Ubiquitin-protein ligase E3A Antibody / UBE3A

E6-associating protein (E6-AP), also designated ubiquitin protein ligase E3A (UBE3A), is a component of the ubiquitin-mediated proteolytic pathway that selectively targets proteins for degradation by the 26S Proteasome. Ubiquitin (Ub) is directly conjugated to protein substrates by the transfer of Ub from anE2 ubiquitin conjugating enzyme to the target protein. This conjugation is facilitated by the enzymatic activity of E3 ubiquitin ligase family members such asE6-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 papilloma virus E6 oncogene, which forms a complex with p53 and there by 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.