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'''Suppressor of fused homolog''' is a [[protein]] that in humans is encoded by the ''SUFU'' [[gene]].<ref name="pmid15367681">{{cite journal | vauthors = Merchant M, Vajdos FF, Ultsch M, Maun HR, Wendt U, Cannon J, Desmarais W, Lazarus RA, de Vos AM, de Sauvage FJ | title = Suppressor of fused regulates Gli activity through a dual binding mechanism | journal = Mol Cell Biol | volume = 24 | issue = 19 | pages = 8627–41 |date=Sep 2004 | pmid = 15367681 | pmc = 516763 | doi = 10.1128/MCB.24.19.8627-8641.2004 }}</ref><ref name="entrez">{{cite web | title = Entrez Gene: SUFU suppressor of fused homolog (Drosophila)| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=51684| accessdate = }}</ref> | |||
== Function == | |||
SUFU encodes a component of the sonic hedgehog ([[sonic hedgehog|SHH]]) / patched ([[PTCH1|PTCH]]) signaling [[hedgehog signaling pathway|pathway]]. Mutations in genes encoding components of this pathway are deleterious for normal development and are associated with cancer-predisposing syndromes (e.g., holoprosencephaly, [[holoprosencephaly|HPE3]]; basal cell nevus syndrome, [[basal cell nevus syndrome|BCNS]]; and Greig cephalopolysyndactyly syndrome; [[Greig cephalopolysyndactyly syndrome|GCPS]]).<ref name="entrez" /> | |||
== Interactions == | |||
SUFU has been shown to [[Protein-protein interaction|interact]] with [[GLI1]],<ref name="pmid10564661">{{cite journal | vauthors = Stone DM, Murone M, Luoh S, Ye W, Armanini MP, Gurney A, Phillips H, Brush J, Goddard A, de Sauvage FJ, Rosenthal A | title = Characterization of the human suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli | journal = J. Cell Sci. | volume = 112 | issue = 23| pages = 4437–48 |date=December 1999 | pmid = 10564661 | doi = | url = | issn = }}</ref><ref name="pmid10559945">{{cite journal | vauthors = Kogerman P, Grimm T, Kogerman L, Krause D, Undén AB, Sandstedt B, Toftgård R, Zaphiropoulos PG | title = Mammalian suppressor-of-fused modulates nuclear-cytoplasmic shuttling of Gli-1 | journal = Nat. Cell Biol. | volume = 1 | issue = 5 | pages = 312–9 |date=September 1999 | pmid = 10559945 | doi = 10.1038/13031 | url = | issn = }}</ref><ref name="pmid12426310">{{cite journal | vauthors = Dunaeva M, Michelson P, Kogerman P, Toftgard R | title = Characterization of the physical interaction of Gli proteins with SUFU proteins | journal = J. Biol. Chem. | volume = 278 | issue = 7 | pages = 5116–22 |date=February 2003 | pmid = 12426310 | doi = 10.1074/jbc.M209492200 | url = | issn = }}</ref> [[GLI3]]<ref name="pmid20360384">{{cite journal | vauthors = Humke EW, Dorn KV, Milenkovic L, Scott MP, Rohatgi R | title = The output of Hedgehog signaling is controlled by the dynamic association between Suppressor of Fused and the Gli proteins | journal = Genes Dev. | volume = 24 | issue = 7 | pages = 670–82 |date=April 2010 | pmid = 20360384 | doi = 10.1101/gad.1902910 | url = | issn = | pmc = 2849124 }}</ref> and [[PEX26]].<ref name="pmid16189514">{{cite journal | vauthors = Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M | title = Towards a proteome-scale map of the human protein-protein interaction network | journal = Nature | volume = 437 | issue = 7062 | pages = 1173–8 |date=October 2005 | pmid = 16189514 | doi = 10.1038/nature04209 | url = | issn = }}</ref> | |||
