Article
Altered Na+ channels promote pause-induced spontaneous diastolic activity in long QT syndrome type 3 myocytes.
Circulation research - 24 Nov 2006
Fredj Sandra, Lindegger Nicolas, Sampson Kevin J, Carmeliet Peter, Kass Robert S
Abstract excerpt
Long QT syndrome (LQTS) type 3 (LQT3), typified by the DeltaKPQ mutation (LQT3 mutation in which amino acid residues 1505 to 1507 [KPQ] are deleted), is caused by increased sodium entry during the action potential plateau resulting from mutation-altered inactivation of the Na(v)1.5 channel. Although rare, LQT3 is the most lethal of common LQTS variants. Here we tested the hypothesis that cellular electrical...
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