Detailed Dissection of UBE3A-Mediated DDI1 Ubiquitination

Overview

Journal Front Physiol

Date 2019 May 28

PMID 31130875

Citations 8

Authors

Nagore Elu

Nerea Osinalde

Javier Beaskoetxea

Juanma Ramirez

Benoit Lectez

Kerman Aloria

Jose Antonio Rodriguez

Jesus M Arizmendi

Ugo Mayor

Affiliations

Soon will be listed here.

Abstract

The ubiquitin E3 ligase UBE3A has been widely reported to interact with the proteasome, but it is still unclear how this enzyme regulates by ubiquitination the different proteasomal subunits. The proteasome receptor DDI1 has been identified both in photoreceptor neurons and in human neuroblastoma cells in culture as a direct substrate of UBE3A. Here, we further characterize this regulation, by identifying the UBE3A-dependent ubiquitination sites and ubiquitin chains formed on DDI1. Additionally, we found one deubiquitinating enzyme that is capable of reversing the action of UBE3A on DDI1. The complete characterization of the ubiquitination pathway of an UBE3A substrate is important due to the role of this E3 ligase in rare neurological disorders as Angelman syndrome.

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