== References == | |||
{{reflist}} | |||
== Further reading == | |||
{{refbegin | 2}} | |||
{{PBB_Further_reading | |||
| citations = | |||
*{{cite journal |vauthors=Kogerman P, Grimm T, Kogerman L, etal |title=Mammalian suppressor-of-fused modulates nuclear-cytoplasmic shuttling of Gli-1 |journal=Nat. Cell Biol. |volume=1 |issue= 5 |pages= 312–9 |year= 1999 |pmid= 10559945 |doi= 10.1038/13031 }} | |||
*{{cite journal |vauthors=Stone DM, Murone M, Luoh S, etal |title=Characterization of the human suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli |journal=J. Cell Sci. |volume=112 |issue= 23|pages= 4437–48 |year= 2000 |pmid= 10564661 |doi= }} | |||
*{{cite journal |vauthors=Murone M, Luoh SM, Stone D, etal |title=Gli regulation by the opposing activities of fused and suppressor of fused |journal=Nat. Cell Biol. |volume=2 |issue= 5 |pages= 310–2 |year= 2000 |pmid= 10806483 |doi= 10.1038/35010610 }} | |||
*{{cite journal | vauthors=Delattre M, Briand S, Paces-Fessy M, Blanchet-Tournier MF |title=The Suppressor of fused gene, involved in Hedgehog signal transduction in Drosophila, is conserved in mammals |journal=Dev. Genes Evol. |volume=209 |issue= 5 |pages= 294–300 |year= 2001 |pmid= 11252182 |doi=10.1007/s004270050255 }} | |||
*{{cite journal | vauthors=Cheng SY, Bishop JM |title=Suppressor of Fused represses Gli-mediated transcription by recruiting the SAP18-mSin3 corepressor complex |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue= 8 |pages= 5442–7 |year= 2002 |pmid= 11960000 |doi= 10.1073/pnas.082096999 | pmc=122788 }} | |||
*{{cite journal |vauthors=Taylor MD, Liu L, Raffel C, etal |title=Mutations in SUFU predispose to medulloblastoma |journal=Nat. Genet. |volume=31 |issue= 3 |pages= 306–10 |year= 2002 |pmid= 12068298 |doi= 10.1038/ng916 }} | |||
*{{cite journal | vauthors=Dunaeva M, Michelson P, Kogerman P, Toftgard R |title=Characterization of the physical interaction of Gli proteins with SUFU proteins |journal=J. Biol. Chem. |volume=278 |issue= 7 |pages= 5116–22 |year= 2003 |pmid= 12426310 |doi= 10.1074/jbc.M209492200 }} | |||
*{{cite journal |vauthors=Strausberg RL, Feingold EA, Grouse LH, etal |title=Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue= 26 |pages= 16899–903 |year= 2003 |pmid= 12477932 |doi= 10.1073/pnas.242603899 | pmc=139241 }} | |||
*{{cite journal |vauthors=Clark HF, Gurney AL, Abaya E, etal |title=The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment |journal=Genome Res. |volume=13 |issue= 10 |pages= 2265–70 |year= 2003 |pmid= 12975309 |doi= 10.1101/gr.1293003 | pmc=403697 }} | |||
*{{cite journal |vauthors=Paces-Fessy M, Boucher D, Petit E, etal |title=The negative regulator of Gli, Suppressor of fused (Sufu), interacts with SAP18, Galectin3 and other nuclear proteins |journal=Biochem. J. |volume=378 |issue= Pt 2 |pages= 353–62 |year= 2004 |pmid= 14611647 |doi= 10.1042/BJ20030786 | pmc=1223961 }} | |||
*{{cite journal |vauthors=Taylor MD, Zhang X, Liu L, etal |title=Failure of a medulloblastoma-derived mutant of SUFU to suppress WNT signaling |journal=Oncogene |volume=23 |issue= 26 |pages= 4577–83 |year= 2004 |pmid= 15077159 |doi= 10.1038/sj.onc.1207605 }} | |||
