Recombinant Mouse Ubiquitin-protein ligase E3A (Ube3a), partial

1. Paternal UBE3A also partially colocalizes with a marker of neural progenitors, SOX2, implying that relaxed or incomplete imprinting of paternal Ube3a reflects an overall immature molecular phenotype PMID: 27306933
2. Using in vivo patch-clamp electrophysiology, study measured the visually evoked responses to square-wave drifting gratings in L2/3 regular-spiking (RS) neurons in control mice, Ube3a-deficient mice (Angelman syndrome model), and mice in which Ube3a was conditionally reinstated in GABAergic neurons; found that Ube3a-deficient mice exhibited enhanced pyramidal neuron excitability in vivo as well as weaker orientation tuning PMID: 28468997
3. Findings show neuronal overexpression of Ube3a isoform 2 causes phenotypes translatable to neurodevelopmental disorders. PMID: 29016856
4. Findings suggest that aberrant function of Ube3a could influence the progression of AD and restoring normal level of Ube3a might be beneficial for AD. PMID: 29016862
5. Encephalomyocarditis virus (EMCV) 3C protease accumulates to higher levels in EMCV-infected E6AP knockdown cells than in control cells, indicating a role for E6AP in in vivo 3C protease concentration regulation. PMID: 29054411
6. The expression of three tumor suppressor genes encoded in the INK4/ARF locus (p15(INK4b), p16(INK4a), and p19(ARF)) was decreased in E6AP(-/-) embryo fibroblasts. PMID: 28074012
7. Maternal loss of Ube3a affects nociception via a central, but not peripheral mechanism. PMID: 28931574
8. Our findings suggest that UBE3A may act locally to regulate individual synapses while also mediating global, neuronwide influences through the regulation of gene transcription PMID: 27339004
9. We report that mice with maternally-inherited deletions of Ube3a that models Angelman syndrome show an increased social preference/interaction. PMID: 28411125
10. increasing UBE3A in the nucleus downregulates the glutamatergic synapse organizer Cbln1, which is needed for sociability in mice PMID: 28297715
11. Based on our findings, we propose that imprinting of UBE3A does not function to reduce the dosage of UBE3A in neurons but rather to regulate some other, as yet unknown, aspect of gene expression or protein function PMID: 28515788
12. the role of UBE3A is investigated in neurite contact guidance during neuronal development, is reported. PMID: 26845073
13. Results substantiate GABAergic Ube3a loss as the principal cause of circuit hyperexcitability in Angelman syndrome mice, lending insight into ictogenic mechanisms in AS. PMID: 27021170
14. Loss of Ube3a from tyrosine hydroxylase-expressing neurons impairs mesoaccumbal, non-canonical GABA co-release and enhances reward-seeking behaviour measured by optical self-stimulation. PMID: 26869263
15. Results suggest that the phenotypes observed in Angelman syndrome mice may be modulated by factors independent of Ube3a genotype PMID: 26028516
16. UBE3A dampens ERK pathway signalling in HPV E6 transformed HeLa cells PMID: 25815718
17. The normal window of development in Angelman syndrome patients is supported by an incompletely silenced paternal allele in developing neurons, resulting in a relative preservation of Ube3a expression during this crucial epoch of early development. PMID: 25894543
18. Inactivation of Ube3a expression elevates BMAL1 levels in brain regions that control circadian behavior of AS-model mice, indicating an important role for Ube3a in modulating BMAL1 turnover. PMID: 25660546
19. This study demonstrated that abnormal sleep patterns arise from a deficit in accumulation of sleep drive, uncovering the Ube3a gene as a novel genetic regulator of sleep homeostasis PMID: 26446213
20. The deficiency of Ube3a in Huntington's disease (HD) mice brain also caused significant increase in global aggregates load, and these aggregates were less ubiquitinated when compared with age-matched HD mice. PMID: 25027318
21. There are distinct neurodevelopmental windows when Ube3a restoration rescues Angelman-syndrome-like phenotypes. Motor deficits are rescued in adolescence. Anxiety, repetitive behavior, and epilepsy are rescued in early development. PMID: 25866966
22. Mature oligodendrocytes express Ube3a in the cortex and white matter tracts during development. PMID: 24254964
23. findings provide novel insight into the regulation of Ube3a by synaptic activity and its potential role in kinase regulation PMID: 24434871
24. These studies demonstrate the feasibility and utility of unsilencing the paternal copy of Ube3a via targeting Ube3a-ATS as a treatment for Angelman syndrome. PMID: 24385930
