Fig. 1

PANX1 Level and Channel Function are Reduced in Immortalized Myoblasts from DMD Patients. A Western blots of PANX1 levels in immortalized human skeletal muscle myoblasts isolated from 3 healthy (Ctl) and 8 dystrophic (DMD) donors and B) the respective quantification. β-actin was used as a loading control. * P < 0.05 compared to Ctl. C Representative western blot of Ctl myoblasts that were treated with either siRNA targeting PANX1 (PANX1 siRNA), a scrambled control (Ctl siRNA) or were untreated (WT: wild-type) (top). The incidence of dye uptake (%) in WT, Ctl siRNA, and PANX1 siRNA cells in high [K⁺] (bottom) (n = 3; one-way ANOVA followed by Tukey’s multiple comparisons test). Data represents mean ± s.d. ** P < 0.01 in comparison to WT; # P < 0.05 in comparison to Ctl siRNA; ns: non-significant. D Incidence of dye uptake in cell lines Ctl1-3 and DMD 1, DMD 2, and DMD 7 in the presence of either low [K⁺] or high [K⁺] (n = 3; two-way ANOVA followed by Sidak’s multiple comparisons test). Data are represented as mean ± s.d. While not indicated on the graph, the difference between low [K⁺] and high [K⁺] was statistically significant for all cell lines (P < 0.0001). There was no statistical difference between the various cell lines in low [K⁺]. The statistical significance between the Ctl and DMD cell lines in high [K⁺] are indicated on the graph: *** P < 0.001 and **** P < 0.0001 in comparison to Ctl 1; # P < 0.05, ### P < 0.001, #### P < 0.0001 in comparison to Ctl 2; ððð P < 0.001 and ðððð P < 0.0001 in comparison to Ctl 3 in high [K.⁺]