Q13217 · DNJC3_HUMAN
- ProteinDnaJ homolog subfamily C member 3
- GeneDNAJC3
- StatusUniProtKB reviewed (Swiss-Prot)
- Organism
- Amino acids504 (go to sequence)
- Protein existenceEvidence at protein level
- Annotation score5/5
Function
function
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
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
GO annotations
all annotations | all molecular function | virus receptor activity | dna binding | rna binding | cytoskeletal motor activity | catalytic activity | gtpase activity | structural molecule activity | transporter activity | cytoskeletal protein binding | lipid binding | cyclase activity | antioxidant activity | oxidoreductase activity | transferase activity | hydrolase activity | lyase activity | isomerase activity | ligase activity | protein tag activity | cargo receptor activity | histone binding | protein folding chaperone | translation regulator activity | nutrient reservoir activity | receptor ligand activity | molecular transducer activity | molecular adaptor activity | toxin activity | cell adhesion mediator activity | molecular function regulator activity | virus coreceptor activity | catalytic activity, acting on a protein | catalytic activity, acting on dna | catalytic activity, acting on rna | molecular carrier activity | transcription regulator activity | general transcription initiation factor activity | molecular sensor activity | molecular sequestering activity | atp-dependent activity | other molecular function | all biological process | mitotic cell cycle | cytokinesis | cytoplasmic translation | immune system process | muscle system process | circulatory system process | renal system process | respiratory system process | carbohydrate metabolic process | generation of precursor metabolites and energy | dna replication | dna repair | dna recombination | chromatin organization | dna-templated transcription | regulation of dna-templated transcription | trna metabolic process | protein folding | protein glycosylation | amino acid metabolic process | modified amino acid metabolic process | lipid metabolic process | vitamin metabolic process | sulfur compound metabolic process | intracellular protein transport | nucleocytoplasmic transport | autophagy | inflammatory response | mitochondrion organization | cytoskeleton organization | microtubule-based movement | peroxisome organization | lysosome organization | chromosome segregation | cell adhesion | establishment or maintenance of cell polarity | programmed cell death | photosynthesis | mrna metabolic process | snrna metabolic process | vesicle-mediated transport | reproductive process | digestive system process | signaling | cell differentiation | protein catabolic process | extracellular matrix organization | regulatory ncrna-mediated gene silencing | telomere organization | cell junction organization | wound healing | ribosome biogenesis | cilium organization | anatomical structure development | cell motility | nervous system process | endocrine process | protein maturation | transmembrane transport | nucleobase-containing small molecule metabolic process | hepaticobiliary system process | membrane organization | protein-containing complex assembly | cell wall organization or biogenesis | nitrogen cycle metabolic process | protein localization to plasma membrane | defense response to other organism | detoxification | meiotic nuclear division | mitotic nuclear division | mitochondrial gene expression | carbohydrate derivative metabolic process | other biological process | all cellular component | nuclear chromosome | extracellular region | extracellular space | cell wall | nucleus | nuclear envelope | nucleoplasm | chromosome | nucleolus | mitochondrion | lysosome | endosome | vacuole | peroxisome | endoplasmic reticulum | golgi apparatus | lipid droplet | microtubule organizing center | cytosol | ribosome | cytoskeleton | plasma membrane | cilium | plastid | thylakoid | external encapsulating structure | extracellular matrix | cytoplasmic vesicle | organelle | other cellular component | |||
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Keywords
- Molecular function
- Biological process
Enzyme and pathway databases
Names & Taxonomy
Protein names
- Recommended nameDnaJ homolog subfamily C member 3
- Alternative names
Gene names
Organism names
- Organism
- Taxonomic lineageEukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Primates > Haplorrhini > Catarrhini > Hominidae > Homo
Accessions
- Primary accessionQ13217
- Secondary accessions
Proteomes
Organism-specific databases
Subcellular Location
Disease & Variants
Involvement in disease
Ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus (ACPHD)
- Note
- DescriptionA disease characterized by juvenile-onset diabetes and neurodegeneration, resulting in ataxia, upper-motor-neuron damage, peripheral neuropathy, hearing loss, and cerebral atrophy.
- See alsoMIM:616192
Variants
We now provide the "Disease & Variants" viewer in its own tab.
The viewer provides 475 variants from UniProt as well as other sources including ClinVar and dbSNP.
Keywords
- Disease
Organism-specific databases
Miscellaneous
Genetic variation databases
PTM/Processing
Features
Showing features for signal, chain, disulfide bond, modified residue, modified residue (large scale data).
