DNAJB11
Description
The DNAJB11 (DnaJ heat shock protein family (Hsp40) member B11) is a protein-coding gene located on chromosome 3.
DnaJ homolog subfamily B member 11 is a protein that in humans is encoded by the DNAJB11 gene.
DNAJB11 acts as a co-chaperone for HSPA5, playing a crucial role in the proper folding, trafficking, and degradation of proteins. It directly binds to unfolded proteins targeted for ER-associated protein degradation (ERAD) and nascent unfolded peptide chains. Notably, DNAJB11 detaches from the HSPA5-unfolded protein complex before folding is fully completed. DNAJB11 may assist in recruiting HSPA5 and other chaperones to the substrate, while also stimulating HSPA5's ATPase activity. Furthermore, DNAJB11 is essential for the maturation and correct trafficking of PKD1.
DNAJB11 is also known as ABBP-2, ABBP2, DJ9, Dj-9, EDJ, ERdj3, ERj3, ERj3p, PKD6, PRO1080, UNQ537.
Associated Diseases
- Autosomal dominant polycystic kidney disease
- Polycystic kidney disease 6 with or without polycystic liver disease
