Figure 3

Figure 3

Effect of subcutaneous CSL525 at 15 mg/kg three times per week in a pseudoxanthoma elasticum abcc6^-/- mouse model. The abcc6^-/- mice were subcutaneously administered CSL525 at 15 mg/kg or 0.9% NaCl thrice weekly for eight weeks. A group of C57BL/6J wild-type mice, also receiving 0.9% NaCl thrice weekly, was included in the study. Each group consisted of five males and five females. All mice were maintained on a standard diet and sacrificed at 13 weeks of age, eight weeks after treatment initiation. (A) Histopathology of vibrissae calcification in the mice muzzle skin (von Kossa staining). Arrows indicate ectopic calcification. Scale bar: 0.4 mm. (B,C) Calcium (Ca) content and percentage inhibition of calcification in the muzzle skin. (D) Plasma pyrophosphate (PPi) concentration. (E) Alkaline phosphatase (ALP) activity in serum. WT, wild-type C57BL/6J mice. Results are presented as mean ± SD. Statistical analysis: one-way ANOVA with Tukey’s post hoc test. (***) p < 0.001, (****) p < 0.0001.