*{{cite journal |vauthors=Deloukas P, Earthrowl ME, Grafham DV, etal |title=The DNA sequence and comparative analysis of human chromosome 10 |journal=Nature |volume=429 |issue= 6990 |pages= 375–81 |year= 2004 |pmid= 15164054 |doi= 10.1038/nature02462 }} | |||
*{{cite journal |vauthors=Gerhard DS, Wagner L, Feingold EA, etal |title=The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC) |journal=Genome Res. |volume=14 |issue= 10B |pages= 2121–7 |year= 2004 |pmid= 15489334 |doi= 10.1101/gr.2596504 | pmc=528928 }} | |||
*{{cite journal |vauthors=Rual JF, Venkatesan K, Hao T, etal |title=Towards a proteome-scale map of the human protein-protein interaction network |journal=Nature |volume=437 |issue= 7062 |pages= 1173–8 |year= 2005 |pmid= 16189514 |doi= 10.1038/nature04209 }} | |||
*{{cite journal |vauthors=Lyle R, Radhakrishna U, Blouin JL, etal |title=Split-hand/split-foot malformation 3 (SHFM3) at 10q24, development of rapid diagnostic methods and gene expression from the region |journal=Am. J. Med. Genet. A |volume=140 |issue= 13 |pages= 1384–95 |year= 2006 |pmid= 16691619 |doi= 10.1002/ajmg.a.31247 }} | |||
}} | |||
{{refend}} | |||
{{PDB Gallery|geneid=51684}} | |||
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External IDs | GeneCards: [1] | ||||||
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Species | Human | Mouse | |||||
Entrez |
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Ensembl |
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UniProt |
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RefSeq (mRNA) |
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RefSeq (protein) |
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Location (UCSC) | n/a | n/a | |||||
PubMed search | n/a | n/a | |||||
Wikidata | |||||||
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Suppressor of fused homolog is a protein that in humans is encoded by the SUFU gene.[1][2]
Function
SUFU encodes a component of the sonic hedgehog (SHH) / patched (PTCH) signaling pathway. Mutations in genes encoding components of this pathway are deleterious for normal development and are associated with cancer-predisposing syndromes (e.g., holoprosencephaly, HPE3; basal cell nevus syndrome, BCNS; and Greig cephalopolysyndactyly syndrome; GCPS).[2]
Interactions
SUFU has been shown to interact with GLI1,[3][4][5] GLI3[6] and PEX26.[7]
References
- ↑ Merchant M, Vajdos FF, Ultsch M, Maun HR, Wendt U, Cannon J, Desmarais W, Lazarus RA, de Vos AM, de Sauvage FJ (Sep 2004). "Suppressor of fused regulates Gli activity through a dual binding mechanism". Mol Cell Biol. 24 (19): 8627–41. doi:10.1128/MCB.24.19.8627-8641.2004. PMC 516763. PMID 15367681.
- ↑ 2.0 2.1 "Entrez Gene: SUFU suppressor of fused homolog (Drosophila)".
- ↑ Stone DM, Murone M, Luoh S, Ye W, Armanini MP, Gurney A, Phillips H, Brush J, Goddard A, de Sauvage FJ, Rosenthal A (December 1999). "Characterization of the human suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli". J. Cell Sci. 112 (23): 4437–48. PMID 10564661.
- ↑ Kogerman P, Grimm T, Kogerman L, Krause D, Undén AB, Sandstedt B, Toftgård R, Zaphiropoulos PG (September 1999). "Mammalian suppressor-of-fused modulates nuclear-cytoplasmic shuttling of Gli-1". Nat. Cell Biol. 1 (5): 312–9. doi:10.1038/13031. PMID 10559945.
