E6AP Rabbit anti-Human, Polyclonal, Invitrogen
Rabbit Polyclonal Antibody
Manufacturer: Invitrogen PA3843
PA3-843 detects Angelman Syndrome-Associated protein (E6-AP) from human tissues. PA3-843 has been successfully used in Western blot and immunoprecipitation procedures. By Western blot, this antibody detects a single ~100 kDa protein representing E6-AP from HeLa cell lysate. IMPORTANT: E6-AP is a very labile protein. Freeze/thaw cycling of samples results in E6-AP degradation which is observed as a prominent 50 kDa band seen in Western blots. PA3-843 antigen is bacterially expressed, full-length human E6-AP.Angelman Syndrome (AS) is a neurological disorder characterized by severe mental retardation, seizures and speech impairment, which results from the lack of a functional maternal copy of the E6-AP gene. E6-AP, or Angelman Syndrome-Associated protein, has been shown to interact with the progesterone receptor as a coactivator, enhancing the transcriptional activity of this steroid receptor. Interestingly, E6-AP (UBE3A) is also a member of the E3 ubiquitin-protein ligase family which plays a role in defining the substrate specificity of the ubiquitin-proteasome degradation system. For example, E6-AP can interact directly with the growth and tumor-suppressive protein, p53 or indirectly as part of a human papillomavirus type 16 or 18 E6 protein complex. This interaction promotes the degradation of p53 via the ubiquitin-proteosome pathway. These two functions of E6-AP, coactivation and degradation, are independent of one another. Furthermore, the coactivation function of E6-AP in AS patients remains intact and the ubiquitin-protease function of the protein was compromised. It is unclear, however, how this causes AS.
|whole serum diluted in PBS with 0.05% sodium azide|
|UBE3A, Angelman Syndrome-Associated protein|
|Immunoprecipitation, Western Blot|
|Full-length human E6-AP:GST fusion protein.|
|-20° C, Avoid Freeze/Thaw Cycles|
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