Type | ID | Position(s) | Source | Description | |||
---|---|---|---|---|---|---|---|
Signal | 1-31 | UniProt | |||||
Sequence: MVAPGSVTSRLGSVFPFLLVLVDLQYEGAEC | |||||||
Chain | PRO_0000071045 | 32-504 | UniProt | DnaJ homolog subfamily C member 3 | |||
Sequence: GVNADVEKHLELGKKLLAAGQLADALSQFHAAVDGDPDNYIAYYRRATVFLAMGKSKAALPDLTKVIQLKMDFTAARLQRGHLLLKQGKLDEAEDDFKKVLKSNPSENEEKEAQSQLIKSDEMQRLRSQALNAFGSGDYTAAIAFLDKILEVCVWDAELRELRAECFIKEGEPRKAISDLKAASKLKNDNTEAFYKISTLYYQLGDHELSLSEVRECLKLDQDHKRCFAHYKQVKKLNKLIESAEELIRDGRYTDATSKYESVMKTEPSIAEYTVRSKERICHCFSKDEKPVEAIRVCSEVLQMEPDNVNALKDRAEAYLIEEMYDEAIQDYETAQEHNENDQQIREGLEKAQRLLKQSQKRDYYKILGVKRNAKKQEIIKAYRKLALQWHPDNFQNEEEKKKAEKKFIDIAAAKEVLSDPEMRKKFDDGEDPLDAESQQGGGGNPFHRSWNSWQGFNPFSSGGPFRFKFHFN | |||||||
Disulfide bond | 248↔258 | UniProt | |||||
Sequence: CLKLDQDHKRC | |||||||
Modified residue | 274 | UniProt | Phosphoserine; by FAM20C | ||||
Sequence: S | |||||||
Modified residue (large scale data) | 274 | PRIDE | Phosphoserine | ||||
Sequence: S | |||||||
Disulfide bond | 313↔329 | UniProt | |||||
Sequence: CHCFSKDEKPVEAIRVC | |||||||
Modified residue (large scale data) | 492 | PRIDE | Phosphoserine | ||||
Sequence: S |
Keywords
- PTM
Proteomic databases
PTM databases
Expression
Tissue specificity
Widely expressed with high level in the pancreas and testis. Also expressed in cell lines with different levels.
Induction
Up-regulated during an endoplasmic reticulum stress via ATF6. Activated in response to infection by influenza virus through the dissociation of DNAJB1. Down-regulated by DNAJB1 and THAP12.
Gene expression databases
Organism-specific databases
Interaction
Subunit
Interacts with EIF2AK4/GCN2; this interaction occurs under endoplasmic reticulum (ER) stress, hypothermic and amino acid starving stress conditions and inhibits EIF2AK4/GCN2 kinase activity. Interacts with EIF2AK3 (By similarity).
Interacts with EIF2AK2 (PubMed:8576172).
Forms a trimeric complex with DNAJB1 and HSPA8 (PubMed:9920933).
Interacts with THAP12 (PubMed:9447982).
Interacts with EIF2AK2 (PubMed:8576172).
Forms a trimeric complex with DNAJB1 and HSPA8 (PubMed:9920933).
Interacts with THAP12 (PubMed:9447982).
Protein-protein interaction databases
Miscellaneous
Structure
Family & Domains
Features
Showing features for repeat, region, domain.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Repeat | 37-70 | TPR 1 | ||||
Sequence: VEKHLELGKKLLAAGQLADALSQFHAAVDGDPDN | ||||||
Repeat | 72-104 | TPR 2 | ||||
Sequence: IAYYRRATVFLAMGKSKAALPDLTKVIQLKMDF | ||||||
Repeat | 105-138 | TPR 3 | ||||
Sequence: TAARLQRGHLLLKQGKLDEAEDDFKKVLKSNPSE | ||||||
Repeat | 154-187 | TPR 4 | ||||
Sequence: MQRLRSQALNAFGSGDYTAAIAFLDKILEVCVWD | ||||||
Repeat | 189-221 | TPR 5 | ||||
Sequence: ELRELRAECFIKEGEPRKAISDLKAASKLKNDN | ||||||
Repeat | 222-255 | TPR 6 | ||||
Sequence: TEAFYKISTLYYQLGDHELSLSEVRECLKLDQDH | ||||||
Repeat | 268-301 | TPR 7 | ||||
Sequence: LNKLIESAEELIRDGRYTDATSKYESVMKTEPSI | ||||||
Repeat | 306-339 | TPR 8 | ||||
Sequence: VRSKERICHCFSKDEKPVEAIRVCSEVLQMEPDN | ||||||
Repeat | 340-373 | TPR 9 | ||||
Sequence: VNALKDRAEAYLIEEMYDEAIQDYETAQEHNEND | ||||||
Region | 375-393 | Flexible linker | ||||
Sequence: QIREGLEKAQRLLKQSQKR | ||||||
Domain | 394-462 | J | ||||
Sequence: DYYKILGVKRNAKKQEIIKAYRKLALQWHPDNFQNEEEKKKAEKKFIDIAAAKEVLSDPEMRKKFDDGE | ||||||
Region | 451-481 | Disordered | ||||
Sequence: DPEMRKKFDDGEDPLDAESQQGGGGNPFHRS |
Domain
The J domain mediates interaction with HSPA8.
Binding to misfolded proteins is mediated by a hydrophobic patch forming a large groove within the first two TPR repeats.
Keywords
- Domain
Phylogenomic databases
Family and domain databases
Sequence
- Sequence statusComplete
- Sequence processingThe displayed sequence is further processed into a mature form.
- Length504
- Mass (Da)57,580
- Last updated1996-11-01 v1
- ChecksumE720A1E7F618B912
Computationally mapped potential isoform sequences
There is 1 potential isoform mapped to this entry
Entry | Entry name | Gene name | Length | ||
---|---|---|---|---|---|
X6R9L0 | X6R9L0_HUMAN | DNAJC3 | 453 |
Keywords
- Technical term
Sequence databases
Nucleotide Sequence | Protein Sequence | Molecule Type | Status | |
---|---|---|---|---|
U28424 EMBL· GenBank· DDBJ | AAC50502.1 EMBL· GenBank· DDBJ | mRNA | ||
AY795482 EMBL· GenBank· DDBJ | AAV40838.1 EMBL· GenBank· DDBJ | Genomic DNA | ||
AL138955 EMBL· GenBank· DDBJ | - | Genomic DNA | No translation available. | |
BC047936 EMBL· GenBank· DDBJ | AAH47936.2 EMBL· GenBank· DDBJ | mRNA |