[HTML][HTML] Angelman syndrome–associated point mutations in the Zn2+-binding N-terminal (AZUL) domain of UBE3A ubiquitin ligase inhibit binding to the proteasome
Deregulation of the HECT ubiquitin ligase UBE3A/E6AP has been implicated in Angelman
syndrome as well as autism spectrum disorders. We and others have previously identified
the 26S proteasome as one of the major UBE3A-interacting protein complexes. Here, we
characterize the interaction of UBE3A and the proteasomal subunit PSMD4 (Rpn10/S5a).
We map the interaction to the highly conserved Zn 2+-binding N-terminal (AZUL) domain of
UBE3A, the integrity of which is crucial for binding to PSMD4. Interestingly, two Angelman …
syndrome as well as autism spectrum disorders. We and others have previously identified
the 26S proteasome as one of the major UBE3A-interacting protein complexes. Here, we
characterize the interaction of UBE3A and the proteasomal subunit PSMD4 (Rpn10/S5a).
We map the interaction to the highly conserved Zn 2+-binding N-terminal (AZUL) domain of
UBE3A, the integrity of which is crucial for binding to PSMD4. Interestingly, two Angelman …