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Protein

Ubiquitin recognition factor in ER-associated degradation protein 1

Gene

UFD1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Essential component of the ubiquitin-dependent proteolytic pathway which degrades ubiquitin fusion proteins. The ternary complex containing UFD1, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. The NPLOC4-UFD1-VCP complex regulates spindle disassembly at the end of mitosis and is necessary for the formation of a closed nuclear envelope. It may be involved in the development of some ectoderm-derived structures (By similarity). Acts as a negative regulator of type I interferon production via the complex formed with VCP and NPLOC4, which binds to DDX58/RIG-I and recruits RNF125 to promote ubiquitination and degradation of DDX58/RIG-I (PubMed:26471729).By similarity1 Publication

Pathwayi: proteasomal ubiquitin-dependent pathway

This protein is involved in the pathway proteasomal ubiquitin-dependent pathway, which is part of Protein degradation.By similarity
View all proteins of this organism that are known to be involved in the pathway proteasomal ubiquitin-dependent pathway and in Protein degradation.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Biological processUbl conjugation pathway

Enzyme and pathway databases

ReactomeiR-HSA-110320 Translesion Synthesis by POLH
R-HSA-5689880 Ub-specific processing proteases
SIGNORiQ92890
UniPathwayi
UPA00144

Protein family/group databases

MoonDBiQ92890 Predicted

Names & Taxonomyi

Protein namesi
Recommended name:
Ubiquitin recognition factor in ER-associated degradation protein 1Imported
Alternative name(s):
Ubiquitin fusion degradation protein 1
Short name:
UB fusion protein 1
Gene namesi
Name:UFD1Imported
Synonyms:UFD1L
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 22

Organism-specific databases

EuPathDBiHostDB:ENSG00000070010.18
HGNCiHGNC:12520 UFD1
MIMi601754 gene
neXtProtiNX_Q92890

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

Organism-specific databases

DisGeNETi7353
MalaCardsiUFD1
OpenTargetsiENSG00000070010
Orphaneti567 22q11.2 deletion syndrome
PharmGKBiPA37167

Polymorphism and mutation databases

BioMutaiUFD1L
DMDMi160332310

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001949841 – 307Ubiquitin recognition factor in ER-associated degradation protein 1Add BLAST307

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1
Modified residuei129PhosphoserineCombined sources1
Modified residuei231PhosphoserineCombined sources1
Modified residuei245PhosphoserineCombined sources1
Modified residuei247PhosphoserineCombined sources1
Modified residuei299PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ92890
PaxDbiQ92890
PeptideAtlasiQ92890
PRIDEiQ92890
ProteomicsDBi75576
75577 [Q92890-1]

2D gel databases

OGPiQ92890

PTM databases

iPTMnetiQ92890
PhosphoSitePlusiQ92890

Expressioni

Tissue specificityi

Found in adult heart, skeletal muscle and pancreas, and in fetal liver and kidney.

Gene expression databases

BgeeiENSG00000070010 Expressed in 229 organ(s), highest expression level in chorionic villus
CleanExiHS_UFD1L
ExpressionAtlasiQ92890 baseline and differential
GenevisibleiQ92890 HS

Organism-specific databases

HPAiHPA030287
HPA030288
HPA073425

Interactioni

Subunit structurei

Heterodimer with NPLOC4, this heterodimer binds VCP and inhibits Golgi membrane fusion (PubMed:11574150, PubMed:26471729). Interacts with USP13 (PubMed:21571647). Interacts with ZFAND2B; probably through VCP (PubMed:24160817).4 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi113200, 103 interactors
ComplexPortaliCPX-137 VCP-NPL4-UFD1 AAA ATPase complex
CORUMiQ92890
DIPiDIP-45954N
IntActiQ92890, 32 interactors
MINTiQ92890
STRINGi9606.ENSP00000263202

Structurei

Secondary structure

1307
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliQ92890
SMRiQ92890
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ92890

