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Q9Z0P7 (SUFU_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 100. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (2) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Interactions·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Suppressor of fused homolog
Gene names
Name:Sufu
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length484 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Negative regulator in the hedgehog signaling pathway. Down-regulates GLI1-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. 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). Ref.1 Ref.6 Ref.7 Ref.9

Subunit structure

Interacts with ULK3; inactivating the protein kinase activity of ULK3. Interacts with RAB23 By similarity. May form homodimers. Part of a DNA-bound corepressor complex containing SAP18, GLI1 and SIN3. Part of a complex containing CTNNB1. Binds BTRC, GLI2, GLI3, SAP18 and STK36. Binds both free and DNA-bound GLI1. Interacts with KIF7. Interacts with GLI3FL and this interaction regulates the formation of either repressor or activator forms of GLI3. Its association with GLI3FL is regulated by Hh signaling and dissociation of the SUFU-GLI3 interaction requires the presence of the ciliary motor KIF3A. Ref.1 Ref.6 Ref.7 Ref.8 Ref.9

Subcellular location

Cytoplasm. Nucleus Ref.6 Ref.9.

Tissue specificity

Widely expressed in adult and fetal tissues. Ref.1 Ref.3

Sequence similarities

Belongs to the SUFU family.

Ontologies

Keywords
   Cellular componentCytoplasm
Nucleus
   Coding sequence diversityAlternative splicing
   PTMAcetylation
Phosphoprotein
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processcytoplasmic sequestering of transcription factor

Inferred from genetic interaction PubMed 16316410. Source: MGI

determination of left/right symmetry

Inferred from mutant phenotype PubMed 16155214. Source: MGI

heart looping

Inferred from mutant phenotype PubMed 16155214. Source: MGI

negative regulation of protein ubiquitination involved in ubiquitin-dependent protein catabolic process

Inferred from genetic interaction PubMed 19684112. Source: MGI

negative regulation of smoothened signaling pathway

Inferred from direct assay Ref.1. Source: MGI

negative regulation of smoothened signaling pathway involved in dorsal/ventral neural tube patterning

Inferred from mutant phenotype PubMed 19684112. Source: MGI

negative regulation of transcription from RNA polymerase II promoter

Inferred from direct assay Ref.1PubMed 16459298. Source: MGI

neural tube closure

Inferred from mutant phenotype PubMed 16155214. Source: MGI

skin development

Inferred from mutant phenotype PubMed 16459298. Source: MGI

smoothened signaling pathway involved in spinal cord motor neuron cell fate specification

Inferred from mutant phenotype PubMed 19684112. Source: MGI

smoothened signaling pathway involved in ventral spinal cord interneuron specification

Inferred from mutant phenotype PubMed 19684112. Source: MGI

spinal cord dorsal/ventral patterning

Inferred from mutant phenotype PubMed 16459298. Source: MGI

   Cellular_componentcilium

Inferred from direct assay PubMed 16254602. Source: MGI

cytoplasm

Inferred from direct assay Ref.1PubMed 16316410. Source: MGI

nucleus

Inferred from direct assay PubMed 16254602. Source: MGI

primary cilium

Inferred from direct assay PubMed 19684112. Source: MGI

   Molecular_functionbeta-catenin binding

Inferred from direct assay Ref.6. Source: MGI

transcription factor binding

Inferred from sequence orthology PubMed 10564661. Source: MGI

Complete GO annotation...

Alternative products

This entry describes 4 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q9Z0P7-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.
Isoform 2 (identifier: Q9Z0P7-2)

The sequence of this isoform differs from the canonical sequence as follows:
     62-106: Missing.
     252-252: Q → QQ
Note: No experimental confirmation available.
Isoform 3 (identifier: Q9Z0P7-3)

The sequence of this isoform differs from the canonical sequence as follows:
     252-252: Q → QQ
     433-484: ILLTEEFVEK...PDVVFDSPLH → VRRSLSSFSSSSCSSLAACPPLPHHPKDRPLWLPC
Isoform 4 (identifier: Q9Z0P7-4)

Also known as: SU(FU)-XL;

The sequence of this isoform differs from the canonical sequence as follows:
     252-252: Q → QQ

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 484484Suppressor of fused homolog
PRO_0000072303

Amino acid modifications

Modified residue3031N6-acetyllysine By similarity
Modified residue4811Phosphoserine By similarity

