KEGG   PATHWAY: hsa04340Help
Entry
hsa04340                    Pathway                                
Name
Hedgehog signaling pathway - Homo sapiens (human)
Description
The Hedgehog (Hh) family of secreted signaling proteins plays a crucial role in development of diverse animal phyla, from Drosophila to humans, regulating morphogenesis of a variety of tissues and organs. Hh signaling is also involved in control of stem cell proliferation in adult tissues and aberrant activation of the Hh pathway has been linked to multiple types of human cancer. Members of the Hh family bind to patched (ptc), thus releasing smoothened (smo) to transduce a signal. Transcriptional activation occurs through the GLI family of proteins resulting in activation of target genes.
Class
Environmental Information Processing; Signal transduction
BRITE hierarchy
Pathway map
Hedgehog signaling pathway
hsa04340

All organismsOrtholog table
Disease
H00263  
Acrocallosal syndrome
H00267  
Holoprosencephaly (HPE)
H00482  
Brachydactyly
H00502  
Pallister-Hall syndrome
H00607  
46,XY disorders of sex development (Disorders of gonadal development)
H00646  
Odontoonychodermal dysplasia
H00652  
Solitary median maxillary central incisor syndrome
H00675  
Acrocapitofemoral dysplasia
H00855  
Triphalangeal thumb
H00886  
Donnai-Barrow syndrome
H00895  
Basal cell nevus syndrome
H01027  
Anophthalmia and microphthalmia (A/M)
H01160  
Schizencephaly
H01226  
GLI3 morphopathies
Drug
D09992  
Vismodegib (USAN/INN)
D10119  
Erismodegib (USAN)
D10324  
Saridegib (USAN)
D10325  
Saridegib hydrochloride (USAN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
6469  
SHH; sonic hedgehog [KO:K11988]
3549  
IHH; indian hedgehog [KO:K11989]
50846  
DHH; desert hedgehog [KO:K11990]
5727  
PTCH1; patched 1 [KO:K06225]
8643  
PTCH2; patched 2 [KO:K11101]
6608  
SMO; smoothened, frizzled family receptor [KO:K06226]
27148  
STK36; serine/threonine kinase 36 [KO:K06228] [EC:2.7.11.1]
51684  
SUFU; suppressor of fused homolog (Drosophila) [KO:K06229]
2735  
GLI1; GLI family zinc finger 1 [KO:K16797]
2736  
GLI2; GLI family zinc finger 2 [KO:K16798]
2737  
GLI3; GLI family zinc finger 3 [KO:K06230]
7471  
WNT1; wingless-type MMTV integration site family, member 1 [KO:K03209]
7472  
WNT2; wingless-type MMTV integration site family member 2 [KO:K00182]
7482  
WNT2B; wingless-type MMTV integration site family, member 2B [KO:K00182]
7473  
WNT3; wingless-type MMTV integration site family, member 3 [KO:K00312]
89780  
WNT3A; wingless-type MMTV integration site family, member 3A [KO:K00312]
54361  
WNT4; wingless-type MMTV integration site family, member 4 [KO:K00408]
7474  
WNT5A; wingless-type MMTV integration site family, member 5A [KO:K00444]
81029  
WNT5B; wingless-type MMTV integration site family, member 5B [KO:K00444]
7475  
WNT6; wingless-type MMTV integration site family, member 6 [KO:K00445]
7476  
WNT7A; wingless-type MMTV integration site family, member 7A [KO:K00572]
7477  
WNT7B; wingless-type MMTV integration site family, member 7B [KO:K00572]
7478  
WNT8A; wingless-type MMTV integration site family, member 8A [KO:K00714]
7479  
WNT8B; wingless-type MMTV integration site family, member 8B [KO:K00714]
7483  
WNT9A; wingless-type MMTV integration site family, member 9A [KO:K01064]
7484  
WNT9B; wingless-type MMTV integration site family, member 9B [KO:K01064]
7480  
WNT10B; wingless-type MMTV integration site family, member 10B [KO:K01357]
80326  
