Figure 5

Phenotypic Features of TKO Larvae

Representative images of the phenotypes observed in 3 to 7 dpf fbn1^–/–; fbn2^–/–; fbn3^+/+, fbn1^–/–; fbn2^–/–; fbn3^+/–, and fbn1^–/–; fbn2^–/–; fbn3^–/– larvae. (A, left) Lateral whole-embryo view of a 5 dpf triple fibrillin knockout (TKO) without complete endocardial detachment and sibling controls, using brightfield microscopy. Accolade: fin-fold atrophy. (A, middle) Reconstructed maximum intensity projection in vivo confocal fluorescent images of the heart of 2 dpf Tg(kdrl:GFP) WT and TKO ZF. Endocardial detachment (asterisk) is observed in the atrium of a subset of TKO ZF. (A, right) Fluorescent ventral images of 7 dpf Tg(kdrl:GFP) TKO and sibling controls with preserved endocardial integrity. White arrowhead indicates dilated BA. (B) Phenotypic distribution (%) of TKO ZF presenting a mild (M) or severe (S) pericardial phenotype and their dynamics over time (3 and 5 dpf) (n = 21). (C) Fourteen-day Kaplan-Meier survival curve of the offspring of an incross of fbn1^–/–; fbn2^–/–; fbn3^+/– ZF (n = 21-40). (D) Quantification of standard length at 3 dpf (n = 17-36). (E) Quantification of BA diameters at 7 dpf during minimal (min) and maximal (max) distension (n = 17-19). Statistical analysis: Fisher exact test B, Kaplan-Meier survival analysis C, one-way analysis of variance followed by Dunnett’s multiple comparison test D, and two-way analysis of variance followed by Tukey’s multiple comparisons test E. Values are mean ± SEM. ∗P < 0.05. Abbreviations as in Figures 1 and 2.