D
IPR024314

Suppressor of fused C-terminal

InterPro entry
Short nameSUFU_C
Overlapping
homologous
superfamilies
 

Description

This entry represents the C-terminal domain of eukaryotic suppressor of fused (Sufu) proteins. It folds into a domain that comprises a four-stranded and a six-stranded β-sheet, both with mixed topologies, which are connected by two antiparallel α-helices
[2, 3]
.

Sufu is a repressor of the Gli and Ci transcription factors of the Hedgehog signalling cascade
[4]
. It functions by binding to these proteins and preventing their translocation to the nucleus. The Sufu C-terminal domain binds to the N-terminal of Gli/Ci, while the N-terminal of Sufu binds to the C-terminal of Gli/Ci. This dual binding mechanism is likely to be an evolutionary advancement in this signalling cascade, which is not present in bacterial homologues
[1]
.

References

1.Suppressor of fused regulates Gli activity through a dual binding mechanism. Merchant M, Vajdos FF, Ultsch M, Maun HR, Wendt U, Cannon J, Desmarais W, Lazarus RA, de Vos AM, de Sauvage FJ. Mol. Cell. Biol. 24, 8627-41, (2004). View articlePMID: 15367681

2.Structural insight into the mutual recognition and regulation between Suppressor of Fused and Gli/Ci. Zhang Y, Fu L, Qi X, Zhang Z, Xia Y, Jia J, Jiang J, Zhao Y, Wu G. Nat Commun 4, 2608, (2013). PMID: 24217340

3.Structural basis of SUFU-GLI interaction in human Hedgehog signalling regulation. Cherry AL, Finta C, Karlstrom M, Jin Q, Schwend T, Astorga-Wells J, Zubarev RA, Del Campo M, Criswell AR, de Sanctis D, Jovine L, Toftgard R. Acta Crystallogr D Biol Crystallogr 69, 2563-79, (2013). PMID: 24311597

4.Hypomorphic Recessive Variants in SUFU Impair the Sonic Hedgehog Pathway and Cause Joubert Syndrome with Cranio-facial and Skeletal Defects. De Mori R, Romani M, D'Arrigo S, Zaki MS, Lorefice E, Tardivo S, Biagini T, Stanley V, Musaev D, Fluss J, Micalizzi A, Nuovo S, Illi B, Chiapparini L, Di Marcotullio L, Issa MY, Anello D, Casella A, Ginevrino M, Leggins AS, Roosing S, Alfonsi R, Rosati J, Schot R, Mancini GMS, Bertini E, Dobyns WB, Mazza T, Gleeson JG, Valente EM. Am J Hum Genet 101, 552-563, (2017). PMID: 28965847

GO terms

biological process

  • None

cellular component

  • None
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