In the absence of the Hh ligand, the receptor PTCH (Patched) acts to prevent high expression and activity of SMO (Smoothened). When Hh is bound, the repression of SMO is relieved which leads to activation of the GLI transcription factors: activators Gli1 and Gli2 and repressor Gli3. Activated GLI then controls the transcription of hedgehog target genes.
Pathway adapted from [http://www.genome.jp/kegg-bin/show_pathway?hsa04340 KEGG]parentid=Q9UMX1; parentsymbol=SUFU; site=HLQERVDkGIETDGS; position=lys257; sitegrpid=5009206; ptm=ub; direction=u
a8bsignaling pathway pertinent to developmentPW:0000650Pathway Ontology27234298PubMedSCF (Fbxl17) ubiquitylation of Sufu regulates Hedgehog signaling and medulloblastoma development.EMBO J2016Raducu MFung ESerres SInfante PBarberis AFischer RBristow CThézénas MLFinta CChristianson JCBuffa FMKessler BMSibson NRDi Marcotullio LToftgård RD'Angiolella Vsignaling pathwayPW:0000003Pathway OntologyHedgehog signaling pathwayPW:0000122Pathway Ontology