A Novel and Lethal De Novo LQT-3 Mutation in a Newborn with Distinct Molecular Pharmacology and Therapeutic Response
Figure 8
Simulation of the impact of the F1473C mutation and its modulation by mexiletine on the ventricular action potential.
A. Shown are the computed action potentials for the F1473C mutant (black trace) as compared to WT (blue trace) at 1Hz. Also shown is the contribution of F1473C late current alone to action potential prolongation, without changing the channel steady state availability (red trace). B. The simulations show the drug markedly rescues the channel function reducing the F1473C APD from 588 ms to 386 ms.