Mechanisms of cardiac arrhythmias and sudden death in transgenic rabbits with long QT syndrome.

@article{Brunner2008MechanismsOC,
  title={Mechanisms of cardiac arrhythmias and sudden death in transgenic rabbits with long QT syndrome.},
  author={Michael Brunner and Xuwen Peng and Gong Xin Liu and Xiao-Qin Ren and Ohad Ziv and B R Choi and Rajesh Mathur and Mohammed Hajjiri and Katja E. Odening and Eric R Steinberg and Eduardo J. Folco and Ekatherini Pringa and Jason N Centracchio and Roland Richard Macharzina and Tammy Donahay and Lorraine M Schofield and Naveed Rana and Malcolm M. Kirk and Gary F. Mitchell and Athena Poppas and Manfred Zehender and Gideon Koren},
  journal={The Journal of clinical investigation},
  year={2008},
  volume={118 6},
  pages={2246-59}
}
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) in the rabbit heart produced transgenic rabbits with a long QT phenotype… CONTINUE READING