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E6-associating protein (E6-AP), also designated ubiquitin protein ligase E3A(UBE3A), is a component of the ubiquitin-mediated proteolytic pathway thatselectively 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 facil-itated by the enzymatic activity of E3 ubiquitin ligase family members such asE6-AP. Several substrates of E6-AP have been identified and include the tumorsuppressor protein p53 and the mammalian homolog of Rad23, HHR23A.Previous studies have indicated that E6-AP associates with the humanpapillomavirus E6 oncogene, which forms a complex with p53 and therebypotentiates E6-AP mediated ubiquitination of p53. Genetic mutations thatimpair E6-AP activity result in the accumulation of p53 in the cytoplasm, andin many instances, these mutations are associated with the development ofthe rare neurodevelopmental disorder Angelman syndrome (AS), which ischaracterized by severe motor dysfunction and mental retardation.
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