A Novel and Lethal De Novo LQT-3 Mutation in a Newborn with Distinct Molecular Pharmacology and Therapeutic Response
Figure 2
The F1473C mutation increases non-inactivating late current.
Whole-cell wild type (WT, A) and mutant (F1473C, B) Na+ channel currents at −10 mV (200 ms pulses applied from a −100 mV holding potential at 0.5 Hz). Shown are averaged raw TTX sensitive traces (WT, n = 11; F1743C, n = 31). C. Averaged WT and F1473C currents normalized to peak current and superimposed at high (left) and low (inset) gain (WT n = 11, F1473C n = 31). D. Bar graph representing mean +/− S.E.M. percentage of current remaining at 200 ms with respect to peak current for both WT and F1473C (WT n = 11, F1473C n = 31); * p<<0.001.