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Protein

UBX domain-containing protein 1

Gene

UBXN1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Ubiquitin-binding protein that interacts with the BRCA1-BARD1 heterodimer, and regulates its activity. Specifically binds 'Lys-6'-linked polyubiquitin chains. Interaction with autoubiquitinated BRCA1, leads to inhibit the E3 ubiquitin-protein ligase activity of the BRCA1-BARD1 heterodimer. Component of a complex required to couple deglycosylation and proteasome-mediated degradation of misfolded proteins in the endoplasmic reticulum that are retrotranslocated in the cytosol.

GO - Molecular functioni

  1. ATPase binding Source: UniProtKB
  2. K6-linked polyubiquitin binding Source: UniProtKB
  3. polyubiquitin binding Source: UniProtKB
  4. ubiquitin binding Source: BHF-UCL
  5. ubiquitin protein ligase binding Source: BHF-UCL

GO - Biological processi

  1. negative regulation of proteasomal ubiquitin-dependent protein catabolic process Source: UniProtKB
  2. negative regulation of protein ubiquitination Source: UniProtKB
  3. proteasome-mediated ubiquitin-dependent protein catabolic process Source: UniProtKB
Complete GO annotation...

Names & Taxonomyi

Protein namesi
Recommended name:
UBX domain-containing protein 1
Alternative name(s):
SAPK substrate protein 1
UBA/UBX 33.3 kDa protein
Gene namesi
Name:UBXN1
Synonyms:SAKS1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 11

Organism-specific databases

HGNCiHGNC:18402. UBXN1.

Subcellular locationi

Cytoplasm By similarity

GO - Cellular componenti

  1. Cdc48p-Npl4p-Ufd1p AAA ATPase complex Source: BHF-UCL
  2. cytoplasm Source: UniProtKB
  3. dendrite Source: Ensembl
  4. neuronal cell body Source: Ensembl
  5. nucleus Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi13 – 131M → T: Abolishes binding to 'Lys-6'-linked polyubiquitin chains and ability to inhibit E3 ubiquitin-protein ligase activity of the BRCA1-BARD1 heterodimer. 1 Publication
Mutagenesisi219 – 2191R → A: Does not affect binding to 'Lys-6'-linked polyubiquitin chains and ability to inhibit E3 ubiquitin-protein ligase activity of the BRCA1-BARD1 heterodimer. 1 Publication

Organism-specific databases

PharmGKBiPA162408336.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methioninei1 – 11Removed1 Publication
Chaini2 – 297296UBX domain-containing protein 1PRO_0000211023Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylalanine1 Publication
Modified residuei199 – 1991Phosphoserine1 Publication
Modified residuei200 – 2001Phosphoserine; by MAPK121 Publication
Modified residuei207 – 2071Phosphothreonine1 Publication
Modified residuei270 – 2701Phosphoserine2 Publications

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiQ04323.
PaxDbiQ04323.
PeptideAtlasiQ04323.
PRIDEiQ04323.

PTM databases

PhosphoSiteiQ04323.

Expressioni

Gene expression databases

BgeeiQ04323.
CleanExiHS_UBXN1.
ExpressionAtlasiQ04323. baseline and differential.
GenevestigatoriQ04323.

Organism-specific databases

HPAiHPA012669.

Interactioni

Subunit structurei

Component of a complex required to couple retrotranslocation, ubiquitination and deglycosylation composed of NGLY1, SAKS1, AMFR, VCP and RAD23B. Interacts with HOMER2 (By similarity). Interacts directly with VCP. Interacts with BRCA1 and BARD1; interaction takes place when BRCA1 is not autoubiquitinated but is strongly enhanced in the presence of autoubiquitinated BRCA1.By similarity3 Publications

Protein-protein interaction databases

BioGridi119239. 107 interactions.
DIPiDIP-29467N.
IntActiQ04323. 30 interactions.
STRINGi9606.ENSP00000294119.

Structurei

3D structure databases

ProteinModelPortaliQ04323.
SMRiQ04323. Positions 2-55.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini2 – 4241UBAPROSITE-ProRule annotationAdd
BLAST
Domaini211 – 29383UBXPROSITE-ProRule annotationAdd
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni43 – 297255Interaction with BRCA1Add
BLAST

Coiled coil

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Coiled coili87 – 17286Sequence AnalysisAdd
BLAST

Domaini

The UBA domain specifically recognizes and binds 'Lys-6'-linked polyubiquitin chains.1 Publication

Sequence similaritiesi

Contains 1 UBA domain.PROSITE-ProRule annotation
Contains 1 UBX domain.PROSITE-ProRule annotation

Keywords - Domaini

Coiled coil

Phylogenomic databases

eggNOGiCOG5207.
GeneTreeiENSGT00530000063500.
HOGENOMiHOG000188321.
HOVERGENiHBG082310.
InParanoidiQ04323.
OMAiQWLEDNQ.
OrthoDBiEOG7SR4PG.
PhylomeDBiQ04323.
TreeFamiTF313944.