WNT10A; wingless-type MMTV integration site family, member 10A [KO:K01357]
7481  
WNT11; wingless-type MMTV integration site family, member 11 [KO:K01384]
51384  
WNT16; wingless-type MMTV integration site family, member 16 [KO:K01558]
650  
BMP2; bone morphogenetic protein 2 [KO:K04662]
652  
BMP4; bone morphogenetic protein 4 [KO:K04662]
64399  
HHIP; hedgehog interacting protein [KO:K06231]
2619  
GAS1; growth arrest-specific 1 [KO:K06232]
4036  
LRP2; low density lipoprotein receptor-related protein 2 [KO:K06233]
51715  
RAB23; RAB23, member RAS oncogene family [KO:K06234]
5566  
PRKACA; protein kinase, cAMP-dependent, catalytic, alpha [KO:K04345] [EC:2.7.11.11]
5567  
PRKACB; protein kinase, cAMP-dependent, catalytic, beta [KO:K04345] [EC:2.7.11.11]
5568  
PRKACG; protein kinase, cAMP-dependent, catalytic, gamma [KO:K04345] [EC:2.7.11.11]
5613  
PRKX; protein kinase, X-linked [KO:K04345] [EC:2.7.11.11]
2932  
GSK3B; glycogen synthase kinase 3 beta [KO:K03083] [EC:2.7.11.26]
122011  
CSNK1A1L; casein kinase 1, alpha 1-like [KO:K08957] [EC:2.7.11.1]
1452  
CSNK1A1; casein kinase 1, alpha 1 [KO:K08957] [EC:2.7.11.1]
1455  
CSNK1G2; casein kinase 1, gamma 2 [KO:K08958] [EC:2.7.11.1]
1456  
CSNK1G3; casein kinase 1, gamma 3 [KO:K08958] [EC:2.7.11.1]
53944  
CSNK1G1; casein kinase 1, gamma 1 [KO:K08958] [EC:2.7.11.1]
1453  
CSNK1D; casein kinase 1, delta [KO:K08959] [EC:2.7.11.1]
1454  
CSNK1E; casein kinase 1, epsilon [KO:K08960] [EC:2.7.11.1]
8945  
BTRC; beta-transducin repeat containing E3 ubiquitin protein ligase [KO:K03362]
23291  
FBXW11; F-box and WD repeat domain containing 11 [KO:K03362]
7546  
ZIC2; Zic family member 2 [KO:K06235]
Compound
C00187  
Cholesterol
Reference
  Authors
Cohen MM Jr.
  Title
The hedgehog signaling network.
  Journal
Am J Med Genet A 123:5-28 (2003)
Reference
  Authors
Lum L, Beachy PA.
  Title
The Hedgehog response network: sensors, switches, and routers.
  Journal
Science 304:1755-9 (2004)
Reference
  Authors
Mullor JL, Sanchez P, Altaba AR.
  Title
Pathways and consequences: Hedgehog signaling in human disease.
  Journal
Trends Cell Biol 12:562-9 (2002)
Reference
  Authors
Ruiz i Altaba A, Stecca B, Sanchez P.
  Title
Hedgehog--Gli signaling in brain tumors: stem cells and paradevelopmental programs in cancer.
  Journal
Cancer Lett 204:145-57 (2004)
Reference
  Authors
Nusse R.
  Title
Wnts and Hedgehogs: lipid-modified proteins and similarities in signaling mechanisms at the cell surface.
  Journal
Development 130:5297-305 (2003)
Reference
  Authors
Kalderon D.
  Title
Hedgehog signaling: Costal-2 bridges the transduction gap.
  Journal
Curr Biol 14:R67-9 (2004)
Reference
  Authors
Beachy PA, Karhadkar SS, Berman DM.
  Title
Tissue repair and stem cell renewal in carcinogenesis.
  Journal
Nature 432:324-31 (2004)
Reference
  Authors
Mann RK, Beachy PA.
  Title
Novel lipid modifications of secreted protein signals.
  Journal
Annu Rev Biochem 73:891-923 (2004)
Reference
  Authors
Frank-Kamenetsky M, Zhang XM, Bottega S, Guicherit O, Wichterle H, Dudek H, Bumcrot D, Wang FY, Jones S, Shulok J, Rubin LL, Porter JA.
  Title
Small-molecule modulators of Hedgehog signaling: identification and characterization of Smoothened agonists and antagonists.
  Journal
J Biol 1:10 (2002)
Reference
  Authors
Koyabu Y, Nakata K, Mizugishi K, Aruga J, Mikoshiba K.
  Title
Physical and functional interactions between Zic and Gli proteins.
  Journal
J Biol Chem 276:6889-92 (2001)
KO pathway
 
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