Gene Symbol: Uba1
Description: ubiquitin-like modifier activating enzyme 1
Alias: A1S9, Sbx, Ube-1, Ube1x, ubiquitin-like modifier-activating enzyme 1, ubiquitin-activating enzyme E1, Chr X
Species: mouse
Products:     Uba1

Top Publications

  1. Imai N, Kaneda S, Nagai Y, Seno T, Ayusawa D, Hanaoka F, et al. Cloning and sequence of a functionally active cDNA encoding the mouse ubiquitin-activating enzyme E1. Gene. 1992;118:279-82 pubmed
    ..The 3495-bp cDNA encodes 1058 amino acids (aa), and shares extensive homology with the human E1 enzyme at both the nucleotide and aa sequence levels. ..
  2. Salido E, Passage M, Yen P, Shapiro L, Mohandas T. An evaluation of the inactive mouse X chromosome in somatic cell hybrids. Somat Cell Mol Genet. 1993;19:65-71 pubmed
    The expression of mouse Zfx, Rps4, Ube1x, and Xist was evaluated in hamster-mouse somatic cell hybrids containing either an active or an inactive mouse X chromosome using polymerase chain reaction of reverse transcribed RNA (RT-PCR)...
  3. Tokumoto M, Nagahama Y, Tokumoto T. Molecular cloning of cDNA encoding a ubiquitin-activating enzyme (E1) from goldfish (Carassius auratus) and expression analysis of the cloned gene. Biochim Biophys Acta. 2000;1492:259-63 pubmed
    ..Recombinant goldfish E1 could transfer ubiquitin to cyclin-selective ubiquitin-conjugating enzyme. Tissue distribution revealed a single 4.0-kb message ubiquitous among tissues. ..
  4. Ehrmann I, Ellis P, Mazeyrat S, Duthie S, Brockdorff N, Mattei M, et al. Characterization of genes encoding translation initiation factor eIF-2gamma in mouse and human: sex chromosome localization, escape from X-inactivation and evolution. Hum Mol Genet. 1998;7:1725-37 pubmed
    ..These results shed light on the evolution of X-Y homologous genes. ..
  5. Saito Y, Doi K, Yamagishi N, Ishihara K, Hatayama T. Screening of Hsp105alpha-binding proteins using yeast and bacterial two-hybrid systems. Biochem Biophys Res Commun. 2004;314:396-402 pubmed
    ..The interaction was validated by the results of a pull-down assay and indirect immunofluorescence analysis. The significance of Hsp105alpha and Hsp105alpha-binding proteins in cells was discussed. ..
  6. Collignon J, Sockanathan S, Hacker A, Cohen Tannoudji M, Norris D, Rastan S, et al. A comparison of the properties of Sox-3 with Sry and two related genes, Sox-1 and Sox-2. Development. 1996;122:509-20 pubmed
    ..However our findings imply that if this is true, then Sry has undergone concomitant changes resulting in loss of CNS expression and altered DNA-binding properties. ..
  7. Nagai Y, Kaneda S, Nomura K, Yasuda H, Seno T, Yamao F. Ubiquitin-activating enzyme, E1, is phosphorylated in mammalian cells by the protein kinase Cdc2. J Cell Sci. 1995;108 ( Pt 6):2145-52 pubmed
    ..These findings suggest that E1 is a target of Cdc2 kinase in the cell, implying that the ubiquitin system may be dynamically involved in cell cycle control through phosphorylation of this key enzyme. ..
  8. CaubĂ­n J, Iglesias T, Bernal J, Munoz A, Marquez G, Barbero J, et al. Isolation of genomic DNA fragments corresponding to genes modulated in vivo by a transcription factor. Nucleic Acids Res. 1994;22:4132-8 pubmed
    ..This method, extensive to other ligand-modulated transcription factors, might be useful to all transcription factors with slight modifications. ..
  9. Chun K, Mackay N, Petrova Benedict R, Robinson B. Pyruvate dehydrogenase deficiency due to a 20-bp deletion in exon II of the pyruvate dehydrogenase (PDH) E1 alpha gene. Am J Hum Genet. 1991;49:414-20 pubmed
    ..The 20-bp deletion was not found in either parent's genomic DNA, and hence we conclude that the mutation must have occurred de novo, either in the germ-line cells or immediately following fertilization. ..

More Information


  1. Levy N, de Boer P, Mattei J, Mitchell M. Molecular analysis of an isoYq chromosome in a sterile male mouse. Mamm Genome. 1996;7:608-9 pubmed
  2. Yang U, Yang H, Kim J, Lee T. The functional role of UBA1 cysteine-278 in ubiquitination. Biochem Biophys Res Commun. 2012;427:587-92 pubmed publisher
    Although total UBA1 levels were unchanged, after oxidation for 60 min, we observed dramatic changes in the levels of BIAM-labeled UBA1 in both the membrane and cytosol fractions that suggested oxidative stress induces translocation of ..
