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Rabbit Anti-UBE3A antibody (bs-11776R)
~~~促销,代码KT001~~~
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说明书: 100ul  200ul
100ul/1680.00元
200ul/2480.00元
大包装/询价

产品编号 bs-11776R
英文名称 UBE3A
中文名称 人乳头状瘤病毒E6相关蛋白抗体
别    名 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.  
研究领域 肿瘤  细胞生物  染色质和核信号  神经生物学  信号转导  细胞周期蛋白  表观遗传学  泛素  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 105kDa
细胞定位 细胞核 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human UBE3A/E6-AP:701-800/875 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed PubMed
产品介绍 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

Entrez Gene: 361585 Rat

Omim: 601623 Human

SwissProt: Q05086 Human

SwissProt: Q96GR7 Human

SwissProt: O08759 Mouse

Unigene: 598862 Human

Unigene: 9002 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品图片
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(bs-11776R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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