UniProtKB - Q9UBS4 (DJB11_HUMAN)
DnaJ homolog subfamily B member 11
DNAJB11
Functioni
As a co-chaperone for HSPA5 it is required for proper folding, trafficking or degradation of proteins (PubMed:10827079, PubMed:15525676, PubMed:29706351).
Binds directly to both unfolded proteins that are substrates for ERAD and nascent unfolded peptide chains, but dissociates from the HSPA5-unfolded protein complex before folding is completed (PubMed:15525676).
May help recruiting HSPA5 and other chaperones to the substrate. Stimulates HSPA5 ATPase activity (PubMed:10827079).
It is necessary for maturation and correct trafficking of PKD1 (PubMed:29706351).
3 PublicationsCaution
GO - Molecular functioni
- misfolded protein binding Source: FlyBase
- signaling receptor binding Source: Ensembl
- unfolded protein binding Source: Ensembl
GO - Biological processi
- mRNA modification Source: Ensembl
- negative regulation of neurogenesis Source: Ensembl
- positive regulation of ATPase activity Source: AgBase
- protein folding Source: InterPro
- protein maturation Source: UniProtKB
Keywordsi
Molecular function | Chaperone |
Enzyme and pathway databases
PathwayCommonsi | Q9UBS4 |
Reactomei | R-HSA-381038, XBP1(S) activates chaperone genes |
SignaLinki | Q9UBS4 |
Names & Taxonomyi
Protein namesi | Recommended name: DnaJ homolog subfamily B member 11Alternative name(s): APOBEC1-binding protein 2 Short name: ABBP-2 DnaJ protein homolog 9 ER-associated DNAJ ER-associated Hsp40 co-chaperone Endoplasmic reticulum DNA J domain-containing protein 3 Short name: ER-resident protein ERdj3 Short name: ERdj3 Short name: ERj3p HEDJ Human DnaJ protein 9 Short name: hDj-9 PWP1-interacting protein 4 |
Gene namesi | Name:DNAJB11 Synonyms:EDJ, ERJ3, HDJ9 ORF Names:PSEC0121, UNQ537/PRO1080 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:14889, DNAJB11 |
MIMi | 611341, gene |
neXtProti | NX_Q9UBS4 |
VEuPathDBi | HostDB:ENSG00000090520 |
Subcellular locationi
Endoplasmic reticulum
- Endoplasmic reticulum lumen 4 Publications
Note: Associated with the ER membrane in a C-terminally epitope-tagged construct.
Endoplasmic reticulum
- endoplasmic reticulum Source: AgBase
- endoplasmic reticulum chaperone complex Source: Ensembl
- endoplasmic reticulum lumen Source: Reactome
Extracellular region or secreted
- extracellular space Source: Ensembl
Nucleus
- nucleus Source: Ensembl
Other locations
- chaperone complex Source: FlyBase
- membrane Source: UniProtKB
Keywords - Cellular componenti
Endoplasmic reticulumPathology & Biotechi
Involvement in diseasei
Polycystic kidney disease 6 with or without polycystic liver disease (PKD6)1 Publication
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_081002 | 54 | P → R in PKD6. 1 PublicationCorresponds to variant dbSNP:rs1553849919EnsemblClinVar. | 1 | |
Natural variantiVAR_081003 | 77 | L → P in PKD6. 1 PublicationCorresponds to variant dbSNP:rs1553850185EnsemblClinVar. | 1 | |
Natural variantiVAR_081004 | 206 – 358 | Missing in PKD6. 1 PublicationAdd BLAST | 153 |
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 53 | H → Q: Loss of HSPA5-binding, but no effect on interaction with denatured substrates. 1 Publication | 1 | |
Mutagenesisi | 169 | C → S: Drastic loss of interaction with denatured substrates. 1 Publication | 1 | |
