Figure 3. Loss of CLN3 triggers activation of c-Abl.

(A) Representative immunoblot image and quantification of c-Abl protein levels in Wt and CLN3^KO cells. Tubulin and Ponceau were used as loading controls. (B) Confocal fluorescence images of HEK293T WT and CLN3^KO cells immunostained for c-Abl protein. Nucleus are outlined by the yellow dashed line using Hoechst staining. Scale bar 10µm. On the right, quantification of the mean intensity of c-Abl at the nucleus from at least 100 cells per condition in each independent experiment, with a total of at least 500 cells. The yellow dots represent the mean of each independent experiment. (C) Immunohistochemistry analysis of c-Abl staining of control (Wt) and Cln3^Δex7/8 animals; Right panels are cropped images from thalamus; Quantification of the number of positive nucleus to c-Abl in the total area from the hippocampus (upper panel) and thalamus (bottom panel). (D) Representative immunoblot image and quantification of pTyr357-YAP in CLN3^KO cells control or treated for 24h with 0.1 µM imatinib (c-Abl inhibitor). GAPDH and Ponceau were used as loading controls. (E) mRNA levels of YAP target genes involved in apoptosis in HEK293T CLN3^KO cells untreated or treated for 24h with imatinib. All the results are mean±SEM of at least three independent experiments. * p<0.05; ** p<0.01; ***p<0.001. * p<0.05; *** p<0.001; using unpaired t-test.