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FLASH GENE
Symbol DNAJC3 contributors: mct - updated : 23-12-2013
HGNC name DnaJ (Hsp40) homolog, subfamily C, member 3
HGNC id 9439
Corresponding disease
JDND juvenile-onset diabetes and central and peripheral neurodegeneration
Location 13q32.1      Physical location : 96.329.392 - 96.447.243
Synonym name
  • protein-kinase, interferon-inducible double stranded RNA dependent inhibitor
  • ER-resident protein ERdj6
  • protein kinase inhibitor p58
  • protein kinase inhibitor of 58 kDa
  • endoplasmic reticulum DnaJ protein 6
  • endoplasmic reticulum DNA J domain-containing protein 6
  • Synonym symbol(s) PRKRI, P58, HP58, P58IPK, ERdj6, P58(IPK)
    DNA
    TYPE functioning gene
    STRUCTURE 117.85 kb     12 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Physical map
    GPC6 13q32 glypican 6 DCT 13q32 dopachrome tautomerase (dopachrome delta-isomerase, tyrosine-related protein 2) TGDS 13q32.1 TDP-glucose 4,6-dehydratase ITR 13q32.1 intimal thickness-related receptor SOX21 13q31-q32 SRY (sex determining region Y)-box 21 LOC387941 13 LOC387941 LOC341583 13q32.1 similar to bromodomain containing 7; bromodomain protein; bromodomain-containing 7 LOC387942 13 LOC387942 ABCC4 13q31 ATP-binding cassette, sub-family C (CFTR/MRP), member 4 CLDN10 13q31-q34 claudin 10 DZIP1 13q32.1 claudin 10 DNAJC3 13q32 DnaJ (Hsp40) homolog, subfamily C, member 3 UGCGL2 13q32.1 UDP-glucose ceramide glucosyltransferase-like 2 HS6ST3 13 heparan sulfate 6-O-sulfotransferase 3 LOC283476 13q32.2 hypothetical protein LOC283476 LOC390420 13 similar to heat shock protein 84 - mouse LOC341592 13q32.2 similar to tubby like protein 3 GPR80 13q32.2 G protein-coupled receptor 80 MBNL2 13q31.1 muscleblind-like 2 (Drosophila) RAP2A 13q34 RAP2A, member of RAS oncogene family LOC121906 13q32.2 similar to Proteasome subunit alpha type 6 (Proteasome iota chain) (Macropain iota chain) (Multicatalytic endopeptidase complex iota chain) (27 kDa prosomal protein) (PROS-27) (p27K) LOC387943 13 LOC387943 KPNB3 13q32.2 karyopherin (importin) beta 3 FARP1 13q32.2-q32.3 FERM, RhoGEF (ARHGEF) and pleckstrin domain protein 1 (chondrocyte-derived) ZNF183L1 13q zinc finger protein 183-like 1
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    12 - 5598 58 504 - 2003 12601012
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveesophagus   highly
     liver   highly Homo sapiens
    Endocrineneuroendocrinepituitary  highly
     pancreas   highly Homo sapiens
    Respiratoryrespiratory tracttrachea  highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / Hematopoieticbone marrow   
    Muscularstriatumskeletal  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • nine N-terminal tetratricopeptide repeat motifs, divided into three subdomains of three motifs each
  • a region of similarity with the DnaJ protein family, with oncogenic properties
  • lack the -KDEL sequence found on most resident ER luminal chaperones
  • conserved J domain attached to the C-terminal end via a flexible linker, and the structure shows the conserved Hsp70-binding histidine-proline-aspartate (HPD) motif to be situated on the very edge of the elongated protein
  • HOMOLOGY
    interspecies homolog to murine Dnajc3
    homolog to Drosophila CG8286
    homolog to C.elegans dnj-7
    Homologene
    FAMILY
  • PRK family, DNAJ chaperone family
  • CATEGORY regulatory , protooncogene
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    text
  • previously unanticipated location in the ER lumen where its putative function as a cochaperone explains the stress-sensitivity phenotype of knockout cells
  • localized in the lumen of the ER
  • basic FUNCTION
  • acting as an inhibitor of the interferon-induced double-stranded RNA-activated protein kinase (PKR)
  • inhibits ER stress and plays an important role in maintaining balance and stability of the ER in retinal capillary endothelial cells (HRCECs)
  • defends cells against endoplasmic reticulum (ER) stress
  • ER molecular chaperone to maintain protein-folding homeostasis
  • act potentially as a co-chaperone, binding un- or misfolded proteins and delivering them to HSPA5
  • plays a crucial role in ER protein folding and the UPR, thus adding evidence that dysfunctions in regulation of ER stress might serve as a common pathway linking diabetes mellitus and neurodegeneration
  • CELLULAR PROCESS protein, translation regulation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • important component of a negative feedback loop used by the cell to inhibit EIF2A signaling, and thus attenuate the unfolded protein response
  • act potentially together with HSPA5 to prevent protein aggregations and promote protein foldings within ER
  • interplay between MAPK14 phosphorylation and DNAJC3 upregulation has key roles in modulating ERP29-induced cell-growth arrest and survival
  • DNAJC3 is a HSPA5 (immunoglobulin heavy-chain binding protein) co-chaperone
  • cell & other
    REGULATION
    induced by interferon
    ASSOCIATED DISORDERS
    corresponding disease(s) JDND
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       gain of function
    during virus infection to inhibit virus-induced apoptosis and inflammation to prolong host survival, even while prolonging viral replication
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • in mice, loss of Dnajc3 leads to hyperglycemia and glucosuria associated with increasing apoptosis of pancreatic beta cells and reduced insulin levels