In CCA cell lines, the stimulation of the pathway with recombinant SHH ligand resulted as being very effective in activating HH-dependent transcriptional activity and in promoting in vitro cholangiocarcinoma cell growth, which were both negatively affected by the administration of the 5E1 Hedgehog-neutralizing antibody and by the genetic modulation of GLI1 [138]. This evidence concerns the gene SHH and cholangiocarcinoma.