Suppressor of fused homolog

Definition:

Negative regulator in the hedgehog/smoothened signaling pathway. Down-regulates GLI1-mediated transactivation of target genes. Down-regulates GLI2-mediated transactivation of target genes. Part of a corepressor complex that acts on DNA-bound GLI1. May also act by linking GLI1 to BTRC and thereby targeting GLI1 to degradation by the proteasome. Sequesters GLI1, GLI2 and GLI3 in the cytoplasm, this effect is overcome by binding of STK36 to both SUFU and a GLI protein. Negative regulator of beta-catenin signaling (By similarity). Regulates the formation of either the repressor form (GLI3R) or the activator form (GLI3A) of the full-length form of GLI3 (GLI3FL). GLI3FL is complexed with SUFU in the cytoplasm and is maintained in a neutral state. Without the Hh signal, the SUFU-GLI3 complex is recruited to cilia, leading to the efficient processing of GLI3FL into GLI3R. When Hh signaling is initiated, SUFU dissociates from GLI3FL and the latter translocates to the nucleus, where it is phosphorylated, destabilized, and converted to a transcriptional activator (GLI3A). Required for normal embryonic development (By similarity). Required for the proper formation of hair follicles and the control of epidermal differentiation (By similarity).

References:

[1]. Michael D Taylor, et al. Mutations in SUFU predispose to medulloblastoma. Nat Genet. 2002 Jul;31(3):306-10. [Content Brief]

[2]. M Murone, et al. Gli regulation by the opposing activities of fused and suppressor of fused. Nat Cell Biol. 2000 May;2(5):310-2. [Content Brief]

[3]. P Kogerman, et al. Mammalian suppressor-of-fused modulates nuclear-cytoplasmic shuttling of Gli-1. Nat Cell Biol. 1999 Sep;1(5):312-9. [Content Brief]

[4]. Mark Merchant, et al. Suppressor of fused regulates Gli activity through a dual binding mechanism. Mol Cell Biol. 2004 Oct;24(19):8627-41. [Content Brief]

[5]. Sumin Chi, et al. Rab23 negatively regulates Gli1 transcriptional factor in a Su(Fu)-dependent manner. Cell Signal. 2012 Jun;24(6):1222-8. [Content Brief]

[6]. Yan Zhang, et al. Structural insight into the mutual recognition and regulation between Suppressor of Fused and Gli/Ci. Nat Commun. 2013;4:2608. [Content Brief]

[7]. Madalina Raducu, et al. SCF (Fbxl17) ubiquitylation of Sufu regulates Hedgehog signaling and medulloblastoma development. EMBO J. 2016 Jul 1;35(13):1400-16. [Content Brief]

[8]. Amy L Cherry, et al. Structural basis of SUFU-GLI interaction in human Hedgehog signalling regulation. Acta Crystallogr D Biol Crystallogr. 2013 Dec;69(Pt 12):2563-79. [Content Brief]

[9]. D M Stone, et al. Characterization of the human suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli. J Cell Sci. 1999 Dec;112 ( Pt 23):4437-48. [Content Brief]

[10]. Roberta De Mori, et al. Hypomorphic Recessive Variants in SUFU Impair the Sonic Hedgehog Pathway and Cause Joubert Syndrome with Cranio-facial and Skeletal Defects. Am J Hum Genet. 2017 Oct 5;101(4):552-563. [Content Brief]

[11]. Hilary F Clark, et al. The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment. Genome Res. 2003 Oct;13(10):2265-70. [Content Brief]

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