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Product Name UBE3A Chinese Name 人乳头状瘤病毒E6相关蛋白抗体 Alias ANCR; Angelman syndrome; AS; CTCL tumor antigen se37 2; E6 AP; E6-AP; E6AP antibody E6AP ubiquitin protein ligase; E6AP ubiquitin protein ligase; E6AP ubiquitin-protein ligase; EPVE6AP; HPVE6A; Human papilloma virus E6 associated protein Angelman syndrome; Human papilloma virus E6 associated protein; Human papillomavirus E6-associated protein; NY REN 54 antigen; NY REN 54 antigen; Oncogenic protein associated protein E6 AP; Oncogenic protein associated protein E6AP; Oncogenic protein-associated protein E6-AP; Renal carcinoma antigen NY REN 54; Renal carcinoma antigen NY-REN-54; UBE 3A; Ube3a; UBE3A protein; UBE3A_HUMAN; Ubiquitin protein ligase E3A; Ubiquitin-protein ligase E3A. Research Area Tumour Cell biology Chromatin and nuclear signals Neurobiology Signal transduction Cyclin Epigenetics Ubiquitin Immunogen Species Rabbit Clonality Polyclonal React Species Human, (predicted: Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 105kDa Cellular localization The nucleus Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human UBE3A/E6-AP: 701-800/875 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail E6-associating protein is a component of the ubiquitin-mediated proteolytic pathway, which selectively targets proteins for degradation by the 26S proteasome. Ubiquitin (Ub) is directly conjugated to protein substrates by the transfer of Ub from an E2 ubiquitin conjugating enzyme to the target protein. This conjugation is facilitated by the enzymatic activity of E3 ubiquitin ligase family members such as E6-AP. Several substrates of E6-AP have been identified and include the tumor suppressor protein p53 and the mammalian homolog of Rad23, HHR23A. Previous studies have indicated that E6-AP associates with the human papillomavirus E6 oncogene, which complexes with p53 and thereby potentiates E6-AP mediated ubiquitination of p53. Genetic mutations that impair E6-AP activity result in the accumulation of p53 in the cytoplasm, and, in many instances, these mutations are associated with the development of the rare neurodevelopmental disorder Angelman syndrome (AS), which is characterized by severe motor dysfunction and mental retardation.
Function:
E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and transfers it to its substrates. Several substrates have been identified including the RAD23A and RAD23B, MCM7 (which is involved in DNA replication), annexin A1, the PML tumor suppressor, and the cell cycle regulator CDKN1B. Additionally, may function as a cellular quality control ubiquitin ligase by helping the degradation of the cytoplasmic misfolded proteins. Finally, UBE3A also promotes its own degradation in vivo.
Subcellular Location:
Nucleus.
Post-translational modifications:
Phosphorylated upon DNA damage, probably by ATM or ATR.
DISEASE:
Defects in UBE3A are a cause of Angelman syndrome (AS) [MIM:105830]; also known as 'happy puppet syndrome'. AS is characterized by features of severe motor and intellectual retardation, microcephaly, ataxia, frequent jerky limb movements and flapping of the arms and hands, hypotonia, hyperactivity, hypopigmentation, seizures, absence of speech, frequent smiling and episodes of paroxysmal laughter, and an unusual facies characterized by macrostomia, a large mandible and open-mouthed expression, a great propensity for protruding the tongue ('tongue thrusting'), and an occipital groove.
Similarity:
Contains 1 HECT (E6AP-type E3 ubiquitin-protein ligase) domain.
SWISS:
Q05086
Gene ID:
7337
Database links:Entrez Gene: 7337 Human
Entrez Gene: 22215 Mouse
Omim: 601623 Human
SwissProt: Q05086 Human
SwissProt: Q96GR7 Human
SwissProt: O08759 Mouse
Unigene: 598862 Human
Unigene: 9002 Mouse
Product Picture Tissue/cell: human breast carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-UBE3A Polyclonal Antibody, Unconjugated(SL11776R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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