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Protein

UBX domain-containing protein 6

Gene

UBXN6

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

Functioni

May negatively regulate the ATPase activity of VCP, an ATP-driven segregase that associates with different cofactors to control a wide variety of cellular processes (PubMed:26475856). As a cofactor of VCP, it may play a role in the transport of CAV1 to lysosomes for degradation (PubMed:21822278, PubMed:23335559). It may also play a role in endoplasmic reticulum-associated degradation (ERAD) of misfolded proteins (PubMed:19275885). Together with VCP and other cofactors, it may play a role in macroautophagy, regulating for instance the clearance of damaged lysosomes (PubMed:27753622).5 Publications

GO - Biological processi

  • endosome to lysosome transport via multivesicular body sorting pathway Source: UniProtKB
  • ERAD pathway Source: UniProtKB
  • macroautophagy Source: UniProtKB

Keywordsi

Biological processUbl conjugation pathway

Names & Taxonomyi

Protein namesi
Recommended name:
UBX domain-containing protein 6Curated
Alternative name(s):
UBX domain-containing protein 11 Publication
Gene namesi
Name:UBXN6Imported
Synonyms:UBXD11 PublicationImported, UBXDC2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000167671.11.
HGNCiHGNC:14928. UBXN6.

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, Cytoskeleton, Endosome, Lysosome, Membrane, Nucleus

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000167671.
PharmGKBiPA162408446.

Polymorphism and mutation databases

BioMutaiUBXN6.
DMDMi30913412.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002110251 – 441UBX domain-containing protein 6Add BLAST441

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei96PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ9BZV1.
MaxQBiQ9BZV1.
PaxDbiQ9BZV1.
PeptideAtlasiQ9BZV1.
PRIDEiQ9BZV1.

PTM databases

iPTMnetiQ9BZV1.
PhosphoSitePlusiQ9BZV1.

Expressioni

Tissue specificityi

Enhanced expression in testis.

Gene expression databases

BgeeiENSG00000167671.
CleanExiHS_UBXN6.
ExpressionAtlasiQ9BZV1. baseline and differential.
GenevisibleiQ9BZV1. HS.

Organism-specific databases

HPAiHPA040906.
HPA041313.
HPA061872.

Interactioni

Subunit structurei

Interacts with VCP through the PUB domain (via C-terminus) and VIM motif (via N-terminus); the interaction is direct (PubMed:18656546, PubMed:19174149, PubMed:21896481, PubMed:21822278, PubMed:26475856). Forms a ternary complex with CAV1 and VCP (PubMed:21822278). Interacts with SYVN1 (PubMed:18656546). Interacts with HERPUD1 (PubMed:18656546). Interacts with VCPKMT (PubMed:23349634). May interact with DERL1 (PubMed:19275885). Interacts with PLAA, VCP and YOD1; may form a complex involved in macroautophagy (PubMed:27753622). Interacts with LMAN1 (PubMed:22337587).9 Publications

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi123263. 55 interactors.
IntActiQ9BZV1. 18 interactors.
MINTiMINT-3064689.
STRINGi9606.ENSP00000301281.

Structurei

3D structure databases

ProteinModelPortaliQ9BZV1.
SMRiQ9BZV1.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini175 – 244PUBSequence analysisAdd BLAST70
Domaini332 – 408UBXPROSITE-ProRule annotationAdd BLAST77

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni1 – 10Mediates interaction with LMAN11 Publication10
Regioni51 – 63VCP/p97-interacting motif (VIM)1 PublicationAdd BLAST13

Domaini

The UBX domain lacks key residues critical for VCP binding.1 Publication

Phylogenomic databases

eggNOGiKOG2699. Eukaryota.
ENOG410XQQK. LUCA.
GeneTreeiENSGT00900000141106.
HOGENOMiHOG000043787.
HOVERGENiHBG061370.
InParanoidiQ9BZV1.
KOiK14011.
OMAiNLAFNEC.
OrthoDBiEOG091G0EB3.
PhylomeDBiQ9BZV1.
TreeFamiTF314617.