Family & Domainsi

Sequence similaritiesi

Belongs to the UFD1 family.Curated

Phylogenomic databases

eggNOGiKOG1816 Eukaryota
COG5140 LUCA
GeneTreeiENSGT00390000002408
HOGENOMiHOG000212737
HOVERGENiHBG001266
InParanoidiQ92890
KOiK14016
OMAiPNYDFKI
OrthoDBiEOG091G0KO7
PhylomeDBiQ92890
TreeFamiTF314581

Family and domain databases

InterProiView protein in InterPro
IPR004854 Ufd1-like
PANTHERiPTHR12555 PTHR12555, 1 hit
PfamiView protein in Pfam
PF03152 UFD1, 1 hit

Sequences (3+)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 4 potential isoforms that are computationally mapped.Show allAlign All

Isoform Short (identifier: Q92890-2) [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
MFSFNMFDHP IPRVFQNRFS TQYRCFSVSM LAGPNDRSDV EKGGKIIMPP
60 70 80 90 100
SALDQLSRLN ITYPMLFKLT NKNSDRMTHC GVLEFVADEG ICYLPHWMMQ
110 120 130 140 150
NLLLEEGGLV QVESVNLQVA TYSKFQPQSP DFLDITNPKA VLENALRNFA
160 170 180 190 200
CLTTGDVIAI NYNEKIYELR VMETKPDKAV SIIECDMNVD FDAPLGYKEP
210 220 230 240 250
ERQVQHEEST EGEADHSGYA GELGFRAFSG SGNRLDGKKK GVEPSPSPIK
260 270 280 290 300
PGDIKRGIPN YEFKLGKITF IRNSRPLVKK VEEDEAGGRF VAFSGEGQSL

RKKGRKP
Note: Major isoform.
Length:307
Mass (Da):34,500
Last modified:November 13, 2007 - v3
Checksum:iFC69860002C042C6
GO
Isoform Long (identifier: Q92890-1) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     106-106: E → EDGLVQLETVNLQVATYSKSKFCYLPHWMMQNLLLEE

Show »
Length:343
Mass (Da):38,725
Checksum:i94D55B70E7F5A27F
GO
Isoform 3 (identifier: Q92890-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     267-307: Missing.

Note: No experimental confirmation available.
Show »
Length:266
Mass (Da):29,901
Checksum:i8CE8E9AE3BF71ED6
GO

Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
C9IZG3C9IZG3_HUMAN
Ubiquitin recognition factor in ER-...
UFD1
190Annotation score:
C9J6N9C9J6N9_HUMAN
Ubiquitin recognition factor in ER-...
UFD1
135Annotation score:
C9J7C8C9J7C8_HUMAN
Ubiquitin recognition factor in ER-...
UFD1
69Annotation score:
C9JNP9C9JNP9_HUMAN
Ubiquitin recognition factor in ER-...
UFD1
159Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti25C → R in AK225877 (Ref. 4) Curated1
Sequence conflicti33G → W in AAD08720 (PubMed:9063746).Curated1
Sequence conflicti183I → H in AAD08720 (PubMed:9063746).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_052436130P → A1 PublicationCorresponds to variant dbSNP:rs17744624Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_006707106E → EDGLVQLETVNLQVATYSKS KFCYLPHWMMQNLLLEE in isoform Long. 1 Publication1
Alternative sequenceiVSP_045044267 – 307Missing in isoform 3. 1 PublicationAdd BLAST41

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U64444 mRNA Translation: AAD08720.1
AF141201 mRNA Translation: AAD28788.1
CR456607 mRNA Translation: CAG30493.1
AK225877 mRNA No translation available.
AC000068 Genomic DNA No translation available.
AC000087 Genomic DNA No translation available.
BC001049 mRNA Translation: AAH01049.1
BC005087 mRNA Translation: AAH05087.1
CCDSiCCDS13761.1 [Q92890-2]
CCDS33600.2 [Q92890-3]
RefSeqiNP_001030324.2, NM_001035247.2 [Q92890-3]
NP_005650.2, NM_005659.6 [Q92890-2]
UniGeneiHs.474213

