Figure 6.
Sufu mutation results in activation of Hh signaling and protein stability variation of Gli2/3. A, in situ hybridization showing up-regulation of Gli1 within the presumptive frontal primordium in Sufufx/fx;Wnt1-Cre (SufuWnt1Cre) mutants compared with littermate wild-type control (n = 3) during calvarial bone development. Dashed lines outline the presumptive frontal primordium. B, Western blot showing a discernable increase of full-length Gli2 (Gli2F) and significant decrease of truncated repressor forms Gli2 (Gli2R) and Gli3 (Gli3R) in mesenchymal cells within the frontal primordium in the Sufu mutant versus the wild type (n = 3). Each image shows a representative result of independent triplicated experiments. Expression levels were quantified from the band intensity as relative values of the target protein/actin expression ratios. *, p < 0.05; **, p < 0.01; ns, non-significant; Student's t test. Error bars, S.D. Wt, wild type; M, Sufufx/fx;Wnt1-Cre.