Mutagenesisi | 171 | C → S: Drastic loss of interaction with denatured substrates. 1 Publication | 1 | |
Mutagenesisi | 193 | C → S: Drastic loss of interaction with denatured substrates. 1 Publication | 1 | |
Mutagenesisi | 196 | C → S: Drastic loss of interaction with denatured substrates. 1 Publication | 1 |
Keywords - Diseasei
Disease variantOrganism-specific databases
DisGeNETi | 51726 |
GeneReviewsi | DNAJB11 |
MalaCardsi | DNAJB11 |
MIMi | 618061, phenotype |
OpenTargetsi | ENSG00000090520 |
Orphaneti | 730, Autosomal dominant polycystic kidney disease |
PharmGKBi | PA27413 |
Miscellaneous databases
Pharosi | Q9UBS4, Tbio |
Genetic variation databases
BioMutai | DNAJB11 |
DMDMi | 18203497 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 22 | By similarityAdd BLAST | 22 | |
ChainiPRO_0000007260 | 23 – 358 | DnaJ homolog subfamily B member 11Add BLAST | 336 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 188 | PhosphothreonineCombined sources | 1 | |
Glycosylationi | 261 | N-linked (GlcNAc...) asparagine1 Publication | 1 |
Post-translational modificationi
Keywords - PTMi
Disulfide bond, Glycoprotein, PhosphoproteinProteomic databases
EPDi | Q9UBS4 |
jPOSTi | Q9UBS4 |
MassIVEi | Q9UBS4 |
MaxQBi | Q9UBS4 |
PaxDbi | Q9UBS4 |
PeptideAtlasi | Q9UBS4 |
PRIDEi | Q9UBS4 |
ProteomicsDBi | 84046 |
TopDownProteomicsi | Q9UBS4 |
2D gel databases
OGPi | Q9UBS4 |
REPRODUCTION-2DPAGEi | IPI00008454 |
PTM databases
GlyConnecti | 1185, 1 N-Linked glycan (1 site) |
GlyGeni | Q9UBS4, 2 sites, 1 N-linked glycan (1 site), 1 O-linked glycan (1 site) |
iPTMneti | Q9UBS4 |
PhosphoSitePlusi | Q9UBS4 |
Expressioni
Tissue specificityi
Inductioni
Gene expression databases
Bgeei | ENSG00000090520, Expressed in tendon of biceps brachii and 216 other tissues |
Genevisiblei | Q9UBS4, HS |
Organism-specific databases
HPAi | ENSG00000090520, Low tissue specificity |
Interactioni
Subunit structurei
Part of a large chaperone multiprotein complex comprising DNAJB11, HSP90B1, HSPA5, HYOU, PDIA2, PDIA4, PDIA6, PPIB, SDF2L1, UGGT1 and very small amounts of ERP29, but not, or at very low levels, CALR nor CANX. Binds to denatured substrates in an ATP-independent manner.
Interacts via the J domain with HSPA5 in an ATP-dependent manner.
3 PublicationsBinary interactionsi
Q9UBS4
With | #Exp. | IntAct |
---|---|---|
HSPA5 [P11021] | 4 | EBI-713113,EBI-354921 |
HTN3 [P15516] | 3 | EBI-713113,EBI-738783 |
slrP [Q8ZQQ2] from Salmonella typhimurium (strain LT2 / SGSC1412 / ATCC 700720). | 4 | EBI-713113,EBI-10712653 |
GO - Molecular functioni
- misfolded protein binding Source: FlyBase
- signaling receptor binding Source: Ensembl
- unfolded protein binding Source: Ensembl
Protein-protein interaction databases
BioGRIDi | 119699, 240 interactors |
DIPi | DIP-29678N |
IntActi | Q9UBS4, 95 interactors |
MINTi | Q9UBS4 |
STRINGi | 9606.ENSP00000414398 |
Miscellaneous databases
RNActi | Q9UBS4, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 25 – 90 | JPROSITE-ProRule annotationAdd BLAST | 66 |
Keywords - Domaini
SignalPhylogenomic databases
eggNOGi | KOG0713, Eukaryota |
GeneTreei | ENSGT00940000155792 |
HOGENOMi | CLU_017633_0_0_1 |
InParanoidi | Q9UBS4 |
OMAi | THLDGHI |
OrthoDBi | 687505at2759 |
PhylomeDBi | Q9UBS4 |
TreeFami | TF105144 |
Family and domain databases
