DNAJB11 gene

DnaJ heat shock protein family (Hsp40) member B11

The information on this page was automatically extracted from online scientific databases.

From NCBI Gene:

This gene encodes a soluble glycoprotein of the endoplasmic reticulum (ER) lumen that functions as a co-chaperone of binding immunoglobulin protein, a 70 kilodalton heat shock protein chaperone required for the proper folding and assembly of proteins in the ER. The encoded protein contains a highly conserved J domain of about 70 amino acids with a characteristic His-Pro-Asp (HPD) motif and may regulate the activity of binding immunoglobulin protein by stimulating ATPase activity. [provided by RefSeq, Mar 2014]

From UniProt:

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).

From NCBI Gene:


From UniProt:

Polycystic kidney disease 6 with or without polycystic liver disease (PKD6): 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. [MIM:618061]

Cytogenetic Location: 3q27.3, which is the long (q) arm of chromosome 3 at position 27.3

Molecular Location: base pairs 186,570,720 to 186,585,793 on chromosome 3 (Homo sapiens Updated Annotation Release 109.20200522, GRCh38.p13) (NCBI)

Cytogenetic Location: 3q27.3, which is the long (q) arm of chromosome 3 at position 27.3
  • ABBP-2
  • ABBP2
  • Dj-9
  • DJ9
  • EDJ
  • ERdj3
  • ERj3
  • ERj3p
  • PKD6
  • PRO1080
  • UNQ537