  3. Ciechanover A. The ubiquitin-proteasome pathway: on protein death and cell life. EMBO J. 1998;17:7151-60 pubmed
  4. Levy N, Navarro A, Bishop C, Mitchell M. The ubiquitin-activating enzyme E1 homologous genes on the mouse Y chromosome (Ube1y) represent one functional gene and six partial pseudogenes. Mamm Genome. 2000;11:164-8 pubmed
  5. Chou A, Maidment N, Klintenberg R, Casida J, Li S, Fitzmaurice A, et al. Ziram causes dopaminergic cell damage by inhibiting E1 ligase of the proteasome. J Biol Chem. 2008;283:34696-703 pubmed publisher
    ..Chronic exposure to widely used dithiocarbamate fungicides may contribute to the development of PD, and elucidation of its mechanism would identify a new potential therapeutic target...
  6. Szczepanowski R, Filipek R, Bochtler M. Crystal structure of a fragment of mouse ubiquitin-activating enzyme. J Biol Chem. 2005;280:22006-11 pubmed
    ..We show that the domain is organized around a conserved folding motif that is also present in the NEDD8- and SUMO-activating enzymes, and we propose a tentative model for full-length ubiquitin-activating enzyme. ..
  7. Carrel L, Clemson C, Dunn J, Miller A, Hunt P, Lawrence J, et al. X inactivation analysis and DNA methylation studies of the ubiquitin activating enzyme E1 and PCTAIRE-1 genes in human and mouse. Hum Mol Genet. 1996;5:391-401 pubmed
    ..Methylation of CpG residues at restriction sites at the 5' end of both genes on the murine inactive X chromosome is consistent with both genes being subject to X inactivation in mouse, in contrast to their expression status in humans. ..
  8. Candille S, Pardue M, McCall M, Peachey N, Gregg R. Localization of the mouse nob (no b-wave) gene to the centromeric region of the X chromosome. Invest Ophthalmol Vis Sci. 1999;40:2748-51 pubmed
    ..8 cM) and Ube1x (5.7 cM)...
  9. Sandstedt S, Tucker P. Evolutionary strata on the mouse X chromosome correspond to strata on the human X chromosome. Genome Res. 2004;14:267-72 pubmed
    ..An analysis of smaller fragments of Smcy, Smcx, Zfy, and Zfx from seven species of Mus confirmed that the strata in Mus musculus were representative of the genus Mus. ..
  10. Disteche C, Zacksenhaus E, Adler D, Bressler S, Keitz B, Chapman V. Mapping and expression of the ubiquitin-activating enzyme E1 (Ube1) gene in the mouse. Mamm Genome. 1992;3:156-61 pubmed
    ..spretus, C57BL/6J, and T(X;16)16H x M. spretus F1 female mice indicates that the mouse Ube1 gene is subject to X-Chr inactivation in vivo. This represents a new example of differences between the sex chromosomes of mouse and human. ..
  11. Wishart T, Mutsaers C, Riessland M, Reimer M, Hunter G, Hannam M, et al. Dysregulation of ubiquitin homeostasis and ?-catenin signaling promote spinal muscular atrophy. J Clin Invest. 2014;124:1821-34 pubmed publisher
    ..perturbations in ubiquitin homeostasis, including reduced levels of ubiquitin-like modifier activating enzyme 1 (UBA1)...
  12. Chen C, Meng Y, Wang L, Wang H, Tian C, Pang G, et al. Ubiquitin-activating enzyme E1 inhibitor PYR41 attenuates angiotensin II-induced activation of dendritic cells via the I?Ba/NF-?B and MKP1/ERK/STAT1 pathways. Immunology. 2014;142:307-19 pubmed publisher
    ..Ubiquitin-activating enzyme (E1/Uba1) is the common first step in ubiquitylation, which decides whether or not the modified protein is ultimately ..
  13. Wade B, Wang C, Yan S, Bhat K, Huang B, Li S, et al. Ubiquitin-activating enzyme activity contributes to differential accumulation of mutant huntingtin in brain and peripheral tissues. J Neurosci. 2014;34:8411-22 pubmed publisher
    ..Our findings suggest that decreased targeting of misfolded Htt to the proteasome for degradation via Ube1 may underlie the preferential accumulation of toxic forms of mHtt in the brain and its selective neurodegeneration. ..
