Article
Mechanisms of cardiac arrhythmias and sudden death in transgenic rabbits with long QT syndrome.
The Journal of clinical investigation - 1 Jun 2008
Brunner Michael, Peng Xuwen, Liu Gong Xin, Ren Xiao-Qin, Ziv Ohad, Choi Bum-Rak, Mathur Rajesh, Hajjiri Mohammed, Odening Katja E, Steinberg Eric, Folco Eduardo J, Pringa Ekatherini, Centracchio Jason, Macharzina Roland R, Donahay Tammy, Schofield Lorraine, Rana Naveed, Kirk Malcolm, Mitchell Gary F, Poppas Athena, Zehender Manfred, Koren Gideon
Abstract excerpt
Long QT syndrome (LQTS) is a heritable disease associated with ECG QT interval prolongation, ventricular tachycardia, and sudden cardiac death in young patients. Among genotyped individuals, mutations in genes encoding repolarizing K+ channels (LQT1:KCNQ1; LQT2:KCNH2) are present in approximately 90% of affected individuals. Expression of pore mutants of the human genes KCNQ1 (KvLQT1-Y315S) and KCNH2 (HERG-G628S)...
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