- ↑ Dunaeva M, Michelson P, Kogerman P, Toftgard R (February 2003). "Characterization of the physical interaction of Gli proteins with SUFU proteins". J. Biol. Chem. 278 (7): 5116–22. doi:10.1074/jbc.M209492200. PMID 12426310.
- ↑ Humke EW, Dorn KV, Milenkovic L, Scott MP, Rohatgi R (April 2010). "The output of Hedgehog signaling is controlled by the dynamic association between Suppressor of Fused and the Gli proteins". Genes Dev. 24 (7): 670–82. doi:10.1101/gad.1902910. PMC 2849124. PMID 20360384.
- ↑ Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M (October 2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514.
Further reading
- Kogerman P, Grimm T, Kogerman L, et al. (1999). "Mammalian suppressor-of-fused modulates nuclear-cytoplasmic shuttling of Gli-1". Nat. Cell Biol. 1 (5): 312–9. doi:10.1038/13031. PMID 10559945.
- Stone DM, Murone M, Luoh S, et al. (2000). "Characterization of the human suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli". J. Cell Sci. 112 (23): 4437–48. PMID 10564661.
- Murone M, Luoh SM, Stone D, et al. (2000). "Gli regulation by the opposing activities of fused and suppressor of fused". Nat. Cell Biol. 2 (5): 310–2. doi:10.1038/35010610. PMID 10806483.
- Delattre M, Briand S, Paces-Fessy M, Blanchet-Tournier MF (2001). "The Suppressor of fused gene, involved in Hedgehog signal transduction in Drosophila, is conserved in mammals". Dev. Genes Evol. 209 (5): 294–300. doi:10.1007/s004270050255. PMID 11252182.
- Cheng SY, Bishop JM (2002). "Suppressor of Fused represses Gli-mediated transcription by recruiting the SAP18-mSin3 corepressor complex". Proc. Natl. Acad. Sci. U.S.A. 99 (8): 5442–7. doi:10.1073/pnas.082096999. PMC 122788. PMID 11960000.
- Taylor MD, Liu L, Raffel C, et al. (2002). "Mutations in SUFU predispose to medulloblastoma". Nat. Genet. 31 (3): 306–10. doi:10.1038/ng916. PMID 12068298.
- Dunaeva M, Michelson P, Kogerman P, Toftgard R (2003). "Characterization of the physical interaction of Gli proteins with SUFU proteins". J. Biol. Chem. 278 (7): 5116–22. doi:10.1074/jbc.M209492200. PMID 12426310.
- Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
- Clark HF, Gurney AL, Abaya E, et al. (2003). "The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment". Genome Res. 13 (10): 2265–70. doi:10.1101/gr.1293003. PMC 403697. PMID 12975309.
- Paces-Fessy M, Boucher D, Petit E, et al. (2004). "The negative regulator of Gli, Suppressor of fused (Sufu), interacts with SAP18, Galectin3 and other nuclear proteins". Biochem. J. 378 (Pt 2): 353–62. doi:10.1042/BJ20030786. PMC 1223961. PMID 14611647.
- Taylor MD, Zhang X, Liu L, et al. (2004). "Failure of a medulloblastoma-derived mutant of SUFU to suppress WNT signaling". Oncogene. 23 (26): 4577–83. doi:10.1038/sj.onc.1207605. PMID 15077159.
- Deloukas P, Earthrowl ME, Grafham DV, et al. (2004). "The DNA sequence and comparative analysis of human chromosome 10". Nature. 429 (6990): 375–81. doi:10.1038/nature02462. PMID 15164054.
- Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
- Rual JF, Venkatesan K, Hao T, et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514.
- Lyle R, Radhakrishna U, Blouin JL, et al. (2006). "Split-hand/split-foot malformation 3 (SHFM3) at 10q24, development of rapid diagnostic methods and gene expression from the region". Am. J. Med. Genet. A. 140 (13): 1384–95. doi:10.1002/ajmg.a.31247. PMID 16691619.
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