Fig. 2: The ATS1 mouse replicates the patientʼs electrical phenotype.

a,c, Representative ECG recordings in Kir2.1WT (top) and Kir2.1∆314-315 (bottom) anesthetized mice showing the different ECG pattern in each group at different timepoints after a single dose of flecainide (40 mg kg−1) and isoproterenol (isoprenaline, ISO, 5 mg kg−1), respectively. b, Quantification of temporal changes in QRS and PR interval durations before and after flecainide. Every value represents ten averaged intervals of ten consecutive beats. Data are expressed as mean ± s.e.m. Statistical analyses were conducted using two-way ANOVA, followed by Tukey’s multiple comparisons (QRS at 240 seconds, P = 0.0039; 300 seconds, P = 0.0027; 360 seconds, P = 0.0002 / PR at 240 seconds, P = 0.0491; 300 seconds, P = 0.0502; 360 seconds, P = 0.0107). d, Corrected QT and PR intervals before and after ISO dose. Each value represents ten averaged intervals from ten consecutive beats. Data are expressed as mean ± s.e.m. Statistical analyses were conducted using two-way ANOVA, followed by Tukey’s multiple comparisons (QTc at 0 seconds, P = 0.0133; 60 seconds, P = 0.0203; 120 seconds and 180 seconds, P = 0.0025 and P = 0.0005; 240 seconds, P = 0.0005; 300 seconds, P = 0.0048; 360 seconds, P = 0.0190). e, Representative lead-II ECG traces and corresponding intracardiac recordings from anesthetized Kir2.1WT and Kir2.1∆314-315 mice. Graphs show the incidence of VT/VF in basal conditions (i; P = 0.0300), after flecainide (ii; P = 0.0075) and after ISO (iii; P = 0.0291). Animals that had at least one AF episode ≥2 seconds after burst pacing or S1-S2 stimulation are represented as graph insets. A period of polymorphic VT is shown (P = 0.2143, P = 0.0351 and P = 0.0291, for basal, flecainide and isoproterenol, respectively). Each value is represented as mean ± s.e.m. Statistical analyses were conducted using two-tailed t-test and Fisher’s exact test. *P < 0.05; **P < 0.01; ***P < 0.001.