CDDi | cd06257, DnaJ, 1 hit |
Gene3Di | 1.10.287.110, 1 hit |
InterProi | View protein in InterPro IPR002939, DnaJ_C IPR001623, DnaJ_domain IPR018253, DnaJ_domain_CS IPR008971, HSP40/DnaJ_pept-bd IPR036869, J_dom_sf |
Pfami | View protein in Pfam PF00226, DnaJ, 1 hit PF01556, DnaJ_C, 1 hit |
PRINTSi | PR00625, JDOMAIN |
SMARTi | View protein in SMART SM00271, DnaJ, 1 hit |
SUPFAMi | SSF46565, SSF46565, 1 hit SSF49493, SSF49493, 2 hits |
PROSITEi | View protein in PROSITE PS00636, DNAJ_1, 1 hit PS50076, DNAJ_2, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All
10 20 30 40 50
MAPQNLSTFC LLLLYLIGAV IAGRDFYKIL GVPRSASIKD IKKAYRKLAL
60 70 80 90 100
QLHPDRNPDD PQAQEKFQDL GAAYEVLSDS EKRKQYDTYG EEGLKDGHQS
110 120 130 140 150
SHGDIFSHFF GDFGFMFGGT PRQQDRNIPR GSDIIVDLEV TLEEVYAGNF
160 170 180 190 200
VEVVRNKPVA RQAPGKRKCN CRQEMRTTQL GPGRFQMTQE VVCDECPNVK
210 220 230 240 250
LVNEERTLEV EIEPGVRDGM EYPFIGEGEP HVDGEPGDLR FRIKVVKHPI
260 270 280 290 300
FERRGDDLYT NVTISLVESL VGFEMDITHL DGHKVHISRD KITRPGAKLW
310 320 330 340 350
KKGEGLPNFD NNNIKGSLII TFDVDFPKEQ LTEEAREGIK QLLKQGSVQK
VYNGLQGY
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketA0A7P0T9E8 | A0A7P0T9E8_HUMAN | DnaJ homolog subfamily B member 11 | DNAJB11 | 162 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 247 | K → R in CAG33377 (Ref. 8) Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_081002 | 54 | P → R in PKD6. 1 PublicationCorresponds to variant dbSNP:rs1553849919EnsemblClinVar. | 1 | |
Natural variantiVAR_081003 | 77 | L → P in PKD6. 1 PublicationCorresponds to variant dbSNP:rs1553850185EnsemblClinVar. | 1 | |
Natural variantiVAR_081004 | 206 – 358 | Missing in PKD6. 1 PublicationAdd BLAST | 153 | |
Natural variantiVAR_016092 | 264 | I → V2 PublicationsCorresponds to variant dbSNP:rs8147Ensembl. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB028859 mRNA Translation: BAA88307.1 AF228505 mRNA Translation: AAF61711.1 AJ250137 mRNA Translation: CAB65118.1 AF277317 mRNA Translation: AAK69110.1 AY359043 mRNA Translation: AAQ89402.1 AK075300 mRNA Translation: BAC11533.1 AK075430 mRNA Translation: BAC11617.1 BT007063 mRNA Translation: AAP35712.1 CR457096 mRNA Translation: CAG33377.1 CH471052 Genomic DNA Translation: EAW78190.1 BC001144 mRNA Translation: AAH01144.1 |
CCDSi | CCDS3277.1 |
PIRi | T52073 |
RefSeqi | NP_057390.1, NM_016306.5 |
Genome annotation databases
Ensembli | ENST00000265028; ENSP00000265028; ENSG00000090520 ENST00000439351; ENSP00000414398; ENSG00000090520 |
GeneIDi | 51726 |
KEGGi | hsa:51726 |
MANE-Selecti | ENST00000265028.8; ENSP00000265028.3; NM_016306.6; NP_057390.1 |
UCSCi | uc003fqi.4, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB028859 mRNA Translation: BAA88307.1 AF228505 mRNA Translation: AAF61711.1 AJ250137 mRNA Translation: CAB65118.1 AF277317 mRNA Translation: AAK69110.1 AY359043 mRNA Translation: AAQ89402.1 AK075300 mRNA Translation: BAC11533.1 AK075430 mRNA Translation: BAC11617.1 BT007063 mRNA Translation: AAP35712.1 CR457096 mRNA Translation: CAG33377.1 CH471052 Genomic DNA Translation: EAW78190.1 BC001144 mRNA Translation: AAH01144.1 |
CCDSi | CCDS3277.1 |
PIRi | T52073 |
RefSeqi | NP_057390.1, NM_016306.5 |
3D structure databases
SMRi | Q9UBS4 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 119699, 240 interactors |
