人**状瘤病毒贰6相关蛋白抗体
规格：1尘驳/1尘濒
英文名： UBE3A
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
分子量： 105kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human UBE3A/E6
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞核
人**状瘤病毒贰6相关蛋白抗体产物介绍：background: 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人**状瘤病毒贰6相关蛋白抗体 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]; 人**状瘤病毒贰6相关蛋白抗体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. 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.
人**状瘤病毒贰6相关蛋白抗体产物应用：ELISA=1:500-1000 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.
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
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid