Hedgehog Signaling Pathway (Homo sapiens)

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1HH-producing cellNucleusCiliumHH-recieving cellActivated formCytoplasmDegradationCSNK1G2EVCPTCH1SMOHHIPDHHCSNK1A1LCSNK1A1CSNK1G1PRKACAGAS1CSNK1G3GRK2CSNK1EKIF3AGLI1CSNK1DARRB1SUFUFBXL17SHHIHHPTCH2HHIPPTCH1PTCH2UbSMURF1UbSMURF2LRP2CDONBOCSMOEVC2PRKACBPRKACGGRK3ARRB2GLI2GLI3GLI1GLI2GLI3KIF7GPR161GPR161SUFUUbDegradationSPOPSPOPLCUL3SPOPSPOPLCUL3GLI1GLI2GLI3PTCH1PTCH2CCND1CCND2BCL2DegradationSMO


Description

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

 

Bibliography

  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 Europe PMC

History

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CompareRevisionActionTimeUserComment
105491view05:46, 8 August 2019KhanspersModified description
105477view05:23, 8 August 2019KhanspersModified description
96941view20:00, 20 April 2018Khanspersedit board height
96859view23:36, 14 April 2018AlexanderPicoOntology Term : 'signaling pathway' added !
96788view19:23, 6 April 2018KhanspersOntology Term : 'signaling pathway pertinent to development' added !
96787view19:22, 6 April 2018KhanspersOntology Term : 'Hedgehog signaling pathway' added !
96786view19:22, 6 April 2018KhanspersModified description
96785view19:21, 6 April 2018Khansperschanged board height
96784view19:21, 6 April 2018KhanspersModified title
96783view19:20, 6 April 2018KhanspersNew pathway

External references

DataNodes

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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

No annotated interactions

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