Smoothened

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Smoothened homolog
Identifiers
Symbol SMO Gx; SMOH
HUGO 11119
Entrez 6608
OMIM 601500
RefSeq NM_005631
UniProt Q8NH34
Other data
Locus Chr. 7 q32

Smoothened is a 7TM protein of the hedgehog pathway conserved from flies to humans. It is the molecular target of the teratogen cyclopamine.[1]

Cellular localization plays an essential role in the function of SMO. Stimulation of the Patched receptor by Sonic hedgehog 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.[2] SMO has also been shown to bind Costal-2 and play a role in the localization of the Ci cytoplasmic complex.

SMO can function as an oncogene. Activating SMO mutations can lead to unregulated activation of the hedgehog pathway and cancer.[3]

[edit] References

  1. ^ Taipale J, Chen JK, Cooper MK, Wang B, Mann RK, Milenkovic L, Scott MP and Beachy PA. (2000) Effects of oncogenic mutations in Smoothened and Patched can be reversed by cyclopamine. Nature, 406:1005-9. Entrez PubMed 10984056
  2. ^ Corbit KC, Aanstad P, Singla V, Norman AR, Stainier DY and Reiter JF.(2005) Vertebrate Smoothened functions at the primary cilium. Nature, 437:1018-21. Entrez PubMed 16136078
  3. ^ Xie J, Murone M, Luoh SM, Ryan A, Gu Q, Zhang C, Bonifas JM, Lam CW, Hynes M, Goddard A, et al (1998) Activating Smoothened mutations in sporadic basal-cell carcinoma. Nature, 391:90-2. Entrez PubMed 9422511

[edit] External links