Natural variations

Alternative sequence62 – 10645Missing in isoform 2.
VSP_013281
Alternative sequence2521Q → QQ in isoform 2, isoform 3 and isoform 4.
VSP_013282
Alternative sequence433 – 48452ILLTE…DSPLH → VRRSLSSFSSSSCSSLAACP PLPHHPKDRPLWLPC in isoform 3.
VSP_013283

Experimental info

Sequence conflict791S → C in AAF61412. Ref.1
Sequence conflict2201L → Q in AAF61412. Ref.1

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified May 1, 1999. Version 1.
Checksum: 6ED5666BC4D4AFB5

FASTA48453,957
        10         20         30         40         50         60 
MAELRPSVAP GPAAPPASGP SAPPAFASLF PPGLHAIYGE CRRLYPDQPN PLQVTAIVKY 

        70         80         90        100        110        120 
WLGGPDPLDY VSMYRNMGSP SANIPEHWHY ISFGLSDLYG DNRVHEFTGT DGPSGFGFEL 

       130        140        150        160        170        180 
TFRLKRETGE SAPPTWPAEL MQGLARYVFQ SENTFCSGDH VSWHSPLDNS ESRIQHMLLT 

       190        200        210        220        230        240 
EDPQMQPVRT PFGVVTFLQI VGVCTEELHS AQQWNGQGIL ELLRTVPIAG GPWLITDMRR 

       250        260        270        280        290        300 
GETIFEIDPH LQERVDKGIE TDGSNLSGVS AKCAWDDLSR PPEDEEDSRS ICLGTQPRRL 

       310        320        330        340        350        360 
SGKDTEQIRE TLRRGLEINS KPVLPPINSQ RQNGLTHDRA PSRKDSLGSD SSTAIIPHEL 

       370        380        390        400        410        420 
IRTRQLESVH LKFNQESGAL IPLCLRGRLL HGRHFTYKSI TGDMAITFVS TGVEGAFATE 

       430        440        450        460        470        480 
EHPYAAHGPW LQILLTEEFV EKMLEDLEDL TSPEEFKLPK EYSWPEKKLK VSILPDVVFD 


SPLH 

« Hide

Isoform 2 [UniParc].

Checksum: 1C36B0D3382C1F95
Show »

FASTA44048,994
Isoform 3 [UniParc].

Checksum: 98B0F3F09CA85B8A
Show »

FASTA46851,759
Isoform 4 (SU(FU)-XL) [UniParc].

Checksum: F9387714357EC8E4
Show »

FASTA48554,085

References

« Hide 'large scale' references
[1]"Mouse suppressor of fused is a negative regulator of sonic hedgehog signaling and alters the subcellular distribution of Gli1."
Ding Q., Fukami S., Meng X., Nishizaki Y., Zhang X., Sasaki H., Dlugosz A., Nakafuku M., Hui C.
Curr. Biol. 9:1119-1122(1999) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 4), FUNCTION, INTERACTION WITH GLI1; GLI2 AND GLI3, TISSUE SPECIFICITY.
[2]"Suppressor of fused gene involved in hedgehog signal transduction in Drosophila melanogaster is conserved in mammals."
Delattre M., Briand S., Paces-Fessy M., Blanchet-Tournier M.-F.
Dev. Genes Evol. 209:294-300(1999) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
Tissue: Embryo.
[3]"Genomic organization and embryonic expression of suppressor of fused, a candidate gene for the split-hand/split-foot malformation type 3."
Grimm T., Teglund S., Tackels D., Sangiorgi E., Gurrieri F., Schwartz C., Toftgaard R.
FEBS Lett. 505:13-17(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] (ISOFORM 1), TISSUE SPECIFICITY.
Strain: 129/SvJ and C57BL/6J.
Tissue: Mammary gland and Testis.
[4]"The transcriptional landscape of the mammalian genome."
Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J. expand/collapse author list , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 2 AND 3).
Strain: C57BL/6J.
Tissue: Cerebellum and Testis.
[5]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
Strain: C57BL/6.
Tissue: Brain.
[6]"Suppressor of fused negatively regulates beta-catenin signaling."
Meng X., Poon R., Zhang X., Cheah A., Ding Q., Hui C.-C., Alman B.
J. Biol. Chem. 276:40113-40119(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, SUBCELLULAR LOCATION, IDENTIFICATION IN A COMPLEX WITH CTNNB1.
[7]"Suppressor of fused represses Gli-mediated transcription by recruiting the SAP18-mSin3 corepressor complex."
Cheng S.Y., Bishop J.M.
Proc. Natl. Acad. Sci. U.S.A. 99:5442-5447(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, INTERACTION WITH SAP18, IDENTIFICATION IN A DNA-BOUND SIN3 COREPRESSOR COMPLEX.
[8]"The mammalian Cos2 homolog Kif7 plays an essential role in modulating Hh signal transduction during development."
Endoh-Yamagami S., Evangelista M., Wilson D., Wen X., Theunissen J.W., Phamluong K., Davis M., Scales S.J., Solloway M.J., de Sauvage F.J., Peterson A.S.
Curr. Biol. 19:1320-1326(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: INTERACTION WITH KIF7.
[9]"The output of Hedgehog signaling is controlled by the dynamic association between Suppressor of Fused and the Gli proteins."
Humke E.W., Dorn K.V., Milenkovic L., Scott M.P., Rohatgi R.
Genes Dev. 24:670-682(2010) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, SUBCELLULAR LOCATION, INTERACTION WITH GLI3.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AF134893 mRNA. Translation: AAF61412.1.
AJ131692 mRNA. Translation: CAB38081.1.
AJ308625 mRNA. Translation: CAC34257.1.
AJ308626 mRNA. Translation: CAC34258.1.
AJ308627 expand/collapse EMBL AC list , AJ308628, AJ308629, AJ308630, AJ308632, AJ308634, AJ308635, AJ308633, AJ308631 Genomic DNA. Translation: CAC34271.1.
AK015885 mRNA. Translation: BAB30017.1.
AK047603 mRNA. Translation: BAC33095.1.
BC048168 mRNA. Translation: AAH48168.1.
BC056997 mRNA. Translation: AAH56997.1.
RefSeqNP_001020562.1. NM_001025391.2.
NP_056567.2. NM_015752.3.
UniGeneMm.41210.

