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Hedgehog (Hh) pathway. PTCH1/2 (patched) constitutively inhibits smoothened (Smo).
Notes: Inhibition of PTCH1, either through Hh ligand binding or inactivating mutations in PTCH1, promotes Smo activation. Smo activation, in turn, leads to GLI1 transcriptional activation and upregulation of Smo-dependent genes. Oral smoothened inhibitors inhibit Smo activation, leading to a downregulation of GLI1-dependent transcriptional activity.
Abbreviation: Shh, sonic Hh.