Family and domain databases

InterProiIPR009060. UBA-like.
IPR015940. UBA/transl_elong_EF1B_N_euk.
IPR000449. UBA/Ts_N.
IPR029071. Ubiquitin-rel_dom.
IPR001012. UBX_dom.
[Graphical view]
PfamiPF00627. UBA. 1 hit.
PF00789. UBX. 1 hit.
[Graphical view]
SMARTiSM00165. UBA. 1 hit.
SM00166. UBX. 1 hit.
[Graphical view]
SUPFAMiSSF46934. SSF46934. 1 hit.
SSF54236. SSF54236. 1 hit.
PROSITEiPS50030. UBA. 1 hit.
PS50033. UBX. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q04323-1) [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
MAELTALESL IEMGFPRGRA EKALALTGNQ GIEAAMDWLM EHEDDPDVDE
60 70 80 90 100
PLETPLGHIL GREPTSSEQG GLEGSGSAAG EGKPALSEEE RQEQTKRMLE
110 120 130 140 150
LVAQKQRERE EREEREALER ERQRRRQGQE LSAARQRLQE DEMRRAAEER
160 170 180 190 200
RREKAEELAA RQRVREKIER DKAERAKKYG GSVGSQPPPV APEPGPVPSS
210 220 230 240 250
PSQEPPTKRE YDQCRIQVRL PDGTSLTQTF RAREQLAAVR LYVELHRGEE
260 270 280 290
LGGGQDPVQL LSGFPRRAFS EADMERPLQE LGLVPSAVLI VAKKCPS
Length:297
Mass (Da):33,325
Last modified:May 16, 2003 - v2
Checksum:iE4E0C8BBAC93F2BB
GO
Isoform 2 (identifier: Q04323-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     283-297: LVPSAVLIVAKKCPS → MAARLETRTRNWGSREACLGKGGMQREGAL

Note: No experimental confirmation available.

Show »
Length:312
Mass (Da):35,107
Checksum:i75C94CB67C03EB78
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti76 – 761G → A in AAA36396 (Ref. 1) Curated
Sequence conflicti148 – 1481E → AE in AAA36396 (Ref. 1) Curated
Sequence conflicti154 – 1541K → N in AAA36396 (Ref. 1) Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti56 – 561L → F.
Corresponds to variant rs11543359 [ dbSNP | Ensembl ].
VAR_057370

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei283 – 29715LVPSA…KKCPS → MAARLETRTRNWGSREACLG KGGMQREGAL in isoform 2. 1 PublicationVSP_020367Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M68864 mRNA. Translation: AAA36396.1.
BC000902 mRNA. Translation: AAH00902.1.
BC001372 mRNA. Translation: AAH01372.1.
BC040129 mRNA. Translation: AAH40129.1.
CCDSiCCDS66105.1. [Q04323-1]
CCDS8029.1. [Q04323-2]
PIRiS27965.
RefSeqiNP_001273006.1. NM_001286077.1. [Q04323-1]
NP_001273007.1. NM_001286078.1.
NP_056937.2. NM_015853.4. [Q04323-2]
XP_005274090.1. XM_005274033.2. [Q04323-2]
UniGeneiHs.351296.
Hs.731509.

Genome annotation databases

EnsembliENST00000294119; ENSP00000294119; ENSG00000162191. [Q04323-2]
ENST00000301935; ENSP00000303991; ENSG00000162191. [Q04323-1]
GeneIDi51035.
KEGGihsa:51035.
UCSCiuc001nuj.3. human. [Q04323-2]
uc001nul.2. human. [Q04323-1]

Polymorphism databases

DMDMi30923268.

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M68864 mRNA. Translation: AAA36396.1.
BC000902 mRNA. Translation: AAH00902.1.
BC001372 mRNA. Translation: AAH01372.1.
BC040129 mRNA. Translation: AAH40129.1.
CCDSiCCDS66105.1. [Q04323-1]
CCDS8029.1. [Q04323-2]
PIRiS27965.
RefSeqiNP_001273006.1. NM_001286077.1. [Q04323-1]
NP_001273007.1. NM_001286078.1.
NP_056937.2. NM_015853.4. [Q04323-2]
XP_005274090.1. XM_005274033.2. [Q04323-2]
UniGeneiHs.351296.
Hs.731509.

3D structure databases

ProteinModelPortaliQ04323.
SMRiQ04323. Positions 2-55.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi119239. 107 interactions.
DIPiDIP-29467N.
IntActiQ04323. 30 interactions.
STRINGi9606.ENSP00000294119.

PTM databases

PhosphoSiteiQ04323.

Polymorphism databases

DMDMi30923268.

