Gene Symbol: Ube3a
Description: ubiquitin protein ligase E3A
Alias: ubiquitin-protein ligase E3A
Species: rat
Products:     Ube3a

Top Publications

  1. Kishino T, Lalande M, Wagstaff J. UBE3A/E6-AP mutations cause Angelman syndrome. Nat Genet. 1997;15:70-3 pubmed
    ..We have found that a 15q inversion inherited by an AS child from her normal mother disrupts the 5' end of the UBE3A (E6-AP) gene, the product of which functions in protein ubiquitination...
  2. Valluy J, Bicker S, Aksoy Aksel A, Lackinger M, Sumer S, Fiore R, et al. A coding-independent function of an alternative Ube3a transcript during neuronal development. Nat Neurosci. 2015;18:666-73 pubmed publisher
    The E3 ubiquitin ligase Ube3a is an important regulator of activity-dependent synapse development and plasticity. Ube3a mutations cause Angelman syndrome and have been associated with autism spectrum disorders (ASD)...
  3. Huibregtse J, Scheffner M, Beaudenon S, Howley P. A family of proteins structurally and functionally related to the E6-AP ubiquitin-protein ligase. Proc Natl Acad Sci U S A. 1995;92:2563-7 pubmed
  4. Huang L, Kinnucan E, Wang G, Beaudenon S, Howley P, Huibregtse J, et al. Structure of an E6AP-UbcH7 complex: insights into ubiquitination by the E2-E3 enzyme cascade. Science. 1999;286:1321-6 pubmed
    ..The crystal structure of the E6AP hect domain bound to the UbcH7 ubiquitin-conjugating enzyme (E2) reveals the determinants of E2-E3 specificity and provides insights into the transfer of ubiquitin from the E2 to the E3. ..
  5. Smith C, DeVera D, Lamb D, Nawaz Z, Jiang Y, Beaudet A, et al. Genetic ablation of the steroid receptor coactivator-ubiquitin ligase, E6-AP, results in tissue-selective steroid hormone resistance and defects in reproduction. Mol Cell Biol. 2002;22:525-35 pubmed
  6. Khan O, Fu G, Ismail A, Srinivasan S, Cao X, Tu Y, et al. Multifunction steroid receptor coactivator, E6-associated protein, is involved in development of the prostate gland. Mol Endocrinol. 2006;20:544-59 pubmed
    ..Collectively, our data suggest that E6-AP deletion attenuates the growth and development of the prostate gland by interfering with AR function as well as by stimulating p53-mediated apoptosis. ..
  7. Shirakura M, Murakami K, Ichimura T, Suzuki R, Shimoji T, Fukuda K, et al. E6AP ubiquitin ligase mediates ubiquitylation and degradation of hepatitis C virus core protein. J Virol. 2007;81:1174-85 pubmed
    ..We propose that the E6AP-mediated ubiquitin-proteasome pathway may affect the production of HCV particles through controlling the amounts of viral nucleocapsid protein. ..
  8. LaVoie M, Cortese G, Ostaszewski B, Schlossmacher M. The effects of oxidative stress on parkin and other E3 ligases. J Neurochem. 2007;103:2354-68 pubmed
  9. Caffino L, Giannotti G, Malpighi C, Racagni G, Filip M, Fumagalli F. Long-term abstinence from developmental cocaine exposure alters Arc/Arg3.1 modulation in the rat medial prefrontal cortex. Neurotox Res. 2014;26:299-306 pubmed publisher
    ..1 translation together with reduced expression of Ubiquitin-protein ligase E3A (Ube3a) that normally causes Arc/Arg3.1 protein degradation via ubiquitination...

More Information


  1. Silva Santos S, van Woerden G, Bruinsma C, Mientjes E, Jolfaei M, Distel B, et al. Ube3a reinstatement identifies distinct developmental windows in a murine Angelman syndrome model. J Clin Invest. 2015;125:2069-76 pubmed publisher
    ..AS) is a severe neurodevelopmental disorder that results from loss of function of the maternal ubiquitin protein ligase E3A (UBE3A) allele. Due to neuron-specific imprinting, the paternal UBE3A copy is silenced...
  2. Hatakeyama S, Jensen J, Weissman A. Subcellular localization and ubiquitin-conjugating enzyme (E2) interactions of mammalian HECT family ubiquitin protein ligases. J Biol Chem. 1997;272:15085-92 pubmed
    ..Furthermore, the presence of two E2 binding sites within Nedd-4 suggests models for ubiquitination involving multiple E2 enzymes associated with E3s. ..
  3. Wu Baer F, Lagrazon K, Yuan W, Baer R. The BRCA1/BARD1 heterodimer assembles polyubiquitin chains through an unconventional linkage involving lysine residue K6 of ubiquitin. J Biol Chem. 2003;278:34743-6 pubmed
    ..The ability of BRCA1/BARD1 to form K6-linked polyubiquitin chains suggests that it may impart unique cellular properties to its natural enzymatic substrates. ..
  4. Dhananjayan S, Ramamoorthy S, Khan O, Ismail A, Sun J, Slingerland J, et al. WW domain binding protein-2, an E6-associated protein interacting protein, acts as a coactivator of estrogen and progesterone receptors. Mol Endocrinol. 2006;20:2343-54 pubmed
    ..Taken together, our data establish the role of WBP-2 and YAP as coactivators for ER and PR transactivation pathways. ..
  5. Lee Y, Giovinazzo D, Kang H, Lee Y, Jeong J, Doulias P, et al. Protein microarray characterization of the S-nitrosoproteome. Mol Cell Proteomics. 2014;13:63-72 pubmed publisher
    ..We identify eight ubiquitin E3 ligases, RNF10, RNF11, RNF41, RNF141, RNF181, RNF208, WWP2, and UBE3A, whose activities are modulated by S-nitrosylation, providing a unique regulatory mechanism of the ubiquitin ..
  6. Gossan N, Zhang F, Guo B, Jin D, Yoshitane H, Yao A, et al. The E3 ubiquitin ligase UBE3A is an integral component of the molecular circadian clock through regulating the BMAL1 transcription factor. Nucleic Acids Res. 2014;42:5765-75 pubmed publisher
    ..Here, we show that activating UBE3A (by expressing viral oncogenes E6/E7) disrupts circadian oscillations in mouse embryonic fibroblasts, measured ..
  7. Meng L, Ward A, Chun S, Bennett C, Beaudet A, Rigo F. Towards a therapy for Angelman syndrome by targeting a long non-coding RNA. Nature. 2015;518:409-12 pubmed publisher
    ..It is caused by maternal deficiency of the imprinted gene UBE3A, encoding an E3 ubiquitin ligase...