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Protein

Ubiquilin-4

Gene

UBQLN4

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

Functioni

Plays a role in the regulation of protein degradation via the ubiquitin-proteasome system (UPS). Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding (via UBA domain) to their polyubiquitin chains and by interacting (via ubiquitin-like domain) with the subunits of the proteasome (PubMed:15280365). Plays a role in the regulation of the proteasomal degradation of non-ubiquitinated GJA1 (By similarity). Acts as an adapter protein that recruits UBQLN1 to the autophagy machinery. Mediates the association of UBQLN1 with autophagosomes and the autophagy-related protein LC3 (MAP1LC3A/B/C) and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion (PubMed:23459205).By similarity2 Publications

GO - Molecular functioni

  • identical protein binding Source: IntAct
  • polyubiquitin modification-dependent protein binding Source: UniProtKB

GO - Biological processi

Keywordsi

Biological processAutophagy

Protein family/group databases

TCDBi8.A.52.1.1 the ubiquitin-related protein degradation (upd) family

Names & Taxonomyi

Protein namesi
Recommended name:
Ubiquilin-4
Alternative name(s):
Ataxin-1 interacting ubiquitin-like protein
Short name:
A1Up
Ataxin-1 ubiquitin-like-interacting protein A1U
Connexin43-interacting protein of 75 kDa
Short name:
CIP75
Gene namesi
Name:UBQLN4
Synonyms:C1orf6, CIP75, UBIN
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000160803.7
HGNCiHGNC:1237 UBQLN4
MIMi605440 gene
neXtProtiNX_Q9NRR5

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, Cytoplasmic vesicle, Endoplasmic reticulum, Nucleus

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi55I → A: Loss of interaction with UBQLN1. 1

Organism-specific databases

DisGeNETi56893
OpenTargetsiENSG00000160803
PharmGKBiPA25619

Polymorphism and mutation databases

BioMutaiUBQLN4
DMDMi45476982

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002110151 – 601Ubiquilin-4Add BLAST601

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki23Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Cross-linki62Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei98PhosphoserineCombined sources1
Modified residuei287PhosphothreonineCombined sources1

Post-translational modificationi

Ubiquitinated; this does not lead to proteasomal degradation. May undergo both 'Lys-48'- and 'Lys-63'-linked polyubiquitination.1 Publication

Keywords - PTMi

Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiQ9NRR5
MaxQBiQ9NRR5
PaxDbiQ9NRR5
PeptideAtlasiQ9NRR5
PRIDEiQ9NRR5
ProteomicsDBi82412
82413 [Q9NRR5-2]
TopDownProteomicsiQ9NRR5-2 [Q9NRR5-2]

PTM databases

iPTMnetiQ9NRR5
PhosphoSitePlusiQ9NRR5

Expressioni

Tissue specificityi

Highly expressed in pancreas, kidney, skeletal muscle, heart and throughout the brain, and at lower levels in placenta, lung and liver.1 Publication

Gene expression databases

BgeeiENSG00000160803 Expressed in 191 organ(s), highest expression level in prefrontal cortex
CleanExiHS_UBQLN4
GenevisibleiQ9NRR5 HS

Organism-specific databases

HPAiHPA061797

Interactioni

Subunit structurei

Homooligomer (PubMed:15280365). Binds signal sequences of proteins that are targeted to the endoplasmic reticulum. Interacts (via UBA domain) with GJA1 (not ubiquitinated) and with ubiquitin; both compete for the same binding site. Interacts (via UBA domain) with ubiquitin and with polyubiquitin chains. Interacts with PSMD2 (By similarity). Interacts (via ubiquitin-like domain) with PSMD4, a regulatory subunit of the 26S proteasome. Interacts with ATXN1/SCA1. Interaction with ATXN1 inhibits polyubiquitination of UBQLN4 and interferes with PSMD4 binding. Interacts with HERPUD1. Interacts (via ubiquitin-like domain) with UBQLN1 (via UBA domain). Interacts (via STI1 1 and 2 domains) with MAP1LC3A/B/C. Interacts with UBQLN2.By similarity4 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi121223, 192 interactors
IntActiQ9NRR5, 159 interactors
MINTiQ9NRR5
STRINGi9606.ENSP00000357292

Structurei

3D structure databases

ProteinModelPortaliQ9NRR5
SMRiQ9NRR5
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini13 – 87Ubiquitin-likePROSITE-ProRule annotationAdd BLAST75
Domaini192 – 229STI1 1Sequence analysisAdd BLAST38
Domaini230 – 261STI1 2Sequence analysisAdd BLAST32
Domaini393 – 440STI1 3Sequence analysisAdd BLAST48
Domaini444 – 476STI1 4Sequence analysisAdd BLAST33
Domaini553 – 598UBAPROSITE-ProRule annotationAdd BLAST46