Proteomic databases

MaxQBiQ04323.
PaxDbiQ04323.
PeptideAtlasiQ04323.
PRIDEiQ04323.

Protocols and materials databases

DNASUi51035.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000294119; ENSP00000294119; ENSG00000162191. [Q04323-2]
ENST00000301935; ENSP00000303991; ENSG00000162191. [Q04323-1]
GeneIDi51035.
KEGGihsa:51035.
UCSCiuc001nuj.3. human. [Q04323-2]
uc001nul.2. human. [Q04323-1]

Organism-specific databases

CTDi51035.
GeneCardsiGC11M062489.
H-InvDBHIX0009719.
HGNCiHGNC:18402. UBXN1.
HPAiHPA012669.
neXtProtiNX_Q04323.
PharmGKBiPA162408336.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG5207.
GeneTreeiENSGT00530000063500.
HOGENOMiHOG000188321.
HOVERGENiHBG082310.
InParanoidiQ04323.
OMAiQWLEDNQ.
OrthoDBiEOG7SR4PG.
PhylomeDBiQ04323.
TreeFamiTF313944.

Miscellaneous databases

ChiTaRSiUBXN1. human.
GenomeRNAii51035.
NextBioi53592.
PROiQ04323.

Gene expression databases

BgeeiQ04323.
CleanExiHS_UBXN1.
ExpressionAtlasiQ04323. baseline and differential.
GenevestigatoriQ04323.

Family and domain databases

InterProiIPR009060. UBA-like.
IPR015940. UBA/transl_elong_EF1B_N_euk.
IPR000449. UBA/Ts_N.
IPR029071. Ubiquitin-rel_dom.
IPR001012. UBX_dom.
[Graphical view]
PfamiPF00627. UBA. 1 hit.
PF00789. UBX. 1 hit.
[Graphical view]
SMARTiSM00165. UBA. 1 hit.
SM00166. UBX. 1 hit.
[Graphical view]
SUPFAMiSSF46934. SSF46934. 1 hit.
SSF54236. SSF54236. 1 hit.
PROSITEiPS50030. UBA. 1 hit.
PS50033. UBX. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. Pollard K.M.
    Submitted (JUN-1991) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
  2. "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] (ISOFORMS 1 AND 2).
    Tissue: Colon, Placenta and Skin.
  3. "A novel UBA and UBX domain protein that binds polyubiquitin and VCP and is a substrate for SAPKs."
    McNeill H., Knebel A., Arthur J.S., Cuenda A., Cohen P.
    Biochem. J. 384:391-400(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 220-231 AND 276-285, PHOSPHORYLATION AT SER-200, INTERACTION WITH VCP.
  4. Lubec G., Chen W.-Q., Sun Y.
    Submitted (DEC-2008) to UniProtKB
    Cited for: PROTEIN SEQUENCE OF 220-231, IDENTIFICATION BY MASS SPECTROMETRY.
    Tissue: Fetal brain cortex.
  5. "UBXD7 binds multiple ubiquitin ligases and implicates p97 in HIF1alpha turnover."
    Alexandru G., Graumann J., Smith G.T., Kolawa N.J., Fang R., Deshaies R.J.
    Cell 134:804-816(2008) [PubMed] [Europe PMC] [Abstract]
    Cited for: UBIQUITIN-BINDING, INTERACTION WITH VCP.
  6. Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-199, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  7. "Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."
    Gauci S., Helbig A.O., Slijper M., Krijgsveld J., Heck A.J., Mohammed S.
    Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT ALA-2, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS], CLEAVAGE OF INITIATOR METHIONINE [LARGE SCALE ANALYSIS].
  8. "Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."
    Mayya V., Lundgren D.H., Hwang S.-I., Rezaul K., Wu L., Eng J.K., Rodionov V., Han D.K.
    Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-270, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Leukemic T-cell.
  9. "The UBXN1 protein associates with autoubiquitinated forms of the BRCA1 tumor suppressor and inhibits its enzymatic function."
    Wu-Baer F., Ludwig T., Baer R.
    Mol. Cell. Biol. 30:2787-2798(2010) [PubMed] [Europe PMC] [Abstract]
    Cited for: UBIQUITIN-BINDING, DOMAIN UBA, INTERACTION WITH BRCA1 AND BARD1, MUTAGENESIS OF MET-13 AND ARG-219.
  10. "Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."
    Olsen J.V., Vermeulen M., Santamaria A., Kumar C., Miller M.L., Jensen L.J., Gnad F., Cox J., Jensen T.S., Nigg E.A., Brunak S., Mann M.
    Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  11. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  12. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT THR-207 AND SER-270, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiUBXN1_HUMAN
AccessioniPrimary (citable) accession number: Q04323
Secondary accession number(s): Q9BV93, Q9BVV5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: May 16, 2003
Last modified: March 4, 2015
This is version 120 of the entry and version 2 of the sequence. [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

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
  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. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.