FIGURE

Figure 5

ID
ZDB-FIG-220131-193
Publication
Klaver et al., 2021 - Protease-dependent defects in N-cadherin processing drive PMM2-CDG pathogenesis
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Figure 5

PC inhibition improves craniofacial phenotypes in pmm2m/m embryos.

(A) Pericardial injection of PC inhibitor. Confocal images of fli1a:EGFP-labeled cartilage structures show PCI injection improves chondrocyte morphology, organization, and differentiation in pmm2m/m embryos. White boxes, central portion of Meckel’s cartilage; orange boxes, lateral regions evaluated for phenotypic rescue. Scale bars: 10 μm. Percentage values indicate number of scored embryos exhibiting pictured phenotype. n = 30 embryos over 4 experiments. (B) Parameters assessed for rescue of central portion of Meckel’s cartilage (white boxes). Cells were scored for percentage of intercalated, ratio between long and short axis (measure of roundness), and percentage of cells with vacuoles. n = 8–15 treated embryos over 4 experiments. (C) Parameters assessed for rescue of lateral portion of Meckel’s cartilage (white boxes). Cells were scored as in B. n = 8–15 treated embryos over 4 experiments. (D) Experimental strategy involving injecting a morpholino targeting furina into mixed progeny of pmm2m/m incross at 1-cell stage and genotyping 3 dpf. Confocal images of fli1a:EGFP-labeled cartilage structures show inhibiting furina improves chondrocyte morphology, organization, and differentiation in pmm2m/m embryos. White boxes, central portion of Meckel’s cartilage; orange boxes, lateral regions evaluated for phenotypic rescue. Scale bars: 10 μm. Percentage values indicate the number of scored embryos exhibiting pictured phenotype. n = 8–15 treated embryos over 4 experiments. (E) Parameters assessed for rescue of central portion of Meckel’s cartilage (white boxes) following morpholino inhibition of furina. Cells were scored as in B. (F) Parameters assessed for rescue of lateral portion of Meckel’s cartilage (orange boxes) following morpholino inhibition of furina. Cells were scored as in B. Error bars show SEM, 2-way ANOVA, *P < 0.05, **P < 0.01, ***P < 0.0001, ****P < 0.0001.

Expression Data

Expression Detail
Antibody Labeling
Phenotype Data
Fish:
Condition:
Knockdown Reagent:
Observed In:
Stage: Days 7-13

Phenotype Detail
Acknowledgments
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