Extended Data Fig. 4: Generation and characterization of Alpk3^W1538X mouse model.

a Representative AccI digest of genotyping PCR products from Alpk3^WT/WT, Alpk3^{W1538X(+/−)}, and Alpk3^W1538X(+/+) mice. b Sanger sequencing chromatograms of WT and Alpk3^W1538X(+/+) mice against reference c57/Bl6 mouse Alpk3 gene. c Summary echocardiography data (continued from Fig. 3i) of 6-week-old mice showing heart rate, left ventricular diastolic and systolic internal diameter, fractional shortening, diastolic posterior and anterior wall thickness, stroke volume, and diastolic volume. Statistical significance established by ordinary one-way ANOVA with Tukey’s multiple comparison test. Error bars represent S.E.M. d Immunofluorescent staining of ACTN2 (green) and MYOM1 (magenta) in 21-dayold Alpk3^WT/WT and Alpk3^W1538X(+/+) mouse hearts. Scale bar = 5 µm. e Immunofluorescent staining of SQSTM1 (red) and DAPI (blue) in 21-day-old Alpk3^WT/WT and Alpk3^W1538X(+/+) mouse hearts. Scale bar = 15 µm.

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