Figure 2. Expression of unexpected GLI3 protein from the frameshifted Gli3 alleles in vivo.

(A) Schematic representation of WT and expected premature GLI3 proteins. When Hh signaling is repressed, it is known that the transcriptional activator of WT GLI3 (GLI3^FL) is proteolytically processed to the repressor form of GLI3^REP13. The anti-GLI3 antibody used in this study was generated against the N-terminal half of human GLI3 shown in the red bar. (B) Western blotting of lysates from WT (NIH3T3) and the six mutant cell lines carrying biallelic frameshift mutations with anti-GLI3 antibody.