Figure 2

Figure 2

Effect of 1 mM CSL525 in an abcc6a^cmg52/cmg52 zebrafish model of pseudoxanthoma elasticum. A total of 100 abcc6a^cmg52/cmg52 zebrafish larvae were exposed to 1 mM CSL525 at 3 days post-fertilization (dpf) for 7 days, while another group of 100 larvae was kept under the same conditions without CSL525. After 7 days of treatment (10 dpf), the fish were euthanized, fixed in 4% paraformaldehyde and stained with Alizarin Red S. (A) Representative images of the Alizarin Red S staining in abcc6^cmg52/cmg52 zebrafish untreated or treated with CSL525. (B) Survival curves during the 7 days of study. (C) Mineralized fraction area of spinal mineralization. Results are presented as mean ± SD. Statistical analysis: (A) Kaplan–Meier survival analysis. (B,C) Mann-Whitney test. (*) p < 0.05.