Reactome | UniProt:Q13217 DNAJC3

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UniProt:Q13217 DNAJC3 Show undefined attributes

chain	signal peptide:1-31 chain:32-504
checksum	E720A1E7F618B912
comment	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.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).SUBCELLULAR LOCATION 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.DOMAIN The J domain mediates interaction with HSPA8.DOMAIN Binding to misfolded proteins is mediated by a hydrophobic patch forming a large groove within the first two TPR repeats.DISEASE The disease is caused by variants affecting the gene represented in this entry.
created	[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
crossReference	[DatabaseIdentifier:12347130] RefSeq:NP_006251.1 [DatabaseIdentifier:12293774] Pharos - Targets:Q13217 [DatabaseIdentifier:12256034] PRO:Q13217 [DatabaseIdentifier:12231004] PDB:2Y4U [DatabaseIdentifier:12231005] PDB:2Y4T [DatabaseIdentifier:12137305] Orphanet:DNAJC3 [DatabaseIdentifier:12133609] OpenTargets:ENSG00000102580 [DatabaseIdentifier:11848916] HPA:ENSG00000102580-DNAJC3 [DatabaseIdentifier:11811696] GlyGen:Q13217 [DatabaseIdentifier:11795948] GeneCards:DNAJC3 [DatabaseIdentifier:11680261] ENSEMBL:ENST00000602402 [DatabaseIdentifier:11557562] ENSEMBL:ENSP00000473631 [DatabaseIdentifier:11463193] ENSEMBL:ENSG00000102580
databaseName	UniProt
dbId	405575
description	recommendedName: DnaJ homolog subfamily C member 3 alternativeName: Endoplasmic reticulum DNA J domain-containing protein 6 shortName: ER-resident protein ERdj6 shortName: ERdj6 alternativeName: Interferon-induced, double-stranded RNA-activated protein kinase inhibitor alternativeName: Protein kinase inhibitor of 58 kDa shortName: Protein kinase inhibitor p58
displayName	UniProt:Q13217 DNAJC3
geneName	DNAJC3 P58IPK PRKRI
identifier	Q13217
isSequenceChanged	false
keyword	3D-structure Antiviral defense Chaperone Deafness Diabetes mellitus Disulfide bond Endoplasmic reticulum Neurodegeneration Neuropathy Phosphoprotein Proteomics identification Reference proteome Repeat Repressor Signal Stress response TPR repeat Translation regulation Unfolded protein response
modified	[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
moleculeType	Protein
name	DNAJC3
otherIdentifier	11717372_s_at 11717373_at 11717374_at 1558080_PM_s_at 1558080_s_at 16775856 208499_PM_s_at 208499_s_at 225284_PM_at 225284_at 235341_PM_at 235341_at 33208_at 3497272 3497277 3497279 3497280 3497281 3497287 3497290 3497291 3497292 3497293 3497301 3497302 3497305 3497306 3497307 3497308 3497317 43650_at 53918_at 5611 57648_at 7969651 A_23_P36962 A_33_P3462960 GE86033 GE86625 GE87711 GO:0004860 GO:0005515 GO:0005576 GO:0005737 GO:0005783 GO:0005788 GO:0005829 GO:0006417 GO:0006986 GO:0016020 GO:0019901 GO:0034975 GO:0034976 GO:0035578 GO:0036494 GO:0043066 GO:0043226 GO:0051087 GO:0051603 GO:0051607 GO:0051787 GO:0070062 GO:0070417 GO:0098542 GO:0098772 GO:1902010 GO:1903561 GO:1903912 HMNXSV003039843 Hs.60054.0.A1_3p_at Hs.6019.0.A1_3p_at Hs2.429981.1.A1_3p_s_at ILMN_1659843 PH_hs_0021974 PH_hs_0037639 TC13000336.hg TC13001241.hg g5453979_3p_a_at
physicalEntity	[EntityWithAccessionedSequence:R-HSA-6806239] DNAJC3 [extracellular region] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-6799146] DNAJC3 [azurophil granule lumen] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-8956975] p-DNAJC3 [endoplasmic reticulum lumen] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-448201] DNAJC3 [endoplasmic reticulum lumen] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-448199] DNAJC3 [cytosol] - Homo sapiens
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:12732835] UCSC:Q13217 DNAJC3 [ReferenceDNASequence:12117857] OMIM:601184 DNAJC3 [ReferenceDNASequence:12041466] NCBI Gene:5611 DNAJC3 [ReferenceDNASequence:11957373] Monarch:5611 DNAJC3 [ReferenceDNASequence:11869809] KEGG:hsa:5611 DNAJC3 [ReferenceDNASequence:11827653] HGNC:9439 DNAJC3 [ReferenceDNASequence:11435577] dbSNP Gene:5611 DNAJC3 [ReferenceDNASequence:11425038] CTD Gene:5611 DNAJC3 [ReferenceDNASequence:11398722] COSMIC - genes:DNAJC3 DNAJC3 [ReferenceDNASequence:11327584] BioGPS Gene:5611 DNAJC3 [ReferenceDNASequence:ensembl:ENSG00000102580] ENSEMBL:ENSG00000102580 DNAJC3
referenceTranscript	[ReferenceRNASequence:12528994] RefSeq:NM_006260.5 DNAJC3
schemaClass	ReferenceGeneProduct
secondaryIdentifier	DNJC3_HUMAN Q86WT9 Q8N4N2
sequenceLength	504
species	[Species:48887] Homo sapiens
stId	uniprot:Q13217
url	http://purl.uniprot.org/uniprot/Q13217

Referrals

(interactor)	[UndirectedInteraction:11207553] UniProt:Q13217 <-> UniProt:Q9P273 (IntAct) [UndirectedInteraction:11207549] UniProt:Q13217 <-> UniProt:Q9UBS3 (IntAct) [UndirectedInteraction:11207547] UniProt:Q13217 <-> UniProt:O75618 (IntAct) [UndirectedInteraction:11207543] UniProt:Q13217 <-> UniProt:O95672 (IntAct) [UndirectedInteraction:11207539] UniProt:Q13217 <-> UniProt:Q68BL8 (IntAct)
(referenceEntity)	[EntityWithAccessionedSequence:R-HSA-6806239] DNAJC3 [extracellular region] [EntityWithAccessionedSequence:R-HSA-6799146] DNAJC3 [azurophil granule lumen] [EntityWithAccessionedSequence:R-HSA-8956975] p-DNAJC3 [endoplasmic reticulum lumen] [EntityWithAccessionedSequence:R-HSA-448201] DNAJC3 [endoplasmic reticulum lumen] [EntityWithAccessionedSequence:R-HSA-448199] DNAJC3 [cytosol]
(referenceSequence)	[ModifiedResidue:8957058] phosphorylated residue at unknown position