DNAJC10

This gene encodes an endoplasmic reticulum co-chaperone which is part of the endoplasmic reticulum-associated degradation complex involved in recognizing and degrading misfolded proteins. The encoded protein reduces incorrect disulfide bonds in misfolded glycoproteins. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Oct 2012]

Full Name

DnaJ (Hsp40) homolog, subfamily C, member 10

Function

Endoplasmic reticulum disulfide reductase involved both in the correct folding of proteins and degradation of misfolded proteins. Required for efficient folding of proteins in the endoplasmic reticulum by catalyzing the removal of non-native disulfide bonds formed during the folding of proteins, such as LDLR. Also involved in endoplasmic reticulum-associated degradation (ERAD) by reducing incorrect disulfide bonds in misfolded glycoproteins recognized by EDEM1. Interaction with HSPA5 is required its activity, not for the disulfide reductase activity, but to facilitate the release of DNAJC10 from its substrate. Promotes apoptotic signaling pathway in response to endoplasmic reticulum stress.

Biological Process

Intrinsic apoptotic signaling pathway in response to endoplasmic reticulum stress Source: UniProtKB
Negative regulation of protein phosphorylation Source: UniProtKB
Positive regulation of ATPase activity Source: UniProtKB
Protein folding in endoplasmic reticulum Source: UniProtKB
Response to endoplasmic reticulum stress Source: UniProtKB
Ubiquitin-dependent ERAD pathway Source: UniProtKB

Cellular Location

Endoplasmic reticulum lumen