Neurologie
