Figure 7.

RNAi-mediated knockdown of paired-like homeodomain 2 in Pa. DSP^{p.Tyr1188His/WT} cardiomyocytes rescues the expression of structural and ion-related units

(A and B) Molecular analyses on 1-month-old Pa. DSP^WT/WT and Pa. DSP^{p.Tyr1188His/WT} cardiomyocytes treated with scramble siRNA or siRNA targeting PITX2 for 72 h. Data are obtained from 3–4 independent experiments.

(A) Representative immunoblots for PITX2, DSP, CX43, NaV1.5, and VIN.

(B) Quantification for PITX2, DSP, CX43, and NaV1.5 normalized to VIN.

(C–E) Molecular analyses on 1-month-old KI. DSP^WT/WT and KI. DSP^{p.Arg1113X/WT} cardiomyocytes. Data are obtained from three independent experiments.

(C) Gene expression levels for DSP and PITX2 normalized to GUS.

(D) Representative immunoblots for PITX2 and VIN.

(E) Quantification of PITX2 protein levels normalized to VIN.

Data are plotted as mean. The dots in (B), (C), and (E) represent technical replicates, whereas the color of each dot indicates the experimental origin (3–4 independent experiments; 3–6 technical replicates). For (B), significance has been assessed on log-transformed data using an ordinary two-way ANOVA followed by a Tukey’s multiple comparisons test (single pooled variance; alpha = 0.05). For (C) and (E), a two-tailed unpaired Student’s t test was applied (^∗p < 0.05, ^∗∗p < 0.01, ^∗∗∗p < 0.001, ^∗∗∗∗p < 0.0001, ns, not significant).