DNAJB11
entrez
51726
canonical
uniprot
Q9UBS4
canonical
ensembl
ENSG00000090520
resolver
ensp
ENSP00000265028
resolver
ensp
ENSP00000414398
resolver
ensp
ENSP00000541584
resolver
enst
ENST00000265028
resolver
enst
ENST00000439351
resolver
genesymbol
DNAJB11
resolver
genesymbol_syn
EDJ
resolver
genesymbol_syn
ERJ3
resolver
genesymbol_syn
HDJ9
resolver
hgnc
HGNC:14889
resolver
uniprot
Q542Y5
resolver
uniprot
Q542Y9
resolver
uniprot
Q6IAQ8
resolver
uniprot
Q96JC6
resolver
uniprot_entry
DJB11_HUMAN
resolver
uniprot_sec
Q542Y5
resolver
uniprot_sec
Q542Y9
resolver
uniprot_sec
Q6IAQ8
resolver
uniprot_sec
Q96JC6
resolver
amino_acid_sequence
MAPQNLSTFCLLLLYLIGAVIAGRDFYKILGVPRSASIKDIKKAYRKLALQLHPDRNPDDPQAQEKFQDLGAAYEVLSDSEKRKQYDTYGEEGLKDGHQSSHGDIFSHFFGDFGFMFGGTPRQQDRNIPRGSDIIVDLEVTLEEVYAGNFVEVVRNKPVARQAPGKRKCNCRQEMRTTQLGPGRFQMTQEVVCDECPNVKLVNEERTLEVEIEPGVRDGMEYPFIGEGEPHVDGEPGDLRFRIKVVKHPIFERRGDDLYTNVTISLVESLVGFEMDITHLDGHKVHISRDKITRPGAKLWKKGEGLPNFDNNNIKGSLIITFDVDFPKEQLTEEAREGIKQLLKQGSVQKVYNGLQGY
uniprot
proteins
disease_involvement
DISEASE: Polycystic kidney disease 6 with or without polycystic liver disease (PKD6) [MIM:618061]: A form of polycystic kidney disease, a disorder characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occur in other organs, particularly the liver. PKD6 inheritance is autosomal dominant. {ECO:0000269|PubMed:29706351}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
function
FUNCTION: 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). {ECO:0000269|PubMed:10827079, ECO:0000269|PubMed:15525676, ECO:0000269|PubMed:29706351}.
uniprot
proteins
mass_dalton
40514
uniprot
proteins
mutagenesis
MUTAGEN 53; /note="H->Q: Loss of HSPA5-binding, but no effect on interaction with denatured substrates."; /evidence="ECO:0000269|PubMed:15525676"; MUTAGEN 169; /note="C->S: Drastic loss of interaction with denatured substrates."; /evidence="ECO:0000269|PubMed:17976514"; MUTAGEN 171; /note="C->S: Drastic loss of interaction with denatured substrates."; /evidence="ECO:0000269|PubMed:17976514"; MUTAGEN 193; /note="C->S: Drastic loss of interaction with denatured substrates."; /evidence="ECO:0000269|PubMed:17976514"; MUTAGEN 196; /note="C->S: Drastic loss of interaction with denatured substrates."; /evidence="ECO:0000269|PubMed:17976514
uniprot
proteins
post_translational_modification
PTM: Contains high-mannose Endo H-sensitive carbohydrates.; PTM: Cys-169, Cys-171, Cys-193 and Cys-196 form intramolecular disulfide bonds. The preferential partner for each Cys is not known.; PTM: Thr-188 was reported to be phosphorylated upon DNA damage by ATM or ATR; however as this position has been shown to be in the ER lumen, the in vivo relevance is not proven.
uniprot
proteins
pubmed
10827079
uniprot
proteins
pubmed
11147971
uniprot
proteins
pubmed
11584023
uniprot
proteins
pubmed
12475965
uniprot
proteins
pubmed
12975309
uniprot
proteins
pubmed
15195998
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15525676
uniprot
proteins
pubmed
15544163
uniprot
proteins
pubmed
16303743
uniprot
proteins
pubmed
17525332
uniprot
proteins
pubmed
17976514
uniprot
proteins
pubmed
19159218
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
29706351
uniprot
proteins
sequence_length
358
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Endoplasmic reticulum lumen {ECO:0000269|PubMed:10827079, ECO:0000269|PubMed:15195998, ECO:0000269|PubMed:15525676, ECO:0000269|PubMed:15544163}. Note=Associated with the ER membrane in a C-terminally epitope-tagged construct.
uniprot
proteins