Hedgehog Signaling Pathway (Homo sapiens)

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Nucleus HH-producing cell Cytoplasm Degradation HH-recieving cell Cilium Activated form GLI1 GLI2 GLI3 FBXL17 HHIP SMO ARRB1 KIF3A ARRB2 CSNK1G2 GRK2 CSNK1A1L CSNK1A1 CSNK1G3 CSNK1D CSNK1G1 CSNK1E GRK3 PTCH1 PTCH2 PRKACA PRKACB PRKACG DHH SHH IHH EVC EVC2 SUFU GLI1 GLI2 GLI3 KIF7 GAS1 LRP2 CDON BOC HHIP Ub PTCH1 Ub PTCH2 SMURF1 SMURF2 SMO GPR161 GPR161 Ub SUFU Degradation SPOP SPOPL CUL3 SPOP SPOPL CUL3 GLI1 GLI2 GLI3 PTCH1 PTCH2 CCND1 CCND2 BCL2 Degradation SMO Name: Hedgehog Signaling Pathway Organism: Homo sapiens


The Hedgehog (Hh) signaling pathway has numerous roles in the control of cell proliferation, tissue patterning, stem cell maintenance and development. The primary cilium is an important center for transduction of the Hedgehog signal in vertebrates. In Hh's absence, the Ptch receptor localizes to the cilium, where it inhibits Smo activation. Gli proteins are phosphorylated by PKA, CKI and GSK3B and partially degraded into truncated Gli repressor form (GliR) that suppresses Hh target gene transcription in the nucleus. In Hh's presence, Ptch disappears from the cilium, and activated Smo contributes to the translocation of the protein complex Gli, Sufu, Kif7 to ciliary tip, where Gli dissociates from the negative regulator Sufu. The production of Gli activator form (GliA) occurs and the increased nuclear accumulation of GliA results in activation transcription of Hh target genes.

Pathway adapted from KEGG

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Ontology Terms



  1. Raducu M, Fung E, Serres S, Infante P, Barberis A, Fischer R, Bristow C, Thézénas ML, Finta C, Christianson JC, Buffa FM, Kessler BM, Sibson NR, Di Marcotullio L, Toftgård R, D'Angiolella V; ''SCF (Fbxl17) ubiquitylation of Sufu regulates Hedgehog signaling and medulloblastoma development.''; EMBO J, 2016 PubMed


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96941view 13:00, 20 April 2018Khanspersedit board height
96859view 16:36, 14 April 2018AlexanderPicoOntology Term : 'signaling pathway' added !
96788view 12:23, 6 April 2018KhanspersOntology Term : 'signaling pathway pertinent to development' added !
96787view 12:22, 6 April 2018KhanspersOntology Term : 'Hedgehog signaling pathway' added !
96786view 12:22, 6 April 2018KhanspersModified description
96785view 12:21, 6 April 2018Khansperschanged board height
96784view 12:21, 6 April 2018KhanspersModified title
96783view 12:20, 6 April 2018KhanspersNew pathway

External references


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NameTypeDatabase referenceComment
ARRB1GeneProductENSG00000137486 (Ensembl)
ARRB2GeneProductENSG00000141480 (Ensembl)
BCL2GeneProductENSG00000171791 (Ensembl)
BOCGeneProductENSG00000144857 (Ensembl)
CCND1GeneProductENSG00000110092 (Ensembl)
CCND2GeneProductENSG00000118971 (Ensembl)
CDONGeneProductENSG00000064309 (Ensembl)
CSNK1A1GeneProductENSG00000113712 (Ensembl)
CSNK1A1LGeneProductENSG00000180138 (Ensembl)
CSNK1DGeneProductENSG00000141551 (Ensembl)
CSNK1EGeneProductENSG00000213923 (Ensembl)
CSNK1G1GeneProductENSG00000169118 (Ensembl)
CSNK1G2GeneProductENSG00000133275 (Ensembl)
CSNK1G3GeneProductENSG00000151292 (Ensembl)
CUL3GeneProductENSG00000036257 (Ensembl)
DHHGeneProductENSG00000139549 (Ensembl)
EVC2GeneProductENSG00000173040 (Ensembl)
EVCGeneProductEVC (HGNC)
FBXL17GeneProductENSG00000145743 (Ensembl)
GAS1GeneProductENSG00000180447 (Ensembl)
GLI1GeneProductENSG00000111087 (Ensembl)
GLI2GeneProductENSG00000074047 (Ensembl)
GLI3GeneProductENSG00000106571 (Ensembl)
GPR161GeneProductENSG00000143147 (Ensembl)
GRK2GeneProductENSG00000173020 (Ensembl)
GRK3GeneProductENSG00000100077 (Ensembl)
HHIPGeneProductENSG00000164161 (Ensembl)
IHHGeneProductENSG00000163501 (Ensembl)
KIF3AGeneProductENSG00000131437 (Ensembl)
KIF7GeneProductENSG00000166813 (Ensembl)
LRP2GeneProductENSG00000081479 (Ensembl)
PRKACAGeneProductENSG00000072062 (Ensembl)
PRKACBGeneProductENSG00000142875 (Ensembl)
PRKACGGeneProductENSG00000165059 (Ensembl)
PTCH1GeneProductENSG00000185920 (Ensembl)
PTCH2GeneProductENSG00000117425 (Ensembl)
SHHGeneProductENSG00000164690 (Ensembl)
SMOGeneProductENSG00000128602 (Ensembl)
SMURF1GeneProductENSG00000198742 (Ensembl)
SMURF2GeneProductENSG00000108854 (Ensembl)
SPOPGeneProductENSG00000121067 (Ensembl)
SPOPLGeneProductENSG00000144228 (Ensembl)
SUFUGeneProductENSG00000107882 (Ensembl)

Annotated Interactions

<cite>No annotated interactions</cite>