Schematic of the sonic hedgehog signaling pathway

Schematic of the sonic hedgehog signaling pathway. (A) In the absence of Sonic hedgehog (Shh), the 12 transmembrane receptor Patched (Ptch) suppresses the seven transmembrane G-coupled receptor Smoothened (Smo) and the transcription factor Gli is sequestered into the cytoplasm by the Suppressor-of-Fuse (SuFu) preventing it from translocating to the nucleus and is degraded leading to a block of transcription. (B) In the presence of Shh, the suppression by Ptch is relieved and Smo activates Gli1 that is translocated to the nucleus and induces the transcription of Gli targets, including many proproliferative genes. (C) In medulloblastoma, loss of Ptch and mutations in Smo or SuFu lead to the constitutive activity of Smo, accumulation of Gli and increased transcription of proproliferative Gli-dependent targets. Asterisk (*) identifies proteins mutated in medulloblastoma.
Schematic of the sonic hedgehog signaling pathway
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Publication

Cerebellum: Development and Medulloblastoma. () Martine F. Roussel, et al. Curr Top Dev Biol. ;94:235-282. Figure: F12.

Gene mentions


Organism Group Word Match Source NCBI Symbol NCBI ID
Drosophila melanogaster Invertebrates HH HH ncbigene_symbol hh 42737
Drosophila melanogaster Invertebrates Ptch PTCH ncbigene_synonym ptc 35851
Drosophila melanogaster Invertebrates Smo SMO ncbigene_symbol smo 33196
Drosophila melanogaster Invertebrates Smo SMO ncbigene_synonym sm 37254
Drosophila melanogaster Invertebrates SuFu SUFU ncbigene_synonym Su(fu) 41565
Drosophila melanogaster Invertebrates OFF OFF ncbigene_symbol Off 249997
Drosophila melanogaster Invertebrates Gli GLI ncbigene_symbol Gli 34927
Drosophila melanogaster Invertebrates Gli GLI ncbigene_synonym ci 43767
Mus musculus Rodents Shh SHH ncbigene_symbol Shh 20423
Mus musculus Rodents Ptch PTCH ncbigene_synonym Ptch1 19206
Mus musculus Rodents Smo SMO ncbigene_symbol Smo 319757
Mus musculus Rodents Smo SMO ncbigene_synonym Smox 228608
Mus musculus Rodents SuFu SUFU ncbigene_symbol Sufu 24069
Mus musculus Rodents Gli G2 ncbigene_synonym Gabrg2 14406
Homo sapiens Primates Shh SHH ncbigene_symbol SHH 6469
Homo sapiens Primates Ptch PTCH ncbigene_synonym PTCH1 5727
Homo sapiens Primates Smo SMO ncbigene_symbol SMO 6608
Homo sapiens Primates Smo SMO ncbigene_synonym SMOX 54498
Homo sapiens Primates SuFu SUFU ncbigene_symbol SUFU 51684
Homo sapiens Primates Gli GLI ncbigene_synonym GLI1 2735
Rattus norvegicus Rodents Shh SHH ncbigene_symbol Shh 29499
Rattus norvegicus Rodents Ptch PTCH ncbigene_synonym Ptch1 89830
Rattus norvegicus Rodents Smo SMO ncbigene_symbol Smo 25273
Rattus norvegicus Rodents SuFu SUFU ncbigene_symbol Sufu 361769
Rattus norvegicus Rodents Gli GLI ncbigene_synonym Gli1 140589
Gallus gallus Vertebrates Shh SHH ncbigene_symbol SHH 395615
Gallus gallus Vertebrates Ptch PTCH ncbigene_synonym PTCH1 395806
Gallus gallus Vertebrates Smo SMO ncbigene_symbol SMO 395949
Gallus gallus Vertebrates SuFu SUFU ncbigene_symbol SUFU 374118
Gallus gallus Vertebrates Gli GLI ncbigene_synonym GLI1 396045
Danio rerio Vertebrates Shh SHH ncbigene_synonym shha 30269
Danio rerio Vertebrates Smo SMO ncbigene_symbol smo 30225
Danio rerio Vertebrates SuFu SUFU ncbigene_symbol sufu 100001615
Danio rerio Vertebrates Gli GLI ncbigene_symbol gli 30097

Chemical mentions

Word Match MeSH Name ChEBI
Cyclopamine Cyclopamine mesh:C000541 cyclopamine chebi:4021

Disease mentions

Word Match MeSH Name DOID