Gene Symbol: DNAJC10
Description: DnaJ heat shock protein family (Hsp40) member C10
Alias: ERdj5, JPDI, MTHr, PDIA19, dnaJ homolog subfamily C member 10, DnaJ (Hsp40) homolog, subfamily C, member 10, ER-resident protein ERdj5, J-domain-containing protein disulfide isomerase-like protein, endoplasmic reticulum DNA J domain-containing protein 5, macrothioredoxin, protein disulfide isomerase family A, member 19
Species: human
Products:     DNAJC10

Top Publications

  1. Dong M, Bridges J, Apsley K, Xu Y, Weaver T. ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein C. Mol Biol Cell. 2008;19:2620-30 pubmed publisher
    ..BiP) and two heat shock protein 40 family members, endoplasmic reticulum-localized DnaJ homologues ERdj4 and ERdj5, were significantly elevated and exhibited prolonged and specific association with the misfolded proprotein; in ..
  2. Hosoda A, Kimata Y, Tsuru A, Kohno K. JPDI, a novel endoplasmic reticulum-resident protein containing both a BiP-interacting J-domain and thioredoxin-like motifs. J Biol Chem. 2003;278:2669-76 pubmed
    ..for proteins containing the C-terminal KDEL motif predicted some novel murine proteins, one of which designated JPDI (J-domain-containing protein disulfide isomerase-like protein) is characterized in this study...
  3. Ushioda R, Hoseki J, Araki K, Jansen G, Thomas D, Nagata K. ERdj5 is required as a disulfide reductase for degradation of misfolded proteins in the ER. Science. 2008;321:569-72 pubmed publisher
    ..Here, we found that an ER-resident protein ERdj5 had a reductase activity, cleaved the disulfide bonds of misfolded proteins, and accelerated ERAD through its ..
  4. Thomas C, Spyrou G. ERdj5 sensitizes neuroblastoma cells to endoplasmic reticulum stress-induced apoptosis. J Biol Chem. 2009;284:6282-90 pubmed publisher
    ..b>ERdj5 is a novel ER chaperone that regulates the ER-associated degradation of misfolded proteins through its ..
  5. Riemer J, Appenzeller Herzog C, Johansson L, Bodenmiller B, Hartmann Petersen R, Ellgaard L. A luminal flavoprotein in endoplasmic reticulum-associated degradation. Proc Natl Acad Sci U S A. 2009;106:14831-6 pubmed publisher
    ..We also identify the ERAD components SEL1L, OS-9 and ERdj5, a known reductase of ERAD substrates, as interaction partners of ERFAD...
  6. Hagiwara M, Maegawa K, Suzuki M, Ushioda R, Araki K, Matsumoto Y, et al. Structural basis of an ERAD pathway mediated by the ER-resident protein disulfide reductase ERdj5. Mol Cell. 2011;41:432-44 pubmed publisher
    ER-associated degradation (ERAD) is an ER quality-control process that eliminates terminally misfolded proteins. ERdj5 was recently discovered to be a key ER-resident PDI family member protein that accelerates ERAD by reducing incorrect ..
  7. Gu S, Chen J, Ying K, Wang S, Jin W, Qian J, et al. Cloning and identification of a novel cDNA which encodes a putative protein with a DnaJ domain and a thioredoxin active motif, human macrothioredoxin. Biochem Genet. 2003;41:245-53 pubmed
    ..So it is named human macrothioredoxin (hMTHr)...
  8. Cunnea P, Miranda Vizuete A, Bertoli G, Simmen T, Damdimopoulos A, Hermann S, et al. ERdj5, an endoplasmic reticulum (ER)-resident protein containing DnaJ and thioredoxin domains, is expressed in secretory cells or following ER stress. J Biol Chem. 2003;278:1059-66 pubmed
    ..Here we characterize a novel human putative ER co-chaperone (ERdj5) containing domains resembling DnaJ, protein-disulfide isomerase, and thioredoxin domains...
  9. Oka O, Pringle M, Schopp I, Braakman I, Bulleid N. ERdj5 is the ER reductase that catalyzes the removal of non-native disulfides and correct folding of the LDL receptor. Mol Cell. 2013;50:793-804 pubmed publisher
    b>ERdj5 is a member of the protein disulfide isomerase family of proteins localized to the endoplasmic reticulum (ER) of mammalian cells. To date, only a limited number of substrates for ERdj5 are known...

More Information


  1. Corazzari M, Lovat P, Armstrong J, Fimia G, Hill D, Birch Machin M, et al. Targeting homeostatic mechanisms of endoplasmic reticulum stress to increase susceptibility of cancer cells to fenretinide-induced apoptosis: the role of stress proteins ERdj5 and ERp57. Br J Cancer. 2007;96:1062-71 pubmed
    ..The ER stress genes ERdj5, ERp57, GRP78, calreticulin and calnexin were induced in neuroectodermal tumour cells by fenretinide...
  2. Lamriben L, Oster M, Tamura T, Tian W, Yang Z, Clausen H, et al. EDEM1's mannosidase-like domain binds ERAD client proteins in a redox-sensitive manner and possesses catalytic activity. J Biol Chem. 2018;293:13932-13945 pubmed publisher
    ..We also determined that both IDRs are required for binding to the ERAD component ERdj5 as an interaction with ERdj5 was not observed with the MLD alone...
  3. Winter J, Curry N, Gildea D, Williams K, Lee M, Hu Y, et al. Modifier locus mapping of a transgenic F2 mouse population identifies CCDC115 as a novel aggressive prostate cancer modifier gene in humans. BMC Genomics. 2018;19:450 pubmed publisher
    ..We identified four genes (CCDC115, DNAJC10, RNF149, and STYXL1), which encompassed all of the following characteristics: 1) one or more germline variants ..
  4. Williams J, Inoue T, Chen G, Tsai B. The nucleotide exchange factors Grp170 and Sil1 induce cholera toxin release from BiP to enable retrotranslocation. Mol Biol Cell. 2015;26:2181-9 pubmed publisher
    ..composed of the E3 ubiquitin ligase Hrd1-Sel1L complex, in part via the activity of the Sel1L-binding partner ERdj5. This J protein stimulates BiP's ATPase activity, allowing BiP to capture the toxin...
  5. Avezov E, Konno T, Zyryanova A, Chen W, Laine R, Crespillo Casado A, et al. Retarded PDI diffusion and a reductive shift in poise of the calcium depleted endoplasmic reticulum. BMC Biol. 2015;13:2 pubmed publisher
    ..5-fold decline in PDI1A mobility, whereas the mobility of the reducing PDI family member, ERdj5 was unaffected...
  6. Ushioda R, Miyamoto A, Inoue M, Watanabe S, Okumura M, Maegawa K, et al. Redox-assisted regulation of Ca2+ homeostasis in the endoplasmic reticulum by disulfide reductase ERdj5. Proc Natl Acad Sci U S A. 2016;113:E6055-E6063 pubmed
    ..Here, we show that ERdj5, a mammalian ER disulfide reductase, which we reported to be involved in the ER-associated degradation of ..
  7. Behnke J, Mann M, Scruggs F, Feige M, Hendershot L. Members of the Hsp70 Family Recognize Distinct Types of Sequences to Execute ER Quality Control. Mol Cell. 2016;63:739-52 pubmed publisher
    ..substrates of the ER Hsp70 system: BiP, Grp170, and three of BiP's DnaJ-family co-factors (ERdj3, ERdj4, and ERdj5)...
  8. Inoue T, Dosey A, Herbstman J, Ravindran M, Skiniotis G, Tsai B. ERdj5 Reductase Cooperates with Protein Disulfide Isomerase To Promote Simian Virus 40 Endoplasmic Reticulum Membrane Translocation. J Virol. 2015;89:8897-908 pubmed publisher
    ..Here we identify ERdj5 as an ER enzyme that reduces SV40's disulfide bonds, a reaction important for its ER membrane transport and ..
  9. Hagiwara M, Nagata K. Redox-dependent protein quality control in the endoplasmic reticulum: folding to degradation. Antioxid Redox Signal. 2012;16:1119-28 pubmed publisher
    ..family molecular chaperone that recruits substrates to the dislocon channel after dissociation from the EDEM1/ERdj5 complex coupled with ATP hydrolysis...
  10. Kimura M, Tamura Y, Guignabert C, Takei M, Kosaki K, Tanabe N, et al. A genome-wide association analysis identifies PDE1A|DNAJC10 locus on chromosome 2 associated with idiopathic pulmonary arterial hypertension in a Japanese population. Oncotarget. 2017;8:74917-74926 pubmed publisher
    ..the genome-wide study were submitted to the validation study, and a novel susceptibility locus, PDE1A|DNAJC10, was identified that maps to 2q32.1 (rs71427857, P = 7...
  11. Galligan J, Petersen D. The human protein disulfide isomerase gene family. Hum Genomics. 2012;6:6 pubmed publisher
    ..Understanding the mechanisms of protein-folding, specifically thiol-disulfide exchange, may lead to development of a novel class of therapeutics that would help alleviate a wide range of diseases by targeting the UPR. ..
  12. Xu W, Liu L, Hornby D. c-IAP1 binds and processes PCSK9 protein: linking the c-IAP1 in a TNF-α pathway to PCSK9-mediated LDLR degradation pathway. Molecules. 2012;17:12086-101 pubmed publisher
    ..The novel pathway described here will open new avenues for exploring novel disease treatments. ..
  13. Tamura T, Cormier J, Hebert D. Characterization of early EDEM1 protein maturation events and their functional implications. J Biol Chem. 2011;286:24906-15 pubmed publisher
    ..Signal sequence cleavage produced a soluble form of EDEM1 that efficiently associated with the oxidoreductase ERdj5 and most effectively accelerated the turnover of a soluble ERAD substrate...
  14. Maattanen P, Gehring K, Bergeron J, Thomas D. Protein quality control in the ER: the recognition of misfolded proteins. Semin Cell Dev Biol. 2010;21:500-11 pubmed publisher
    ..In another example the protein disulfide isomerase ERdj5 binds specifically to EDEM (which is probably a mannosidase) and a lectin OS9, and reduces the disulfide bonds of ..
  15. Athanasiou D, Bevilacqua D, Aguilà M, McCulley C, Kanuga N, Iwawaki T, et al. The co-chaperone and reductase ERdj5 facilitates rod opsin biogenesis and quality control. Hum Mol Genet. 2014;23:6594-606 pubmed publisher
    ..Here, we show that the BiP co-chaperone and reductase protein ERdj5 (DNAJC10) regulates this process...
  16. Ushioda R, Nagata K. The endoplasmic reticulum-associated degradation and disulfide reductase ERdj5. Methods Enzymol. 2011;490:235-58 pubmed publisher
    ..After briefly mentioned on productive folding of nascent polypeptides in the ER, we here overview the above three processes in ERAD system by highlighting on novel ERAD factors such as EDEM and ERdj5 in mammals and yeasts.
  17. Williams J, Inoue T, Banks L, Tsai B. The ERdj5-Sel1L complex facilitates cholera toxin retrotranslocation. Mol Biol Cell. 2013;24:785-95 pubmed publisher
    ..Using loss-of-function and gain-of-function approaches, we demonstrate that the ER-resident factor ERdj5 promotes CTA1 retrotranslocation, in part, via its J domain...
  18. Hulleman J, Genereux J, Nguyen A. Mapping wild-type and R345W fibulin-3 intracellular interactomes. Exp Eye Res. 2016;153:165-169 pubmed publisher
    ..Eight of these interactions (ANXA5, ERdj5, PDIA4, P4HB, PDIA6, RCN1, SDF2L1, and TXNDC5) were verified at the western blotting level...
  19. Maegawa K, Watanabe S, Noi K, Okumura M, Amagai Y, Inoue M, et al. The Highly Dynamic Nature of ERdj5 Is Key to Efficient Elimination of Aberrant Protein Oligomers through ER-Associated Degradation. Structure. 2017;25:846-857.e4 pubmed publisher
    b>ERdj5, composed of an N-terminal J domain followed by six thioredoxin-like domains, is the largest protein disulfide isomerase family member and functions as an ER-localized disulfide reductase that enhances ER-associated degradation (..
  20. Wang M, Bridges J, Na C, Xu Y, Weaver T. Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein C. J Biol Chem. 2009;284:33377-83 pubmed publisher
    ..These results support a model in which MKS3 links the ER lumenal quality control machinery with the cytosolic degradation apparatus. ..