FIGURE SUMMARY
Title

The Abcc6a Knockout Zebrafish Model as a Novel Tool for Drug Screening for Pseudoxanthoma Elasticum

Authors
Van Gils, M., Willaert, A., Coucke, P.J., Vanakker, O.M.
Source
Full text @ Front Pharmacol
FIGURE 1

Light microscopy examples of ARS-stained abcc6acmg52/cmg52 specimens. Examples of 10 dpf untreated (A,C,E,G,I) and treated (B,D,F,H,J) abcc6acmg52/cmg52 animals. Compounds per set are (A,B) 80 µM vitamin K1, (C,D) 20 µM sodium thiosulfate (E,F) 100 µM etidronate, (G,H) 100 µM alendronate and (I,J) 10 mM magnesium citrate.
FIGURE 2

Compound treatment effects on the abcc6acmg52/cmg52 spinal hypermineralization phenotype. After semiquantification and statistical analysis, mean ± SD percentile values of the spinal mineralization were normalized per compound to the respective control values of the untreated abcc6acmg52/cmg52 fish. (A) 80 µM VK1 (58 ± 28) versus C (100 ± 48), 42% reduction (B) 20 µM STS (45 ± 40) versus C (100 ± 65), 55% reduction (C) 100 µM Etid. (65 ± 43) versus C (100 ± 67), 35% reduction (D) 100 µM Alen. (61 ± 39) versus C (100 ± 58), 39% reduction (E) 10 mM MgC (55 ± 27) versus C (100 ± 55), 45% reduction (F) 5 mM MgC (70 ± 42) versus C (100 ± 55), 30% reduction. Treatments resulted in significant reductions (*p < 0.05), except for 5 mM MgC.
FIGURE 3

Example of ectopic speckling following 35 µM STS-treatment. Snapshot group images of (A) control and (B) 35 µM STS-treated larvae at 10 dpf with unknown genotypes. 35µM sodium thiosulfate abolished spinal mineralization and concomitantly ventral mineralized nodules were identified (arrowheads) in all treated animals. Opacity of the specimens prevented identification of the anatomical sites of the nodules.
FIGURE 4

Schematic representation of the anti-mineralization effects of the tested compounds. For the compounds evaluated in the present study, the type of administration or absorption is mentioned in the top row, as well as the type of administration used in any mouse or human studies which were done with this compound. The anti mineralizing effects of the different compounds are shown, as well as where these effect take place in the cascade to form hydroxyapatite crystals. Finally, the differences between the hypermineralization in zebrafish and mammals with respect to abcc6a and ABCC6 deficiency are shown. Figure created with BioRender.com.
Acknowledgments
This image is the copyrighted work of the attributed author or publisher, and ZFIN has permission only to display this image to its users. Additional permissions should be obtained from the applicable author or publisher of the image. Full text @ Front Pharmacol
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