Hedgehog signaling pathway Gene Set

Dataset KEGG Pathways 2026
Category structural or functional annotations
Type pathway
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. (Kyoto Encyclopedia of Genes and Genomes, hsa04340)
External Link https://www.kegg.jp/entry/hsa04340
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56 proteins participating in the Hedgehog signaling pathway pathway from the KEGG Pathways 2026 dataset.

Symbol Name
ARRB1 arrestin, beta 1
ARRB2 arrestin, beta 2
BCL2 B-cell CLL/lymphoma 2
BOC BOC cell adhesion associated, oncogene regulated
BTRC beta-transducin repeat containing E3 ubiquitin protein ligase
CCND1 cyclin D1
CCND2 cyclin D2
CDON cell adhesion associated, oncogene regulated
CSNK1A1 casein kinase 1, alpha 1
CSNK1A1L casein kinase 1, alpha 1-like
CSNK1D casein kinase 1, delta
CSNK1E casein kinase 1, epsilon
CSNK1G1 casein kinase 1, gamma 1
CSNK1G2 casein kinase 1, gamma 2
CSNK1G3 casein kinase 1, gamma 3
CUL1 cullin 1
CUL3 cullin 3
DHH desert hedgehog
DISP1 dispatched homolog 1 (Drosophila)
EFCAB7 EF-hand calcium binding domain 7
EVC Ellis van Creveld protein
EVC2 Ellis van Creveld syndrome 2
FBXW11 F-box and WD repeat domain containing 11
GAS1 growth arrest-specific 1
GLI1 GLI family zinc finger 1
GLI2 GLI family zinc finger 2
GLI3 GLI family zinc finger 3
GPR161 G protein-coupled receptor 161
GRK2 G protein-coupled receptor kinase 2
GRK3 G protein-coupled receptor kinase 3
GSK3B glycogen synthase kinase 3 beta
HHAT hedgehog acyltransferase
HHATL hedgehog acyltransferase-like
HHIP hedgehog interacting protein
IHH indian hedgehog
IQCE IQ motif containing E
KIF3A kinesin family member 3A
KIF7 kinesin family member 7
LRP2 low density lipoprotein receptor-related protein 2
MEGF8 multiple EGF-like-domains 8
MGRN1 mahogunin ring finger 1, E3 ubiquitin protein ligase
MOSMO modulator of smoothened
PRKACB protein kinase, cAMP-dependent, catalytic, beta
PRKACG protein kinase, cAMP-dependent, catalytic, gamma
PRKCA protein kinase C, alpha
PTCH1 patched 1
PTCH2 patched 2
SCUBE2 signal peptide, CUB domain, EGF-like 2
SHH sonic hedgehog
SMOX spermine oxidase
SMURF1 SMAD specific E3 ubiquitin protein ligase 1
SMURF2 SMAD specific E3 ubiquitin protein ligase 2
SPOP speckle-type POZ protein
SPOPL speckle-type POZ protein-like
SUFU suppressor of fused homolog (Drosophila)
TPTEP2-CSNK1E TPTEP2-CSNK1E readthrough