Genome annotation databases

EnsembliENST00000263202; ENSP00000263202; ENSG00000070010 [Q92890-2]
ENST00000399523; ENSP00000382439; ENSG00000070010 [Q92890-3]
GeneIDi7353
KEGGihsa:7353
UCSCiuc002zpm.3 human [Q92890-2]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U64444 mRNA Translation: AAD08720.1
AF141201 mRNA Translation: AAD28788.1
CR456607 mRNA Translation: CAG30493.1
AK225877 mRNA No translation available.
AC000068 Genomic DNA No translation available.
AC000087 Genomic DNA No translation available.
BC001049 mRNA Translation: AAH01049.1
BC005087 mRNA Translation: AAH05087.1
CCDSiCCDS13761.1 [Q92890-2]
CCDS33600.2 [Q92890-3]
RefSeqiNP_001030324.2, NM_001035247.2 [Q92890-3]
NP_005650.2, NM_005659.6 [Q92890-2]
UniGeneiHs.474213

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2YUJNMR-A11-193[»]
5B6CX-ray1.55B225-235[»]
5C1BX-ray3.08U/V221-241[»]
ProteinModelPortaliQ92890
SMRiQ92890
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113200, 103 interactors
ComplexPortaliCPX-137 VCP-NPL4-UFD1 AAA ATPase complex
CORUMiQ92890
DIPiDIP-45954N
IntActiQ92890, 32 interactors
MINTiQ92890
STRINGi9606.ENSP00000263202

Protein family/group databases

MoonDBiQ92890 Predicted

PTM databases

iPTMnetiQ92890
PhosphoSitePlusiQ92890

Polymorphism and mutation databases

BioMutaiUFD1L
DMDMi160332310

2D gel databases

OGPiQ92890

Proteomic databases

EPDiQ92890
PaxDbiQ92890
PeptideAtlasiQ92890
PRIDEiQ92890
ProteomicsDBi75576
75577 [Q92890-1]

Protocols and materials databases

DNASUi7353
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000263202; ENSP00000263202; ENSG00000070010 [Q92890-2]
ENST00000399523; ENSP00000382439; ENSG00000070010 [Q92890-3]
GeneIDi7353
KEGGihsa:7353
UCSCiuc002zpm.3 human [Q92890-2]

Organism-specific databases

CTDi7353
DisGeNETi7353
EuPathDBiHostDB:ENSG00000070010.18
GeneCardsiUFD1
HGNCiHGNC:12520 UFD1
HPAiHPA030287
HPA030288
HPA073425
MalaCardsiUFD1
MIMi601754 gene
neXtProtiNX_Q92890
OpenTargetsiENSG00000070010
Orphaneti567 22q11.2 deletion syndrome
PharmGKBiPA37167
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1816 Eukaryota
COG5140 LUCA
GeneTreeiENSGT00390000002408
HOGENOMiHOG000212737
HOVERGENiHBG001266
InParanoidiQ92890
KOiK14016
OMAiPNYDFKI
OrthoDBiEOG091G0KO7
PhylomeDBiQ92890
TreeFamiTF314581

Enzyme and pathway databases

UniPathwayi
UPA00144

ReactomeiR-HSA-110320 Translesion Synthesis by POLH
R-HSA-5689880 Ub-specific processing proteases
SIGNORiQ92890

Miscellaneous databases

ChiTaRSiUFD1L human
EvolutionaryTraceiQ92890
GeneWikiiUFD1L
GenomeRNAii7353
PROiPR:Q92890
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000070010 Expressed in 229 organ(s), highest expression level in chorionic villus
CleanExiHS_UFD1L
ExpressionAtlasiQ92890 baseline and differential
GenevisibleiQ92890 HS

Family and domain databases

InterProiView protein in InterPro
IPR004854 Ufd1-like
PANTHERiPTHR12555 PTHR12555, 1 hit
PfamiView protein in Pfam
PF03152 UFD1, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiUFD1_HUMAN
AccessioniPrimary (citable) accession number: Q92890
Secondary accession number(s): A8MW31, Q9Y5N0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 13, 2007
Last modified: November 7, 2018
This is version 157 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. Human chromosome 22
    Human chromosome 22: entries, gene names and cross-references to MIM
  7. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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