25. Activating UBE3A disrupts circadian oscillations in mouse embryonic fibroblasts and rhythms in endogenous mRNA and protein levels of BMAL1. PMID: 24728990
26. Aging-dependent Ube3a levels result in differential ubiquitination and degradation of Htt fragments, thereby contributing to the age-related neurotoxicity of mutant Htt. PMID: 24706802
27. This study demonistrated that Changes in mGlu5 receptor-dependent synaptic plasticity and coupling to homer proteins in the hippocampus of Ube3A hemizygous mice. PMID: 24672001
28. These results demonstrate that UBE3A plays a role in MC1R transcriptional regulation. PMID: 21733131
29. E6AP may negatively control adipogenesis by inhibiting C/EBPalpha expression. PMID: 23762344
30. MeCP2 and E6AP play a role in the transcriptional control of common target gene expression. PMID: 23791832
31. This study described a novel phenotype of severely distended Golgi cisternae in the Angelman syndrome model mouse. PMID: 23447592
32. The present study is the first to determine that the Ube3a protein ablation seen in the Angelman syndrome mouse model is also characteristic of Angelman syndrome patients PMID: 22560727
33. findings showed that Ube3a-ATS is an atypical RNAPII transcript that functions to suppress paternal Ube3a expression PMID: 22493002
34. the loss of function of Ube3a might be associated with the synaptic abnormalities observed in HD. PMID: 22787151
35. study reports that Ube3a regulates glucocorticoid receptor (GR) transactivation; the GR signaling pathway is disrupted in Ube3a-maternal-deficient mice brain that eventually leads to increased susceptibility to stress and anxiety in these Angelman syndrome mice PMID: 22215440
36. This study demonistrated that Ube3a deficices mice product produces an excitatory/inhibitory imbalance through neuron type-specific synaptic in visual cortex. PMID: 22681684
37. our study implicates E6AP as an important regulator of the cellular response to stress, in particular through the regulation of replicative and oncogene-induced senescence. PMID: 21927031
38. The results suggest that Ube3a gene dosage may contribute to the autism traits of individuals with maternal 15q11-13 duplication and support the idea that increased E3A ubiquitin ligase gene dosage results in reduced excitatory synaptic transmission. PMID: 21974935
39. In a mouse model of Angelman syndrome, since hippocampal and Purkinje cells have the highest expression of UBE3A in a mouse model of Angelman syndrome, these cells would have the greatest sensitivity to the UBE3A m-\p+ maternal\paternal genotype. PMID: 19563863
40. HERC2 acts as a regulator of E6AP. PMID: 21493713
41. The levels of total Akt and phosphorylated Akt (active Akt) are increased in E6-AP overexpressing prostate gland and LNCaP cells suggesting that E6-AP regulates the PI3K-Akt signaling pathway. PMID: 20826237
42. Data show RNA interference that targets Ube3a in P19 cells caused downregulation of Mc1r and Nr4a2, whereas overexpression of Ube3a results in the upregulation of Mc1r and Nr4a2. PMID: 20571502
43. Ube3a exhibits brain cell type-specific imprinting, with monoallelic expression from the maternal allele in neurons, but biallelic expression in glial cells. The antisense Ube3a transcript is reciprocally imprinted only in neurons. PMID: 12668607
44. Ube3a expression is primarily neuronal in all brain regions and present in GABAergic interneurons as well as principal neurons. PMID: 20423730
45. E6-associated protein is required for human papillomavirus type 16 E6 to cause cervical cancer. PMID: 20530688
46. polycomb protein Ring1B regulation by self-ubiquitination or by E6-AP may have implications to the pathogenesis of Angelman syndrome PMID: 20351251
47. As demonstrated by optical imaging, rapid ocular dominance plasticity after brief monocular deprivation was severely impaired during the critical period in the visual cortex of Ube3a maternal-deficient (m-/p+) mice. PMID: 20212164
48. Disruption of Ube3A function in neurons leads to an increase in Arc expression and a concomitant deregulation in AMPA receptors at synapses; which may contribute to the cognitive dysfunction that occurs in Angelman Syndrome. PMID: 20211139
49. The imprinted Ube3a antisense transcript is regulated by the U exons rather than Snurf/Snrpn exon 1 (Ube3a antisense RNA). PMID: 15226413
50. loss of E6AP catalytic activity and improper regulation of E6AP substrate are important in the development of Angelman syndrome PMID: 15263005

Show More

Hide All

KEGG: mmu:22215

STRING: 10090.ENSMUSP00000103161

UniGene: Mm.9002