Figure 5.

CMG2 phosphorylation levels are increased in COL6-RD patient-derived fibroblasts. (A) Western blotting for CMG2 receptor and its tyrosine-phosphorylated form after immunoprecipitation (IP) of control, COL6-RD patient, and CRISPR-treated COL6-RD patient-derived fibroblasts. Blots of equally loaded protein extracts prior to IP (input) are also shown. The marker GAPDH is used as the loading control. (B) Total quantification of the IP phosphorylated form of CMG2 versus IP CMG2 from the three phenotypes is shown. Data are expressed as the mean ± SEM; n = 3; independent primary cell preparations were used for all panels. * p < 0.05, ** p < 0.01; one-way ANOVA/Bonferroni’s multiple comparison test.