Phylogenomic databases

eggNOGiKOG0010 Eukaryota
COG5272 LUCA
GeneTreeiENSGT00390000005720
HOVERGENiHBG064537
InParanoidiQ9NRR5
KOiK04523
OMAiLVIKTAH
OrthoDBiEOG091G08WB
PhylomeDBiQ9NRR5
TreeFamiTF314412

Family and domain databases

InterProiView protein in InterPro
IPR006636 STI1_HS-bd
IPR015940 UBA
IPR009060 UBA-like_sf
IPR015496 Ubiquilin
IPR029071 Ubiquitin-like_domsf
IPR000626 Ubiquitin_dom
PANTHERiPTHR10677 PTHR10677, 1 hit
PfamiView protein in Pfam
PF00627 UBA, 1 hit
PF00240 ubiquitin, 1 hit
SMARTiView protein in SMART
SM00727 STI1, 4 hits
SM00165 UBA, 1 hit
SM00213 UBQ, 1 hit
SUPFAMiSSF46934 SSF46934, 1 hit
SSF54236 SSF54236, 1 hit
PROSITEiView protein in PROSITE
PS50030 UBA, 1 hit
PS50053 UBIQUITIN_2, 1 hit

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket
Isoform 1 (identifier: Q9NRR5-1) [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
MAEPSGAETR PPIRVTVKTP KDKEEIVICD RASVKEFKEE ISRRFKAQQD
60 70 80 90 100
QLVLIFAGKI LKDGDTLNQH GIKDGLTVHL VIKTPQKAQD PAAATASSPS
110 120 130 140 150
TPDPASAPST TPASPATPAQ PSTSGSASSD AGSGSRRSSG GGPSPGAGEG
160 170 180 190 200
SPSATASILS GFGGILGLGS LGLGSANFME LQQQMQRQLM SNPEMLSQIM
210 220 230 240 250
ENPLVQDMMS NPDLMRHMIM ANPQMQQLME RNPEISHMLN NPELMRQTME
260 270 280 290 300
LARNPAMMQE MMRNQDRALS NLESIPGGYN ALRRMYTDIQ EPMFSAAREQ
310 320 330 340 350
FGNNPFSSLA GNSDSSSSQP LRTENREPLP NPWSPSPPTS QAPGSGGEGT
360 370 380 390 400
GGSGTSQVHP TVSNPFGINA ASLGSGMFNS PEMQALLQQI SENPQLMQNV
410 420 430 440 450
ISAPYMRSMM QTLAQNPDFA AQMMVNVPLF AGNPQLQEQL RLQLPVFLQQ
460 470 480 490 500
MQNPESLSIL TNPRAMQALL QIQQGLQTLQ TEAPGLVPSL GSFGISRTPA
510 520 530 540 550
PSAGSNAGST PEAPTSSPAT PATSSPTGAS SAQQQLMQQM IQLLAGSGNS
560 570 580 590 600
QVQTPEVRFQ QQLEQLNSMG FINREANLQA LIATGGDINA AIERLLGSQL

S
Length:601
Mass (Da):63,853
Last modified:March 15, 2004 - v2
Checksum:iE57B9FFEF90793FE
GO
Isoform 2 (identifier: Q9NRR5-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     93-226: AATASSPSTP...HMIMANPQMQ → PPAAPSLPAA...AAQLHGLHQS
     227-601: Missing.

Note: No experimental confirmation available.
Show »
Length:226
Mass (Da):24,527
Checksum:i69101551F1551A93
GO

Sequence cautioni

The sequence AAF19084 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAH06410 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti188 – 189QL → HV in AAF80171 (PubMed:11001934).Curated2
Sequence conflicti298R → Q in AAF80171 (PubMed:11001934).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_052685495I → M2 PublicationsCorresponds to variant dbSNP:rs2297792Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04118793 – 226AATAS…NPQMQ → PPAAPSLPAADAEPRVTLHP YQSPSHAGIAADPAGTTDLA DRGPWAGTQPWLLWDIPDPS TLSRQQRRVYARGPHFLTSH ASHIFSNRGFQRPAATHAAD DPAFGWKWKLTGADARSEIS AAAGAAQLHGLHQS in isoform 2. 1 PublicationAdd BLAST134
Alternative sequenceiVSP_041188227 – 601Missing in isoform 2. 1 PublicationAdd BLAST375

