Article
Arrhythmias precede cardiomyopathy and remodeling of Ca2+ handling proteins in a novel model of long QT syndrome.
Journal of molecular and cellular cardiology - 1 Oct 2018
Montnach Jérôme, Chizelle Franck F, Belbachir Nadjet, Castro Claire, Li Linwei, Loussouarn Gildas, Toumaniantz Gilles, Carcouët Agnès, Meinzinger Anne Julia, Shmerling Doron, Benitah Jean-Pierre, Gómez Ana Maria, Charpentier Flavien, Baró Isabelle
Abstract excerpt
AIM: Deletion of QKP1507-1509 amino-acids in SCN5A gene product, the voltage-gated Na+ channel Nav1.5, has been associated with a large phenotypic spectrum of type 3 long QT syndrome, conduction disorder, dilated cardiomyopathy and high incidence of sudden death. The aim of this study was to develop and characterize a novel model of type 3 long QT syndrome to study the consequences of the QKP1507-1509 deletion....
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