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Protein

Suppressor of fused homolog

Gene

Sufu

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Negative regulator in the hedgehog/smoothened signaling pathway (PubMed:16155214, PubMed:16459298). Down-regulates GLI1-mediated transactivation of target genes (PubMed:11960000). Part of a corepressor complex that acts on DNA-bound GLI1 (PubMed:11960000). May also act by linking GLI1 to BTRC and thereby targeting GLI1 to degradation by the proteasome (By similarity). Sequesters GLI1, GLI2 and GLI3 in the cytoplasm, this effect is overcome by binding of STK36 to both SUFU and a GLI protein (PubMed:10531011, PubMed:16459298). Negative regulator of beta-catenin signaling (PubMed:11477086). Regulates the formation of either the repressor form (GLI3R) or the activator form (GLI3A) of the full-length form of GLI3 (GLI3FL) (PubMed:10531011, PubMed:20360384). GLI3FL is complexed with SUFU in the cytoplasm and is maintained in a neutral state (PubMed:10531011, PubMed:20360384). Without the Hh signal, the SUFU-GLI3 complex is recruited to cilia, leading to the efficient processing of GLI3FL into GLI3R (PubMed:10531011, PubMed:20360384). 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) (PubMed:10531011, PubMed:20360384). Required for normal embryonic development (PubMed:16155214, PubMed:16459298). Required for the proper formation of hair follicles and the control of epidermal differentiation (PubMed:16155214, PubMed:16459298, PubMed:23034632).By similarity7 Publications

GO - Molecular functioni

  • beta-catenin binding Source: MGI
  • protein kinase binding Source: MGI
  • transcription factor binding Source: MGI

GO - Biological processi

Keywordsi

Molecular functionDevelopmental protein

Enzyme and pathway databases

ReactomeiR-MMU-5610780 Degradation of GLI1 by the proteasome
R-MMU-5610785 GLI3 is processed to GLI3R by the proteasome
R-MMU-5610787 Hedgehog 'off' state
R-MMU-5632684 Hedgehog 'on' state

Names & Taxonomyi

Protein namesi
Recommended name:
Suppressor of fused homolog1 Publication
Gene namesi
Name:Sufu1 PublicationImported
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 19

Organism-specific databases

MGIiMGI:1345643 Sufu

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Nucleus

Pathology & Biotechi

Disruption phenotypei

Complete embryonic lethality at about 10.5 dpc due to defects in neural tube closure, abnormal somites and abnormal heart looping (PubMed:16155214, PubMed:16459298). Heterozygous mice are born at the expected Mendelian rate and are fertile. After 1.5 years, they develop a skin phenotype characterized by ventral alopecia, increased pigmentation, with papules and nodules on paws and tail (PubMed:16459298).2 Publications

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000723031 – 484Suppressor of fused homologAdd BLAST484

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki257Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
Modified residuei301PhosphoserineBy similarity1
Modified residuei303N6-acetyllysineBy similarity1
Cross-linki321Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)By similarity
Modified residuei342PhosphoserineBy similarity1
Modified residuei346PhosphoserineCombined sources1
Modified residuei352PhosphoserineBy similarity1
Modified residuei353PhosphothreonineBy similarity1
Modified residuei481PhosphoserineBy similarity1

Post-translational modificationi

Polyubiquitinated at Lys-257 by the SCF(FBXL17) complex, leading to its subsequent degradation and allowing the release of GLI1 for proper hedgehog/smoothened signal transduction. Ubiquitination is impaired by phosphorylation at Ser-342, Ser-346, Ser-352 and Thr-353.By similarity
Phosphorylation at Ser-342, Ser-346, Ser-352 and Thr-353 prevents ubiquitination by the SCF(FBXL17) complex.By similarity

Keywords - PTMi

Acetylation, Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

PaxDbiQ9Z0P7
PeptideAtlasiQ9Z0P7
PRIDEiQ9Z0P7

PTM databases

iPTMnetiQ9Z0P7
PhosphoSitePlusiQ9Z0P7

Expressioni

Tissue specificityi

Widely expressed in adult and fetal tissues.2 Publications

Gene expression databases

BgeeiENSMUSG00000025231 Expressed in 281 organ(s), highest expression level in ear vesicle
CleanExiMM_SUFU
ExpressionAtlasiQ9Z0P7 baseline and differential
GenevisibleiQ9Z0P7 MM

Interactioni

Subunit structurei

May form homodimers (By similarity). Interacts with ULK3; inactivating the protein kinase activity of ULK3. Interacts with RAB23 (By similarity). Part of a DNA-bound corepressor complex containing SAP18, GLI1 and SIN3 (PubMed:11960000). Part of a complex containing CTNNB1 (PubMed:11477086). Binds BTRC, GLI2, GLI3, SAP18 and STK36 (PubMed:20360384, PubMed:23034632). Binds both free and DNA-bound GLI1 (PubMed:10531011). Interacts with KIF7 (PubMed:19592253). Interacts with GLI3FL and this interaction regulates the formation of either repressor or activator forms of GLI3 (PubMed:20360384). Its association with GLI3FL is regulated by Hh signaling and dissociation of the SUFU-GLI3 interaction requires the presence of the ciliary motor KIF3A (PubMed:20360384).By similarity6 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi204881, 22 interactors
ComplexPortaliCPX-147 GLI1-SUFU complex
CPX-149 GLI2-SUFU complex
CPX-151 GLI3-SUFU complex
CORUMiQ9Z0P7
IntActiQ9Z0P7, 21 interactors
STRINGi10090.ENSMUSP00000107498