DIPi | DIP-29678N |
IntActi | Q9UBS4, 95 interactors |
MINTi | Q9UBS4 |
STRINGi | 9606.ENSP00000414398 |
PTM databases
GlyConnecti | 1185, 1 N-Linked glycan (1 site) |
GlyGeni | Q9UBS4, 2 sites, 1 N-linked glycan (1 site), 1 O-linked glycan (1 site) |
iPTMneti | Q9UBS4 |
PhosphoSitePlusi | Q9UBS4 |
Genetic variation databases
BioMutai | DNAJB11 |
DMDMi | 18203497 |
2D gel databases
OGPi | Q9UBS4 |
REPRODUCTION-2DPAGEi | IPI00008454 |
Proteomic databases
EPDi | Q9UBS4 |
jPOSTi | Q9UBS4 |
MassIVEi | Q9UBS4 |
MaxQBi | Q9UBS4 |
PaxDbi | Q9UBS4 |
PeptideAtlasi | Q9UBS4 |
PRIDEi | Q9UBS4 |
ProteomicsDBi | 84046 |
TopDownProteomicsi | Q9UBS4 |
Protocols and materials databases
Antibodypediai | 2272, 247 antibodies from 29 providers |
DNASUi | 51726 |
Genome annotation databases
Ensembli | ENST00000265028; ENSP00000265028; ENSG00000090520 ENST00000439351; ENSP00000414398; ENSG00000090520 |
GeneIDi | 51726 |
KEGGi | hsa:51726 |
MANE-Selecti | ENST00000265028.8; ENSP00000265028.3; NM_016306.6; NP_057390.1 |
UCSCi | uc003fqi.4, human |
Organism-specific databases
CTDi | 51726 |
DisGeNETi | 51726 |
GeneCardsi | DNAJB11 |
GeneReviewsi | DNAJB11 |
HGNCi | HGNC:14889, DNAJB11 |
HPAi | ENSG00000090520, Low tissue specificity |
MalaCardsi | DNAJB11 |
MIMi | 611341, gene 618061, phenotype |
neXtProti | NX_Q9UBS4 |
OpenTargetsi | ENSG00000090520 |
Orphaneti | 730, Autosomal dominant polycystic kidney disease |
PharmGKBi | PA27413 |
VEuPathDBi | HostDB:ENSG00000090520 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0713, Eukaryota |
GeneTreei | ENSGT00940000155792 |
HOGENOMi | CLU_017633_0_0_1 |
InParanoidi | Q9UBS4 |
OMAi | THLDGHI |
OrthoDBi | 687505at2759 |
PhylomeDBi | Q9UBS4 |
TreeFami | TF105144 |
Enzyme and pathway databases
PathwayCommonsi | Q9UBS4 |
Reactomei | R-HSA-381038, XBP1(S) activates chaperone genes |
SignaLinki | Q9UBS4 |
Miscellaneous databases
BioGRID-ORCSi | 51726, 48 hits in 1059 CRISPR screens |
ChiTaRSi | DNAJB11, human |
GeneWikii | DNAJB11 |
GenomeRNAii | 51726 |
Pharosi | Q9UBS4, Tbio |
PROi | PR:Q9UBS4 |
RNActi | Q9UBS4, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000090520, Expressed in tendon of biceps brachii and 216 other tissues |
Genevisiblei | Q9UBS4, HS |
Family and domain databases
CDDi | cd06257, DnaJ, 1 hit |
Gene3Di | 1.10.287.110, 1 hit |
InterProi | View protein in InterPro IPR002939, DnaJ_C IPR001623, DnaJ_domain IPR018253, DnaJ_domain_CS IPR008971, HSP40/DnaJ_pept-bd IPR036869, J_dom_sf |
Pfami | View protein in Pfam PF00226, DnaJ, 1 hit PF01556, DnaJ_C, 1 hit |
PRINTSi | PR00625, JDOMAIN |
SMARTi | View protein in SMART SM00271, DnaJ, 1 hit |
SUPFAMi | SSF46565, SSF46565, 1 hit SSF49493, SSF49493, 2 hits |
PROSITEi | View protein in PROSITE PS00636, DNAJ_1, 1 hit PS50076, DNAJ_2, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | DJB11_HUMAN | |
Accessioni | Q9UBS4Primary (citable) accession number: Q9UBS4 Secondary accession number(s): Q542Y5 Q96JC6 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | October 18, 2001 |
Last sequence update: | May 1, 2000 | |
Last modified: | February 23, 2022 | |
This is version 194 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Reference proteomeDocuments
- Human chromosome 3
Human chromosome 3: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot