Figure 4.

Stronger APD prolongation for the Timothy mutation Ca_V1.2^G406R compared to Ca_V1.2^L566P. (A) Model of the voltage dependence of inactivation of calcium currents in cardiomyocytes for heterozygous patients using the ten Tusscher and Panfilov variants for Ca_V1.2^G406R (blue) and Ca_V1.2^L566P (red), calculated with Ca²⁺ as charge carrier. Indicted are the percentage of mutant Ca_V1.2 transcripts in the hearts of heterozygous patients. (B) In silico ventricular action potential for Ca_V1.2^WT (black) or the heterozygous mutations Ca_V1.2^G406R (blue) and Ca_V1.2^L566P (red). (C) Corresponding intracellular Ca²⁺ concentrations during ventricular action potential.