Loss of luminal Ca2+ activation in the cardiac ryanodine receptor is associated with ventricular fibrillation and sudden death.

@article{Jiang2007LossOL,
  title={Loss of luminal Ca2+ activation in the cardiac ryanodine receptor is associated with ventricular fibrillation and sudden death.},
  author={Dawei Jiang and Wenqian Chen and Ruiwu Wang and Lin Zhang and S R Wayne Chen},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2007},
  volume={104 46},
  pages={18309-14}
}
Different forms of ventricular arrhythmias have been linked to mutations in the cardiac ryanodine receptor (RyR)2, but the molecular basis for this phenotypic heterogeneity is unknown. We have recently demonstrated that an enhanced sensitivity to luminal Ca(2+) and an increased propensity for spontaneous Ca(2+) release or store-overload-induced Ca(2+) release (SOICR) are common defects of RyR2 mutations associated with catecholaminergic polymorphic or bidirectional ventricular tachycardia. Here… CONTINUE READING