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High UBE3A expression is associated with Liver Tumorigenesis.
These data provide structural and functional insights into the dynamics of the full-length E6AP-E6-p53 enzyme-substrate complex.
UBE3A regulates an imprinted gene network involving DNA methylation and H2A.Z deposition.
ANCR overexpression inhibited NSCLC cell migration and invasion and downregulated TGF-beta1 expression, while TGF-beta1 treatment showed no significant effects on ANCR expression but promoted NSCLC cell migration and invasion.
Results from a study on gene expression variability markers in early-stage human embryos shows that UBE3A is a putative expression variability marker for the 3-day, 8-cell embryo stage.
The divergence of E6 proteins from either MAML1 or E6AP binding preference is a major event in papillomavirus evolution.
Data report the establishment and thorough characterization of a new iPSC line from a patient with Angelman syndrome, harboring a defined three-base pair deletion within the maternally inherited UBE3A allele. Using computer modeling of the mutant protein, a local destabilization around the catalytic cleft of UBE3A was proposed, likely impairing the binding of substrates.
We demonstrate that E6AP regulates p27 expression by inhibiting its transcription in an E2F1-dependent manner. Concomitant knockdown of E6AP and p27 partially restores PC cell growth, supporting the contribution of p27 to the overall effect of E6AP on prostate tumorigenesis.
novel IVS15-1G>C and c.2540 C>T mutations of the UBE3A gene probably underlie the AS in the two families
Findings show neuronal overexpression of Ube3a isoform 2 causes phenotypes translatable to neurodevelopmental disorders.
This study demonstrate the importance of the N-terminal domain of full length E6AP for diubiquitin chain specificity.
our data demonstrates that E6AP facilitates ubiquitination and subsequent degradation of G-CSFR leading to attenuation of its downstream signaling and inhibition of granulocytic differentiation.
Novel intragenic deletions within the UBE3A gene have been reported in two unrelated patients with Angelman syndrome.
Data suggest ordered binding of UBCH7-ubiquitin to E6AP Site 1 (for E6AP-Cys820/ubiquitin thioester formation) and E6AP Site 2 (for subsequent chain elongation); proximal indexation accounts for symmetric structure of E6AP, requirement for oligomerization in polyubiquitin chain formation, and mechanistic rationale for Cys820-ubiquitin thioester as platform in chain assembly. (UBCH7 = ubiquitin-conjugating enzyme UBCH7)
E6AP (UBE3A) abundance is down-regulated in a proportion of NSCLC (non-small cell lung cancer) patients, and this correlates with low p16INK4a in tumors and worse overall survival.
study has broad implications for human disorders associated with UBE3A gain or loss of function and suggests that dysfunctional UBE3A might affect additional proteins and pathways that are sensitive to proteasome activity
We found that KD of E6AP attenuates cancer cell growth by promoting cellular senescence in vivo, which correlates with restoration of tumor suppression by PML.
HCV core protein inhibits E6AP expression via DNA methylation to protect itself from ubiquitin-dependent proteasomal degradation and stimulate virus propagation.
E6AP suppresses breast cancer metastasis by regulating actin cytoskeleton remodeling through the control of ECT2 and Rho GTPase activity.
Dube3a is neither imprinted nor preferentially expressed from the maternal allele in fly neurons.
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
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
Findings suggest that aberrant function of Ube3a could influence the progression of AD and restoring normal level of Ube3a might be beneficial for AD.
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.
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.
Maternal loss of Ube3a affects nociception via a central, but not peripheral mechanism.
Our findings suggest that UBE3A may act locally to regulate individual synapses while also mediating global, neuronwide influences through the regulation of gene transcription
treatment of topotecan, a brain-penetrating topoisomerase 1 inhibitor, to HD transgenic mouse considerably improved its motor behavioural abnormalities. Finally, we show that topotecan treatment to HD mouse not only inhibits the expression of transgenic mutant huntingtin, but also at the same time induces the expression of Ube3a
We report that mice with maternally-inherited deletions of Ube3a that models Angelman syndrome show an increased social preference/interaction.
increasing UBE3A in the nucleus downregulates the glutamatergic synapse organizer Cbln1, which is needed for sociability in mice
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
the role of UBE3A is investigated in neurite contact guidance during neuronal development, is reported.
Results substantiate GABAergic Ube3a loss as the principal cause of circuit hyperexcitability in Angelman syndrome mice, lending insight into ictogenic mechanisms in AS.
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.
Results suggest that the phenotypes observed in Angelman syndrome mice may be modulated by factors independent of Ube3a genotype
UBE3A dampens ERK pathway signalling in HPV E6 transformed HeLa cells
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.
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.
Drosophila endogenously expresses Dube3a (fly UBE3A homolog) in glial cells and neurons. A robust seizure-like phenotype was observed in flies overexpressing Dube3a in glial cells, but not neurons. Glial-specific knockdown of ATPalpha also produced seizure-like behavior, and this phenotype was rescued by simultaneously overexpressing ATPalpha and Dube3a in glia.
Negative regulation of Tkv by Ube3a was conserved in mammalian cells. These results reveal a critical role for Ube3a in regulating NMJ synapse development by repressing BMP signaling. This study sheds new light onto the neuronal functions of UBE3A and provides novel perspectives for understanding the pathogenesis of UBE3A-associated disorders.
Data indicate that E3 ligase called ube3 (dube3a) is required for brain development as mutants have defective mushroom bodies.
Rpn10 is targeted for degradation by Ube3a.
In Drosophila melanogaster the human homologue ube3a regulates the actin cytoskeleton and neuronal homeostasis.
MeCP2 and E6AP play a role in the transcriptional control of common target gene expression.
Ube3a regulates serotonin and dopamine synthesis in the fly brain by increasing GTP cyclohydrolase I activity.
dube3a null mutants appear normal externally, but display abnormal locomotive behavior and circadian rhythms, and defective long-term memory; overexpression in the nervous system caused locomotion defects, dependent on the ubiquitin ligase activity
Overexpression of dUBE3A decreased dendritic branching.
In the skeletal muscle of neonate pigs, both NECD and SNRPN were maternally imprinted, while UBE3A was not imprinted.
This gene encodes an E3 ubiquitin-protein ligase, part of the ubiquitin protein degradation system. This imprinted gene is maternally expressed in brain and biallelically expressed in other tissues. Maternally inherited deletion of this gene causes Angelman Syndrome, characterized by severe motor and intellectual retardation, ataxia, hypotonia, epilepsy, absence of speech, and characteristic facies. The protein also interacts with the E6 protein of human papillomavirus types 16 and 18, resulting in ubiquitination and proteolysis of tumor protein p53. Alternative splicing of this gene results in three transcript variants encoding three isoforms with different N-termini. Additional transcript variants have been described, but their full length nature has not been determined.
CTCL tumor antigen se37-2
, E6AP ubiquitin-protein ligase
, human papilloma virus E6-associated protein
, human papillomavirus E6-associated protein
, oncogenic protein-associated protein E6-AP
, renal carcinoma antigen NY-REN-54
, ubiquitin-protein ligase E3A
, ubiquitin protein ligase E3A (human papilloma virus E6-associated protein, Angelman syndrome)
, ubiquitin protein ligase E3A
, E6-AP ubiquitin protein ligase
, ubiquitin conjugating enzyme E3A
, drosophila angelman syndrome
, angelman syndrome
, ubiquitin-protein ligase E3A-like