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF188240 mRNA Translation: AAF80171.1
AK098368 mRNA No translation available.
AK314413 mRNA Translation: BAG37034.1
AL355388 Genomic DNA No translation available.
BC006410 mRNA Translation: AAH06410.1 Different initiation.
BC018403 mRNA Translation: AAH18403.1
BC063841 mRNA Translation: AAH63841.1
AF113544 mRNA Translation: AAF19084.1 Different initiation.
CCDSiCCDS1127.1 [Q9NRR5-1]
RefSeqiNP_001291271.1, NM_001304342.1
NP_064516.2, NM_020131.4 [Q9NRR5-1]
UniGeneiHs.283739

Genome annotation databases

EnsembliENST00000368309; ENSP00000357292; ENSG00000160803 [Q9NRR5-1]
GeneIDi56893
KEGGihsa:56893
UCSCiuc001fna.4 human [Q9NRR5-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF188240 mRNA Translation: AAF80171.1
AK098368 mRNA No translation available.
AK314413 mRNA Translation: BAG37034.1
AL355388 Genomic DNA No translation available.
BC006410 mRNA Translation: AAH06410.1 Different initiation.
BC018403 mRNA Translation: AAH18403.1
BC063841 mRNA Translation: AAH63841.1
AF113544 mRNA Translation: AAF19084.1 Different initiation.
CCDSiCCDS1127.1 [Q9NRR5-1]
RefSeqiNP_001291271.1, NM_001304342.1
NP_064516.2, NM_020131.4 [Q9NRR5-1]
UniGeneiHs.283739

3D structure databases

ProteinModelPortaliQ9NRR5
SMRiQ9NRR5
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi121223, 192 interactors
IntActiQ9NRR5, 159 interactors
MINTiQ9NRR5
STRINGi9606.ENSP00000357292

Protein family/group databases

TCDBi8.A.52.1.1 the ubiquitin-related protein degradation (upd) family

PTM databases

iPTMnetiQ9NRR5
PhosphoSitePlusiQ9NRR5

Polymorphism and mutation databases

BioMutaiUBQLN4
DMDMi45476982

Proteomic databases

EPDiQ9NRR5
MaxQBiQ9NRR5
PaxDbiQ9NRR5
PeptideAtlasiQ9NRR5
PRIDEiQ9NRR5
ProteomicsDBi82412
82413 [Q9NRR5-2]
TopDownProteomicsiQ9NRR5-2 [Q9NRR5-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000368309; ENSP00000357292; ENSG00000160803 [Q9NRR5-1]
GeneIDi56893
KEGGihsa:56893
UCSCiuc001fna.4 human [Q9NRR5-1]

Organism-specific databases

CTDi56893
DisGeNETi56893
EuPathDBiHostDB:ENSG00000160803.7
GeneCardsiUBQLN4
HGNCiHGNC:1237 UBQLN4
HPAiHPA061797
MIMi605440 gene
neXtProtiNX_Q9NRR5
OpenTargetsiENSG00000160803
PharmGKBiPA25619
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0010 Eukaryota
COG5272 LUCA
GeneTreeiENSGT00390000005720
HOVERGENiHBG064537
InParanoidiQ9NRR5
KOiK04523
OMAiLVIKTAH
OrthoDBiEOG091G08WB
PhylomeDBiQ9NRR5
TreeFamiTF314412

Miscellaneous databases

ChiTaRSiUBQLN4 human
GenomeRNAii56893
PROiPR:Q9NRR5
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000160803 Expressed in 191 organ(s), highest expression level in prefrontal cortex
CleanExiHS_UBQLN4
GenevisibleiQ9NRR5 HS

Family and domain databases

InterProiView protein in InterPro
IPR006636 STI1_HS-bd
IPR015940 UBA
IPR009060 UBA-like_sf
IPR015496 Ubiquilin
IPR029071 Ubiquitin-like_domsf
IPR000626 Ubiquitin_dom
PANTHERiPTHR10677 PTHR10677, 1 hit
PfamiView protein in Pfam
PF00627 UBA, 1 hit
PF00240 ubiquitin, 1 hit
SMARTiView protein in SMART
SM00727 STI1, 4 hits
SM00165 UBA, 1 hit
SM00213 UBQ, 1 hit
SUPFAMiSSF46934 SSF46934, 1 hit
SSF54236 SSF54236, 1 hit
PROSITEiView protein in PROSITE
PS50030 UBA, 1 hit
PS50053 UBIQUITIN_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiUBQL4_HUMAN
AccessioniPrimary (citable) accession number: Q9NRR5
Secondary accession number(s): A6ND44
, B2RAY7, Q5VYA0, Q5VYA1, Q9BR98, Q9UHX4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 15, 2004
Last sequence update: March 15, 2004
Last modified: September 12, 2018
This is version 155 of the entry and version 2 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: 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
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