Figure 1
From: Acitretin mitigates uroporphyrin-induced bone defects in congenital erythropoietic porphyria models
Zebrafish model of CEP develops bone phenotype resembling human disease. (A) 6dpf zebrafish larvae were injected with uro-I or vehicle and imaged by confocal microscopy at 7dpf. Porphyrin was detected only in the bones of uro-I-injected group. Arrowhead-operculum; box-vertebrae. (B) Larvae were treated as in (A) and injected with calcein prior to imaging. Arrowhead-operculum; arrow-4th vertebra. (C) Quantification of bone volume in larvae from (B); bone volume was normalized to vehicle-injected larvae set to 100%. Symbols represent individual larvae (14–18/group) from 4–5 independent experiments. (D) Larvae were treated as in (A). At 7dpf bones were harvested and imaged by epifluorescence microscopy pre and post HCl bone demineralization, arrow-notochord. (E) Hydroxyapatite was incubated with calcein/uro-I/copro-I and imaged by epifluorescence microscopy. Scale bars: 200 µm (A-D); 50 µm (E). Three-dimensional image reconstruction (A, B) was performed using Imaris 3D software v7.7 (http://imaris.oxinst.com/). **p < 0.01.