  14. Odorisio T, Mahadevaiah S, McCarrey J, Burgoyne P. Transcriptional analysis of the candidate spermatogenesis gene Ube1y and of the closely related Ube1x shows that they are coexpressed in spermatogonia and spermatids but are repressed in pachytene spermatocytes. Dev Biol. 1996;180:336-43 pubmed
    ..Ube1y, together with a ubiquitously expressed homologue on the X chromosome (Ube1x), encodes ubiquitin-activating enzyme E1, an enzyme essential for eukaryotic cell proliferation...
  15. Trausch J, Grenfell S, Handley Gearhart P, Ciechanover A, Schwartz A. Immunofluorescent localization of the ubiquitin-activating enzyme, E1, to the nucleus and cytoskeleton. Am J Physiol. 1993;264:C93-102 pubmed
    ..The variable distribution of E1 among cell lines, including its apparent cytoskeletal association, suggests pleiotropic functions of this enzyme and the ubiquitin-conjugating system...
  16. Santori F, Holmberg K, Ostrov D, Gascoigne N, Vukmanovic S. Distinct footprints of TCR engagement with highly homologous ligands. J Immunol. 2004;172:7466-75 pubmed
    ..These findings explain differences in functional versatility of TCR ligands, as well as anomalies in the relationship between affinity/avidity of the TCR for the peptide/MHC and cellular responses of T cells. ..
  17. Hurst L. Embryonic growth and the evolution of the mammalian Y chromosome. II. Suppression of selfish Y-linked growth factors may explain escape from X-inactivation and rapid evolution of Sry. Heredity (Edinb). 1994;73 ( Pt 3):233-43 pubmed
    ..The case for fast sequence evolution as the product of maternal/foetal conflict is strengthened by consideration of the rapid evolution of placental lactogens in both ruminants and rodents. ..
  18. Chang B, Li W. Estimating the intensity of male-driven evolution in rodents by using X-linked and Y-linked Ube 1 genes and pseudogenes. J Mol Evol. 1995;40:70-7 pubmed
    ..From the intron sequences of the Ube 1 genes, we calculated the divergence of the Y-linked genes (Y = 0.161) and that of the X-linked genes (X = 0...
  19. Kalantry S, Purushothaman S, Bowen R, Starmer J, Magnuson T. Evidence of Xist RNA-independent initiation of mouse imprinted X-chromosome inactivation. Nature. 2009;460:647-51 pubmed publisher
    ..Xp-linked gene silencing associated with mouse imprinted XCI, therefore, can initiate in the embryo independently of Xist RNA. ..
  20. Williams L, Kalantry S, Starmer J, Magnuson T. Transcription precedes loss of Xist coating and depletion of H3K27me3 during X-chromosome reprogramming in the mouse inner cell mass. Development. 2011;138:2049-57 pubmed publisher
  21. Mugford J, Yee D, Magnuson T. Failure of extra-embryonic progenitor maintenance in the absence of dosage compensation. Development. 2012;139:2130-8 pubmed publisher
    ..Conversely, unlike the epiblast, in which XCI is not required for progenitor cell maintenance, we demonstrate that dosage compensation is indispensable for the maintenance of trophoblast progenitors. ..
  22. Kay G, Ashworth A, Penny G, Dunlop M, Swift S, Brockdorff N, et al. A candidate spermatogenesis gene on the mouse Y chromosome is homologous to ubiquitin-activating enzyme E1. Nature. 1991;354:486-9 pubmed
    The human X-linked gene A1S9 complements a temperature-sensitive cell-cycle mutation in mouse L cells, and encodes the ubiquitin-activating enzyme E1...
  23. Mitchell M, Woods D, Tucker P, Opp J, Bishop C. Homology of a candidate spermatogenic gene from the mouse Y chromosome to the ubiquitin-activating enzyme E1. Nature. 1991;354:483-6 pubmed
    ..The critical role of this enzyme in nuclear DNA replication together with the testis-specific expression of Sby suggests Sby as a candidate for the spermatogenic gene Spy. ..
  24. Xu J, Burgoyne P, Arnold A. Sex differences in sex chromosome gene expression in mouse brain. Hum Mol Genet. 2002;11:1409-19 pubmed
    ..Six X-linked homologues (Usp9x, Ube1x, Smcx, Eif2s3x, Utx and Dbx) were also expressed in brain, and in adulthood all of these transcripts were ..
  25. Gregg R, Mukhopadhyay S, Candille S, Ball S, Pardue M, McCall M, et al. Identification of the gene and the mutation responsible for the mouse nob phenotype. Invest Ophthalmol Vis Sci. 2003;44:378-84 pubmed
    ..The nob mouse is a model for human CSNB1. This model will be useful in defining the role of nyctalopin in signal transmission between photoreceptors and retinal bipolar cells. ..