Science · 1999 · 563 citations · 32 references
Life SciencesBiochemistryMolecular BiologyEnzymologyStructural BiochemistryStructural BiologyEnzyme FunctionEnzyme-substrate RecognitionProtein StructureStructural EnzymologyAngelman SyndromeE2 Ubiquitin-conjugating EnzymesE3 Ubiquitin Ligase Substrate RecognitionE6 OncoproteinRegulation By E3 Ubiquitin LigasesTumor Suppressor P53Ubiquitin SystemUbiquitin-mediated Proteolysis
The E6AP ubiquitin-protein ligase (E3) mediates the human papillomavirus-induced degradation of the p53 tumor suppressor in cervical cancer and is mutated in Angelman syndrome, a neurological disorder. The crystal structure of the catalytic hect domain of E6AP reveals a bilobal structure with a broad catalytic cleft at the junction of the two lobes. The cleft consists of conserved residues whose mutation interferes with ubiquitin-thioester bond formation and is the site of Angelman syndrome mutations. The crystal structure of the E6AP hect domain bound to the UbcH7 ubiquitin-conjugating enzyme (E2) reveals the determinants of E2-E3 specificity and provides insights into the transfer of ubiquitin from the E2 to the E3.
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T. Alwyn Jones, Jin-yu Zou, Sandra W. Cowan et al. · Acta Crystallographica Section A Foundations of Crystallography · 1991 · 12.7K citations