Figure 9.
AON-treatment effect at protein level and impact on ciliation. (A) CEP290 protein analysis in control and mutant cell lines untreated or treated during 48 h with 75 nM of H36D. (B) Quantification of CEP290 protein abundance relative to β-Actin. Bars correspond to the mean value ± SEM from three independent experiments = not significant. A.U. = arbitrary unit. (C) Quantification of CEP290 immunofluorescence intensity at the basal body in each cell line. Each dot depicts the labeling intensity of the protein in individual cells recorded automatically from six microscope fields. The solid line indicates the mean. (D) Quantification of CP110 immunofluorescence intensity at centrosomes in quiescent fibroblasts. All automatic intensity measures were recorded from six fields. (E) Percentage of ciliated cells and (F) length of cilia axonemes in control and mutant fibroblasts. A minimum of 90 ciliated cells were considered for each cell lines. C corresponds to C1–C3 pooled values. * p ≤ 0.05, ** p ≤ 0.01, *** p ≤ 0.001, **** p ≤ 0.0001, n.s = not significant, A.U. = arbitrary unit.