Harman Patil (Editor)

Smoothened

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Species
  
Human

Entrez
  
6608

Human
  
Mouse

Ensembl
  
ENSG00000128602

Smoothened

Aliases
  
SMO, FZD11, Gx, SMOH, smoothened, frizzled class receptor

External IDs
  
MGI: 108075 HomoloGene: 4115 GeneCards: SMO

Smoothened is a protein that in humans is encoded by the SMO gene. Smoothened is not a G protein-coupled receptor (it is instead a GPCR-like receptor) that is a component of the hedgehog signaling pathway and is conserved from flies to humans. It is the molecular target of the teratogen cyclopamine.

Contents

Function

Cellular localization plays an essential role in the function of SMO. Stimulation of the patched receptor by the sonic hedgehog ligand leads to translocation of SMO to the primary cilium. Furthermore, SMO that is mutated in the domain required for ciliary localisation cannot contribute to pathway activation. SMO has also been shown to bind the kinesin motor protein Costal-2 and play a role in the localization of the Ci (Cubitus interruptus transcription factor) complex.

SMO can function as an oncogene. Activating SMO mutations can lead to unregulated activation of the hedgehog pathway and cancer.

Agonists

  • Smoothened agonist (SAG)
  • Purmorphamine
  • oxysterols including 20(S)-OHC and 20(S)-yne
  • Antagonists

  • Cyclopamine
  • Itraconazole
  • Vismodegib (Erivedge), a smoothened receptor inhibitor for the treatment of basal-cell carcinoma, being investigated for the treatment of other types of cancer
  • SANT-1
  • Sonidegib
  • References

    Smoothened Wikipedia