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Gene Review

Sufu  -  suppressor of fused homolog (Drosophila)

Mus musculus

Synonyms: 2810026F04Rik, Su(Fu), Suppressor of fused homolog, b2b273Clo
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Disease relevance of Sufu

  • Here, we show that ablation of the mouse Suppressor of fused (Sufu), an intracellular pathway component, leads to embryonic lethality at approximately E9.5 with cephalic and neural tube defects [1].
  • Sufu(+/-) mice develop a skin phenotype with basaloid changes and jaw keratocysts, characteristic features of Gorlin syndrome, a human genetic disease linked to enhanced Hh signaling [1].

High impact information on Sufu

  • Studies with both invertebrates and vertebrates have led to a model in which a protein complex composed of Fu, Sufu, and Cos2 controls the processing, activity, and subcellular distribution of the Ci/Gli transcription factors responsible for Hh target gene activation [2].
  • The resemblance of Sufu homozygotes to Ptch1 homozygotes is consistent with mouse Sufu being a conserved negative modulator of Hh signaling [3].
  • This report, in conjunction with recent studies, points out the importance of Sufu in mouse embryonic development [4].
  • Mouse Sufu is expressed in various tissues, particularly in the nervous system, ectoderm, and limbs, throughout the developing embryo [4].
  • Despite the robust constitutive pathway activation in the Sufu(-/-) fibroblasts, the GLI1 steady-state localization remained largely cytoplasmic, implying the presence of an effective nuclear export mechanism [1].

Biological context of Sufu

  • Sufu (Suppressor of fused) is a negative regulator of the Hedgehog signal-transduction pathway, interacting directly with the Gli family of transcription factors [5].


  1. Genetic elimination of Suppressor of fused reveals an essential repressor function in the mammalian Hedgehog signaling pathway. Svärd, J., Heby-Henricson, K., Henricson, K.H., Persson-Lek, M., Rozell, B., Lauth, M., Bergström, A., Ericson, J., Toftgård, R., Teglund, S. Dev. Cell (2006) [Pubmed]
  2. Mice deficient in the fused homolog do not exhibit phenotypes indicative of perturbed hedgehog signaling during embryonic development. Chen, M.H., Gao, N., Kawakami, T., Chuang, P.T. Mol. Cell. Biol. (2005) [Pubmed]
  3. Cardiac and CNS defects in a mouse with targeted disruption of suppressor of fused. Cooper, A.F., Yu, K.P., Brueckner, M., Brailey, L.L., Johnson, L., McGrath, J.M., Bale, A.E. Development (2005) [Pubmed]
  4. Genomic organization, chromosomal assignment, and expression analysis of the mouse suppressor of fused gene (Sufu) coding a Gli protein partner. Simon-Chazottes, D., Paces-Fessy, M., Lamour-Isnard, C., Guénet, J.L., Blanchet-Tournier, M.F. Mamm. Genome (2000) [Pubmed]
  5. The negative regulator of Gli, Suppressor of fused (Sufu), interacts with SAP18, Galectin3 and other nuclear proteins. Paces-Fessy, M., Boucher, D., Petit, E., Paute-Briand, S., Blanchet-Tournier, M.F. Biochem. J. (2004) [Pubmed]
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