DISEASES

Disease-gene associations mined from literature

Literature on DNAJB9

DNAJB9 [ENSP00000249356]

DnaJ homolog subfamily B member 9; Co-chaperone for Hsp70 protein HSPA5/BiP that acts as a key repressor of the ERN1/IRE1-mediated unfolded protein response (UPR) (By similarity). J domain-containing co-chaperones stimulate the ATPase activity of Hsp70 proteins and are required for efficient substrate recognition by Hsp70 proteins . In the unstressed endoplasmic reticulum, interacts with the luminal region of ERN1/IRE1 and selectively recruits HSPA5/BiP: HSPA5/BiP disrupts the dimerization of the active ERN1/IRE1 luminal region, thereby inactivating ERN1/IRE1 (By similarity). Also involved in endoplasmic reticulum-associated degradation (ERAD) of misfolded proteins. Required for survival of B- cell progenitors and normal antibody production (By similarity).

Synonyms:  DNAJB9,  DNAJB9p,  hDNAJB9,  Q6FIF1,  Q9UBS3 ...

Linkouts:  STRING  Pharos  UniProt  OMIM