Family and domain databases

InterProiView protein in InterPro
IPR036339. PUB-like_dom_sf.
IPR018997. PUB_domain.
IPR029071. Ubiquitin-like_domsf.
IPR001012. UBX_dom.
PfamiView protein in Pfam
PF09409. PUB. 1 hit.
PF00789. UBX. 1 hit.
SMARTiView protein in SMART
SM00580. PUG. 1 hit.
SM00166. UBX. 1 hit.
SUPFAMiSSF143503. SSF143503. 1 hit.
SSF54236. SSF54236. 1 hit.
PROSITEiView protein in PROSITE
PS50033. UBX. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: Q9BZV1-1) [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
MKKFFQEFKA DIKFKSAGPG QKLKESVGEK AHKEKPNQPA PRPPRQGPTN
60 70 80 90 100
EAQMAAAAAL ARLEQKQSRA WGPTSQDTIR NQVRKELQAE ATVSGSPEAP
110 120 130 140 150
GTNVVSEPRE EGSAHLAVPG VYFTCPLTGA TLRKDQRDAC IKEAILLHFS
160 170 180 190 200
TDPVAASIMK IYTFNKDQDR VKLGVDTIAK YLDNIHLHPE EEKYRKIKLQ
210 220 230 240 250
NKVFQERINC LEGTHEFFEA IGFQKVLLPA QDQEDPEEFY VLSETTLAQP
260 270 280 290 300
QSLERHKEQL LAAEPVRAKL DRQRRVFQPS PLASQFELPG DFFNLTAEEI
310 320 330 340 350
KREQRLRSEA VERLSVLRTK AMREKEEQRG LRKYNYTLLR VRLPDGCLLQ
360 370 380 390 400
GTFYARERLG AVYGFVREAL QSDWLPFELL ASGGQKLSED ENLALNECGL
410 420 430 440
VPSALLTFSW DMAVLEDIKA AGAEPDSILK PELLSAIEKL L
Length:441
Mass (Da):49,754
Last modified:June 1, 2001 - v1
Checksum:iFC06E6ADA1A1036C
GO
Isoform 2 (identifier: Q9BZV1-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-53: Missing.

Show »
Length:388
Mass (Da):43,809
Checksum:i072249F3D9C6FAD8
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06192431A → T. Corresponds to variant dbSNP:rs1127888Ensembl.1
Natural variantiVAR_047821425P → L. Corresponds to variant dbSNP:rs35436704Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0074531 – 53Missing in isoform 2. 2 PublicationsAdd BLAST53

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF272893 mRNA. Translation: AAK13257.1.
AF272894 mRNA. Translation: AAK13258.1.
CH471139 Genomic DNA. Translation: EAW69219.1.
CH471139 Genomic DNA. Translation: EAW69222.1.
BC007414 mRNA. Translation: AAH07414.2.
BC008288 mRNA. Translation: AAH08288.1.
BC017113 mRNA. Translation: AAH17113.1.
CCDSiCCDS12129.1. [Q9BZV1-1]
CCDS54201.1. [Q9BZV1-2]
RefSeqiNP_001164562.1. NM_001171091.1. [Q9BZV1-2]
NP_079517.1. NM_025241.2. [Q9BZV1-1]
UniGeneiHs.435255.

Genome annotation databases

EnsembliENST00000301281; ENSP00000301281; ENSG00000167671. [Q9BZV1-1]
ENST00000394765; ENSP00000378246; ENSG00000167671. [Q9BZV1-2]
GeneIDi80700.
KEGGihsa:80700.
UCSCiuc002mam.3. human. [Q9BZV1-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiUBXN6_HUMAN
AccessioniPrimary (citable) accession number: Q9BZV1
Secondary accession number(s): D6W626
, Q96AH1, Q96IK9, Q9BZV0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 16, 2003
Last sequence update: June 1, 2001
Last modified: November 22, 2017
This is version 133 of the entry and version 1 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

Caution

Was reported to interact with MDM2 and USP7 and to act in a complex with VCP in cooperation with USP7 to promote MDM2 deubiquitination and stabilization. However, the corresponding article has been retracted.1 Publication

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: 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. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot