Mutant ryanodine receptors in catecholaminergic polymorphic ventricular tachycardia generate delayed afterdepolarizations due to increased propensity to Ca2+ waves.

@article{Paavola2007MutantRR,
  title={Mutant ryanodine receptors in catecholaminergic polymorphic ventricular tachycardia generate delayed afterdepolarizations due to increased propensity to Ca2+ waves.},
  author={Jere Paavola and Matti Viitasalo and P{\"a}ivi J Laitinen-Forsblom and Michael Pasternack and Heikki Swan and Ilkka Tikkanen and Lauri Toivonen and Kimmo K. Kontula and Mika Laine},
  journal={European heart journal},
  year={2007},
  volume={28 9},
  pages={1135-42}
}
AIMS Mutations in cardiac ryanodine receptors (RyR2s) are linked to catecholaminergic polymorphic ventricular tachycardia (CPVT), characterized by risk of polymorphic ventricular tachyarrhythmias and sudden death during exercise. Arrhythmias are caused by gain-of-function defects in RyR2, but cellular arrhythmogenesis remains elusive. METHODS AND RESULTS We recorded endocardial monophasic action potentials (MAPs) at right ventricular septum in 15 CPVT patients with a RyR2 mutation (P2,328S… CONTINUE READING
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