To investigate Hh signaling mechanisms in fibroblasts from C-S and COPD patients, we used modulators of the Hh pathway; the Smo agonists namely purmorphamine (PUR) and SAG, the Smo antagonist cyclopamine (CPN) and the Gli1 antagonist Gant61 (G61). This evidence concerns the gene GLI1 and chronic obstructive pulmonary disease.