Suppressor of Fused inhibits mammalian Hedgehog signaling in the absence of cilia

Jinping Jia, Åsa Kolterud, Huiqing Zeng, Amber Hoover, Stephan Teglund, Rune Toftgård, Aimin Liu

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109 Scopus citations


The Hedgehog (Hh) family of secreted proteins regulates mammalian development and cancer formation through Gli transcription factors, which exist in both activator and repressor forms. In vertebrates, the primary cilia play an essential role in Hh signal transduction and are required for both the activator and repressor activities of Gli proteins. In the current study, we demonstrate that mouse Suppressor of Fused (Sufu) interacts with Gli proteins and inhibits Gli activator activity in the absence of cilia. Removal of Sufu in both Smoothened (Smo) and Ift88 mutants, respectively, leads to full activation of Hh signaling, suggesting that Smo-mediated repression of Sufu, but not the inhibitory function of Sufu, requires cilia. Finally, we show that Sufu is important for proper activator/repressor ratio of Gli3 protein in mice, both in the presence and absence of cilia.

Original languageEnglish (US)
Pages (from-to)452-460
Number of pages9
JournalDevelopmental biology
Issue number2
StatePublished - Jun 15 2009

All Science Journal Classification (ASJC) codes

  • Molecular Biology
  • Developmental Biology
  • Cell Biology


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