Structurei

3D structure databases

ProteinModelPortaliQ9Z0P7
SMRiQ9Z0P7
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni279 – 360Intrinsically disorderedBy similarityAdd BLAST82

Sequence similaritiesi

Belongs to the SUFU family.Curated

Phylogenomic databases

eggNOGiENOG410IFRJ Eukaryota
ENOG410XRUC LUCA
GeneTreeiENSGT00390000009747
HOGENOMiHOG000007864
HOVERGENiHBG061539
InParanoidiQ9Z0P7
KOiK06229
OMAiGDNIPWR
PhylomeDBiQ9Z0P7
TreeFamiTF324548

Family and domain databases

Gene3Di3.30.1360.230, 1 hit
InterProiView protein in InterPro
IPR020941 SUFU-like_domain
IPR024314 SUFU_C
IPR038489 SUFU_C_sf
IPR037181 SUFU_N
IPR007768 Suppressor_of_fused
IPR016591 Suppressor_of_fused_euk
PANTHERiPTHR10928 PTHR10928, 1 hit
PfamiView protein in Pfam
PF05076 SUFU, 1 hit
PF12470 SUFU_C, 1 hit
PIRSFiPIRSF011844 Suppressor_of_fused_protein, 1 hit
SUPFAMiSSF103359 SSF103359, 1 hit

Sequences (4+)i

Sequence statusi: Complete.

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 4 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q9Z0P7-1) [UniParc]FASTAAdd to basket

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.

« Hide
        10         20         30         40         50
MAELRPSVAP GPAAPPASGP SAPPAFASLF PPGLHAIYGE CRRLYPDQPN
60 70 80 90 100
PLQVTAIVKY WLGGPDPLDY VSMYRNMGSP SANIPEHWHY ISFGLSDLYG
110 120 130 140 150
DNRVHEFTGT DGPSGFGFEL TFRLKRETGE SAPPTWPAEL MQGLARYVFQ
160 170 180 190 200
SENTFCSGDH VSWHSPLDNS ESRIQHMLLT EDPQMQPVRT PFGVVTFLQI
210 220 230 240 250
VGVCTEELHS AQQWNGQGIL ELLRTVPIAG GPWLITDMRR GETIFEIDPH
260 270 280 290 300
LQERVDKGIE TDGSNLSGVS AKCAWDDLSR PPEDEEDSRS ICLGTQPRRL
310 320 330 340 350
SGKDTEQIRE TLRRGLEINS KPVLPPINSQ RQNGLTHDRA PSRKDSLGSD
360 370 380 390 400
SSTAIIPHEL IRTRQLESVH LKFNQESGAL IPLCLRGRLL HGRHFTYKSI
410 420 430 440 450
TGDMAITFVS TGVEGAFATE EHPYAAHGPW LQILLTEEFV EKMLEDLEDL
460 470 480
TSPEEFKLPK EYSWPEKKLK VSILPDVVFD SPLH
Length:484
Mass (Da):53,957
Last modified:May 1, 1999 - v1
Checksum:i6ED5666BC4D4AFB5
GO
Isoform 2 (identifier: Q9Z0P7-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     62-106: Missing.
     252-252: Q → QQ

Note: No experimental confirmation available.
Show »
Length:440
Mass (Da):48,994
Checksum:i1C36B0D3382C1F95
GO
Isoform 3 (identifier: Q9Z0P7-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     252-252: Q → QQ
     433-484: ILLTEEFVEK...PDVVFDSPLH → VRRSLSSFSSSSCSSLAACPPLPHHPKDRPLWLPC

Show »
Length:468
Mass (Da):51,759
Checksum:i98B0F3F09CA85B8A
GO
Isoform 4 (identifier: Q9Z0P7-4) [UniParc]FASTAAdd to basket
Also known as: SU(FU)-XL

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

Show »
Length:485
Mass (Da):54,085
Checksum:iF9387714357EC8E4
GO

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D3Z7B6D3Z7B6_MOUSE
Suppressor of fused homolog
Sufu
467Annotation score:
A0A0R4J1M0A0A0R4J1M0_MOUSE
Suppressor of fused homolog
Sufu
297Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti79S → C in AAF61412 (PubMed:10531011).Curated1
Sequence conflicti220L → Q in AAF61412 (PubMed:10531011).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_01328162 – 106Missing in isoform 2. 1 PublicationAdd BLAST45
Alternative sequenceiVSP_013282252Q → QQ in isoform 2, isoform 3 and isoform 4. 2 Publications1
Alternative sequenceiVSP_013283433 – 484ILLTE…DSPLH → VRRSLSSFSSSSCSSLAACP PLPHHPKDRPLWLPC in isoform 3. 1 PublicationAdd BLAST52

