DNAJC3

This gene encodes a protein with multiple tetratricopeptide repeat (TPR) motifs as well as the highly conserved J domain found in DNAJ chaperone family members. It is a member of the tetratricopeptide repeat family of proteins and acts as an inhibitor of the interferon-induced, dsRNA-activated protein kinase (PKR). [provided by RefSeq, Jul 2010]

DnaJ Heat Shock Protein Family (Hsp40) Member C3

Involved in the unfolded protein response (UPR) during endoplasmic reticulum (ER) stress. Acts as a negative regulator of the EIF2AK4/GCN2 kinase activity by preventing the phosphorylation of eIF-2-alpha at 'Ser-52' and hence attenuating general protein synthesis under ER stress, hypothermic and amino acid starving stress conditions (By similarity).

Co-chaperone of HSPA8/HSC70, it stimulates its ATPase activity. May inhibit both the autophosphorylation of EIF2AK2/PKR and the ability of EIF2AK2 to catalyze phosphorylation of the EIF2A. May inhibit EIF2AK3/PERK activity.

Cellular response to cold Source: UniProtKB
Defense response to virus Source: UniProtKB-KW
Negative regulation of apoptotic process Source: ParkinsonsUK-UCL
Negative regulation of endoplasmic reticulum stress-induced eIF2 alpha phosphorylation Source: ParkinsonsUK-UCL
Positive regulation of translation initiation in response to endoplasmic reticulum stress Source: UniProtKB
Protein folding in endoplasmic reticulum Source: GO_Central
Proteolysis involved in cellular protein catabolic process Source: Ensembl
Response to unfolded protein Source: UniProtKB-KW

Endoplasmic reticulum

Ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus (ACPHD):
A disease characterized by juvenile-onset diabetes and neurodegeneration, resulting in ataxia, upper-motor-neuron damage, peripheral neuropathy, hearing loss, and cerebral atrophy.