Literature associating DNAJB11 and Gaucher's disease
DNAJB11 [ENSP00000414398]
DnaJ homolog subfamily B member 11; 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 . May help recruiting HSPA5 and other chaperones to the substrate. Stimulates HSPA5 ATPase activity . It is necessary for maturation and correct trafficking of PKD1 .
Synonyms: DNAJB11, DNAJB11p, hDNAJB11, Q9UBS4, Q9UBS4p ...