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF134893 mRNA Translation: AAF61412.1
AJ131692 mRNA Translation: CAB38081.1
AJ308625 mRNA Translation: CAC34257.1
AJ308626 mRNA Translation: CAC34258.1
AJ308627
, 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
CCDSiCCDS29879.1 [Q9Z0P7-1]
CCDS38008.1 [Q9Z0P7-4]
RefSeqiNP_001020562.1, NM_001025391.2 [Q9Z0P7-1]
NP_056567.2, NM_015752.3 [Q9Z0P7-4]
UniGeneiMm.41210

Genome annotation databases

EnsembliENSMUST00000039922; ENSMUSP00000049109; ENSMUSG00000025231 [Q9Z0P7-1]
ENSMUST00000111867; ENSMUSP00000107498; ENSMUSG00000025231 [Q9Z0P7-4]
GeneIDi24069
KEGGimmu:24069
UCSCiuc008htn.2 mouse [Q9Z0P7-2]
uc008hto.2 mouse [Q9Z0P7-3]
uc008htq.2 mouse [Q9Z0P7-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF134893 mRNA Translation: AAF61412.1
AJ131692 mRNA Translation: CAB38081.1
AJ308625 mRNA Translation: CAC34257.1
AJ308626 mRNA Translation: CAC34258.1
AJ308627
, 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
CCDSiCCDS29879.1 [Q9Z0P7-1]
CCDS38008.1 [Q9Z0P7-4]
RefSeqiNP_001020562.1, NM_001025391.2 [Q9Z0P7-1]
NP_056567.2, NM_015752.3 [Q9Z0P7-4]
UniGeneiMm.41210

3D structure databases

ProteinModelPortaliQ9Z0P7
SMRiQ9Z0P7
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi204881, 22 interactors
ComplexPortaliCPX-147 GLI1-SUFU complex
CPX-149 GLI2-SUFU complex
CPX-151 GLI3-SUFU complex
CORUMiQ9Z0P7
IntActiQ9Z0P7, 21 interactors
STRINGi10090.ENSMUSP00000107498

PTM databases

iPTMnetiQ9Z0P7
PhosphoSitePlusiQ9Z0P7

Proteomic databases

PaxDbiQ9Z0P7
PeptideAtlasiQ9Z0P7
PRIDEiQ9Z0P7

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000039922; ENSMUSP00000049109; ENSMUSG00000025231 [Q9Z0P7-1]
ENSMUST00000111867; ENSMUSP00000107498; ENSMUSG00000025231 [Q9Z0P7-4]
GeneIDi24069
KEGGimmu:24069
UCSCiuc008htn.2 mouse [Q9Z0P7-2]
uc008hto.2 mouse [Q9Z0P7-3]
uc008htq.2 mouse [Q9Z0P7-1]

Organism-specific databases

CTDi51684
MGIiMGI:1345643 Sufu

Phylogenomic databases

eggNOGiENOG410IFRJ Eukaryota
ENOG410XRUC LUCA
GeneTreeiENSGT00390000009747
HOGENOMiHOG000007864
HOVERGENiHBG061539
InParanoidiQ9Z0P7
KOiK06229
OMAiGDNIPWR
PhylomeDBiQ9Z0P7
TreeFamiTF324548

Enzyme and pathway databases

ReactomeiR-MMU-5610780 Degradation of GLI1 by the proteasome
R-MMU-5610785 GLI3 is processed to GLI3R by the proteasome
R-MMU-5610787 Hedgehog 'off' state
R-MMU-5632684 Hedgehog 'on' state

Miscellaneous databases

PROiPR:Q9Z0P7
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000025231 Expressed in 281 organ(s), highest expression level in ear vesicle
CleanExiMM_SUFU
ExpressionAtlasiQ9Z0P7 baseline and differential
GenevisibleiQ9Z0P7 MM

Family and domain databases

Gene3Di3.30.1360.230, 1 hit
InterProiView protein in InterPro
IPR020941 SUFU-like_domain
IPR024314 SUFU_C
IPR038489 SUFU_C_sf
IPR037181 SUFU_N
IPR007768 Suppressor_of_fused
IPR016591 Suppressor_of_fused_euk
PANTHERiPTHR10928 PTHR10928, 1 hit
PfamiView protein in Pfam
PF05076 SUFU, 1 hit
PF12470 SUFU_C, 1 hit
PIRSFiPIRSF011844 Suppressor_of_fused_protein, 1 hit
SUPFAMiSSF103359 SSF103359, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiSUFU_MOUSE
AccessioniPrimary (citable) accession number: Q9Z0P7
Secondary accession number(s): Q8C8B4
, Q99JG0, Q9D521, Q9JLU1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 29, 2005
Last sequence update: May 1, 1999
Last modified: November 7, 2018
This is version 142 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
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