3D structure databases

ProteinModelPortalQ9Z0P7.
SMRQ9Z0P7. Positions 27-262.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid204881. 21 interactions.
IntActQ9Z0P7. 18 interactions.

PTM databases

PhosphoSiteQ9Z0P7.

Proteomic databases

PaxDbQ9Z0P7.
PRIDEQ9Z0P7.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000039922; ENSMUSP00000049109; ENSMUSG00000025231. [Q9Z0P7-1]
ENSMUST00000111867; ENSMUSP00000107498; ENSMUSG00000025231. [Q9Z0P7-4]
ENSMUST00000120778; ENSMUSP00000112653; ENSMUSG00000025231.
GeneID24069.
KEGGmmu:24069.
UCSCuc008htn.1. mouse. [Q9Z0P7-2]
uc008hto.1. mouse. [Q9Z0P7-3]
uc008htq.1. mouse. [Q9Z0P7-1]

Organism-specific databases

CTD51684.
MGIMGI:1345643. Sufu.

Phylogenomic databases

eggNOGNOG72477.
GeneTreeENSGT00390000009747.
HOGENOMHOG000007864.
HOVERGENHBG061539.
KOK06229.
OMAGDTAITF.
OrthoDBEOG7RV9FV.
PhylomeDBQ9Z0P7.
TreeFamTF324548.

Gene expression databases

ArrayExpressQ9Z0P7.
BgeeQ9Z0P7.
CleanExMM_SUFU.
GenevestigatorQ9Z0P7.

Family and domain databases

InterProIPR020941. SUFU-like_domain.
IPR024314. SUFU_C.
IPR007768. Suppressor_of_fused.
IPR016591. Suppressor_of_fused_euk.
[Graphical view]
PANTHERPTHR10928. PTHR10928. 1 hit.
PfamPF05076. SUFU. 1 hit.
PF12470. SUFU_C. 1 hit.
[Graphical view]
PIRSFPIRSF011844. Suppressor_of_fused_protein. 1 hit.
ProtoNetSearch...

Other

NextBio304049.
PROQ9Z0P7.
SOURCESearch...

Entry information

Entry nameSUFU_MOUSE
AccessionPrimary (citable) accession number: Q9Z0P7
Secondary accession number(s): Q8C8B4 expand/collapse secondary AC list , Q99JG0, Q9D521, Q9JLU1
Entry history
Integrated into UniProtKB/Swiss-Prot: March 29, 2005
Last sequence update: May 1, 1999
Last modified: April 16, 2014
This is version 100 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot