Gene Symbol: UBE2J1
Description: ubiquitin conjugating enzyme E2 J1
Alias: CGI-76, HSPC153, HSPC205, HSU93243, NCUBE-1, NCUBE1, UBC6, UBC6E, Ubc6p, ubiquitin-conjugating enzyme E2 J1, E2 ubiquitin-conjugating enzyme J1, HSUBC6e, non-canonical ubiquitin-conjugating enzyme 1, ubiquitin-conjugating enzyme E2, J1 (UBC6 homolog, yeast), ubiquitin-conjugating enzyme E2, J1, U, yeast ubiquitin-conjugating enzyme UBC6 homolog E
Species: human
Products:     UBE2J1

Top Publications

  1. Burr M, Cano F, Svobodova S, Boyle L, Boname J, Lehner P. HRD1 and UBE2J1 target misfolded MHC class I heavy chains for endoplasmic reticulum-associated degradation. Proc Natl Acad Sci U S A. 2011;108:2034-9 pubmed publisher
    ..identify an essential role for the E3 ligase HRD1 (Synoviolin) together with the E2 ubiquitin-conjugating enzyme UBE2J1 in the ubiquitination and dislocation of misfolded MHC class I heavy chains...
  2. Mueller B, Klemm E, Spooner E, Claessen J, Ploegh H. SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins. Proc Natl Acad Sci U S A. 2008;105:12325-30 pubmed publisher
    ..We identified AUP1, UBXD8, UBC6e, and OS9 as functionally important components of this degradation complex in mammalian cells, as confirmed by ..
  3. Burr M, van den Boomen D, Bye H, Antrobus R, Wiertz E, Lehner P. MHC class I molecules are preferentially ubiquitinated on endoplasmic reticulum luminal residues during HRD1 ubiquitin E3 ligase-mediated dislocation. Proc Natl Acad Sci U S A. 2013;110:14290-5 pubmed publisher
    ..reticulum (ER)-associated degradation (ERAD) by the ubiquitin E3 ligase HRD1, and E2 ubiquitin conjugating enzyme UBE2J1, and represent one of the few known endogenous ERAD substrates...
  4. Feng T, Deng L, Lu X, Pan W, Wu Q, Dai J. Ubiquitin-conjugating enzyme UBE2J1 negatively modulates interferon pathway and promotes RNA virus infection. Virol J. 2018;15:132 pubmed publisher
    ..We found that silencing UBE2J1 significantly impaired DENV infection, while overexpression of UBE2J1 enhanced DENV infection...
  5. Koenig P, Nicholls P, Schmidt F, Hagiwara M, Maruyama T, Frydman G, et al. The E2 ubiquitin-conjugating enzyme UBE2J1 is required for spermiogenesis in mice. J Biol Chem. 2014;289:34490-502 pubmed publisher
    ..Dislocation substrates are ubiquitylated in the cytosol by E2 ubiquitin-conjugating/E3 ligase complexes. UBE2J1 is one of the well-characterized E2 enzymes that participate in this process...
  6. Palmer C, Galan Caridad J, Weisberg S, Lei L, Esquilin J, Croft G, et al. Zfx facilitates tumorigenesis caused by activation of the Hedgehog pathway. Cancer Res. 2014;74:5914-24 pubmed publisher
    ..medulloblastoma cell line to characterize direct, evolutionarily conserved targets of Zfx, identifying Dis3L and Ube2j1 as two targets required for the growth of the human medulloblastoma cells...
  7. Ma H, Dang Y, Wu Y, Jia G, Anaya E, Zhang J, et al. A CRISPR-Based Screen Identifies Genes Essential for West-Nile-Virus-Induced Cell Death. Cell Rep. 2015;12:673-83 pubmed publisher
    ..Among the genes identified, seven genes, EMC2, EMC3, SEL1L, DERL2, UBE2G2, UBE2J1, and HRD1, stood out as having the strongest phenotype, whose knockout conferred strong protection against WNV-..
  8. Barrera M, Aguilera S, Castro I, Cortes J, Bahamondes V, Quest A, et al. Pro-inflammatory cytokines enhance ERAD and ATF6? pathway activity in salivary glands of Sjögren's syndrome patients. J Autoimmun. 2016;75:68-81 pubmed publisher
    ..ATF6f cleavage fragment, and increased expression of ERAD machinery components, such as EDEM1, p97, SEL1L, gp78, UBE2J1, UBE2G2, HERP and DERLIN1, were observed compared to controls...
  9. Rukova B, Staneva R, Hadjidekova S, Stamenov G, Milanova V, Toncheva D. Whole genome methylation analyses of schizophrenia patients before and after treatment. Biotechnol Biotechnol Equip. 2014;28:518-524 pubmed
    ..i>C16orf70, CST3, DDRGK1, FA2H, FLJ30058, MFSD2B, RFX4, UBE2J1, ZNF311) and male schizophrenia patients with complete remission and control male pool after treatment (..

More Information


  1. Chen Q, Zhong Y, Wu Y, Liu L, Wang P, Liu R, et al. HRD1-mediated ERAD tuning of ER-bound E2 is conserved between plants and mammals. Nat Plants. 2016;2:16094 pubmed publisher
    ..Consistently, UBE2J1, the homologue of UBC32 in mammals, was also identified to be targeted by HRD1 for degradation...
  2. Grove D, Rosser M, Watkins R, Cyr D. Analysis of CFTR folding and degradation in transiently transfected cells. Methods Mol Biol. 2011;741:219-32 pubmed publisher
    ..Methods to detect complexes formed between CFTR folding intermediates and ER quality control factors will also be described. ..
  3. El Khouri E, Le Pavec G, Toledano M, Delaunay Moisan A. RNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR). J Biol Chem. 2013;288:31177-91 pubmed publisher
    ..Our data thus identify RNF185 and RNF5 as a novel E3 ligase module that is central to the control of CFTR degradation. ..
  4. Lester D, Farquharson C, Russell G, Houston B. Identification of a family of noncanonical ubiquitin-conjugating enzymes structurally related to yeast UBC6. Biochem Biophys Res Commun. 2000;269:474-80 pubmed
    ..on chick growth plate chondrocytes we isolated a cDNA encoding a noncanonical ubiquitin-conjugating enzyme (NCUBE1) structurally similar to yeast UBC6...
  5. Wu C, Conze D, Li X, Ying S, Hanover J, Ashwell J. TNF-alpha induced c-IAP1/TRAF2 complex translocation to a Ubc6-containing compartment and TRAF2 ubiquitination. EMBO J. 2005;24:1886-98 pubmed
    ..The ER transmembrane Ubc6 bound to c-IAP1 and served as a cognate E2 for c-IAP1's E3 activity in vitro...
  6. Walter J, Urban J, Volkwein C, Sommer T. Sec61p-independent degradation of the tail-anchored ER membrane protein Ubc6p. EMBO J. 2001;20:3124-31 pubmed
    ..we analyzed the degradation pathway of a tail-anchored ER membrane protein, the ubiquitin-conjugating enzyme 6 (Ubc6p). In contrast to other ubiquitin conjugating enzymes (Ubcs), Ubc6p is naturally short-lived...
  7. Zhong Y, Shen H, Wang Y, Yang Y, Yang P, Fang S. Identification of ERAD components essential for dislocation of the null Hong Kong variant of α-1-antitrypsin (NHK). Biochem Biophys Res Commun. 2015;458:424-8 pubmed publisher
    ..In addition, knockdown of 7 of the 21 components enhanced NHK dislocation. This study uncovers a complex functional network of proteins required for NHK dislocation. ..
  8. Sato T, Sako Y, Sho M, Momohara M, Suico M, Shuto T, et al. STT3B-dependent posttranslational N-glycosylation as a surveillance system for secretory protein. Mol Cell. 2012;47:99-110 pubmed publisher
    ..Hence we postulate that STT3B-dependent posttranslational N-glycosylation is part of a triage-salvage system recognizing cryptic N-glycosylation sites of secretory proteins to preserve protein homeostasis. ..
  9. Fallon L, Bélanger C, Corera A, Kontogiannea M, Regan Klapisz E, Moreau F, et al. A regulated interaction with the UIM protein Eps15 implicates parkin in EGF receptor trafficking and PI(3)K-Akt signalling. Nat Cell Biol. 2006;8:834-42 pubmed
    ..Considering the role of Akt in neuronal survival, our results have broad new implications for understanding the pathogenesis of Parkinson's disease. ..
  10. Pattaro C, De Grandi A, Vitart V, Hayward C, Franke A, Aulchenko Y, et al. A meta-analysis of genome-wide data from five European isolates reveals an association of COL22A1, SYT1, and GABRR2 with serum creatinine level. BMC Med Genet. 2010;11:41 pubmed publisher
    ..including the gamma-aminobutyric acid receptor rho-2 (GABRR2) gene and the ubiquitin-conjugating enzyme E2-J1 (UBE2J1) gene (replication p value = 3.6 x 10(-3))...
  11. Lenk U, Yu H, Walter J, Gelman M, Hartmann E, Kopito R, et al. A role for mammalian Ubc6 homologues in ER-associated protein degradation. J Cell Sci. 2002;115:3007-14 pubmed
    ..In yeast, the tail-anchored ubiquitin-conjugating enzyme Ubc6p, which is localized to the cytoplasmic surface of the ER, participates in ER-associated degradation (ERAD) of ..
  12. Imai Y, Soda M, Inoue H, Hattori N, Mizuno Y, Takahashi R. An unfolded putative transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of Parkin. Cell. 2001;105:891-902 pubmed
    ..Moreover, the insoluble form of Pael receptor accumulates in the brains of AR-JP patients. Here, we show that the unfolded Pael receptor is a substrate of Parkin, the accumulation of which may cause selective neuronal death in AR-JP. ..
  13. Hagiwara M, Ling J, Koenig P, Ploegh H. Posttranscriptional Regulation of Glycoprotein Quality Control in the Endoplasmic Reticulum Is Controlled by the E2 Ub-Conjugating Enzyme UBC6e. Mol Cell. 2016;63:753-67 pubmed publisher
    ..Functional UBC6e requires its precise location in the ER to form a supramolecular complex with Derlin2...
  14. Klemm E, Spooner E, Ploegh H. Dual role of ancient ubiquitous protein 1 (AUP1) in lipid droplet accumulation and endoplasmic reticulum (ER) protein quality control. J Biol Chem. 2011;286:37602-14 pubmed publisher
    ..These findings indicate a possible connection between ER protein quality control and lipid droplets. ..
  15. Oh R, Bai X, Rommens J. Human homologs of Ubc6p ubiquitin-conjugating enzyme and phosphorylation of HsUbc6e in response to endoplasmic reticulum stress. J Biol Chem. 2006;281:21480-90 pubmed
    ..We have undertaken characterization studies of two human homologs, hsUbc6 and hsUbc6e. Both possess tail-anchored protein motifs, display high conservation in their catalytic domains, and are ..
  16. Kim B, Zavacki A, Curcio Morelli C, Dentice M, Harney J, Larsen P, et al. Endoplasmic reticulum-associated degradation of the human type 2 iodothyronine deiodinase (D2) is mediated via an association between mammalian UBC7 and the carboxyl region of D2. Mol Endocrinol. 2003;17:2603-12 pubmed
    ..Mammalian UBC6 (MmUBC6) does not directly associate with D2 but can associate with a complex containing UBC7 and D2...
  17. Tyler R, Pearce M, Shaler T, Olzmann J, Greenblatt E, Kopito R. Unassembled CD147 is an endogenous endoplasmic reticulum-associated degradation substrate. Mol Biol Cell. 2012;23:4668-78 pubmed publisher
    ..These data establish unassembled CD147(CG) as an endogenous, constitutive ERAD substrate of the OS-9/SEL1L/Hrd1 pathway. ..
  18. Menon M, Tiedje C, Lafera J, Ronkina N, Konen T, Kotlyarov A, et al. Endoplasmic reticulum-associated ubiquitin-conjugating enzyme Ube2j1 is a novel substrate of MK2 (MAPKAP kinase-2) involved in MK2-mediated TNF? production. Biochem J. 2013;456:163-72 pubmed publisher
    ..Using phosphoproteomics we identified the ER (endoplasmic reticulum)-associated ubiquitin-conjugating enzyme Ube2j1 as a potential substrate of MK2...
  19. Younger J, Chen L, Ren H, Rosser M, Turnbull E, Fan C, et al. Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator. Cell. 2006;126:571-82 pubmed
    ..We identified an ER membrane-associated ubiquitin ligase complex containing the E3 RMA1, the E2 Ubc6e, and Derlin-1 that cooperates with the cytosolic Hsc70/CHIP E3 complex to triage CFTR and CFTR Delta F508...
  20. Imai Y, Soda M, Hatakeyama S, Akagi T, Hashikawa T, Nakayama K, et al. CHIP is associated with Parkin, a gene responsible for familial Parkinson's disease, and enhances its ubiquitin ligase activity. Mol Cell. 2002;10:55-67 pubmed
    ..Furthermore, CHIP enhanced the ability of Parkin to inhibit cell death induced by Pael-R. Taken together, these results indicate that CHIP is a mammalian E4-like molecule that positively regulates Parkin E3 activity. ..
  21. Claessen J, Mueller B, Spooner E, Pivorunas V, Ploegh H. The transmembrane segment of a tail-anchored protein determines its degradative fate through dislocation from the endoplasmic reticulum. J Biol Chem. 2010;285:20732-9 pubmed publisher
    ..b>UBC6e is a tail-anchored E2 ubiquitin-conjugating enzyme that is part of a dislocation complex nucleated by the ER-..
  22. Elangovan M, Chong H, Park J, Yeo E, Yoo Y. The role of ubiquitin-conjugating enzyme Ube2j1 phosphorylation and its degradation by proteasome during endoplasmic stress recovery. J Cell Commun Signal. 2017;: pubmed publisher
    The human Ube2j1 and Ube2j2 are the only ubiquitin-conjugating enzymes (E2s) that are localized to endoplasmic reticulum (ER) through its C-terminal transmembrane domains...
  23. Kalia S, Lee S, Smith P, Liu L, Crocker S, Thorarinsdottir T, et al. BAG5 inhibits parkin and enhances dopaminergic neuron degeneration. Neuron. 2004;44:931-45 pubmed
    ..This contrasts with the antideath functions ascribed to BAG family members and suggests a potential role for BAG5 in promoting neurodegeneration in sporadic PD through its functional interactions with parkin and Hsp70. ..
  24. Fernández Lloris R, Osses N, Jaffray E, Shen L, Vaughan O, Girwood D, et al. Repression of SOX6 transcriptional activity by SUMO modification. FEBS Lett. 2006;580:1215-21 pubmed
    ..Furthermore, co-expression of SOX6 with SUMO2 results in the appearance of SOX6 in a punctate nuclear pattern that colocalized with promyelocytic leukemia protein, which was partially abolished by mutations in SOX6 sumoylation sites. ..