Specific programme: March of Dimes Foundation
UPV/EHU Partner Status:  Grantee
UPV/EHU PI: Ugo Mayor
Project start: 01/06/2015
Project end:   31/05/2018

Brief description:  The work we are proposing will help understand the mechanisms by which tightly regulated levels of the ubiquitin ligase UBE3A are critical for appropriate brain development and function. Both absence and excess of this protein in the brain lead to neurodevelopmental disorders. The lack of ubiquitination activity by UBE3A is sufficient to cause Angelman Syndrome, and excess of UBE3A has been directly
associated to dup15q associated autism. Which proteins are regulated by UBE3A in the brain is still unknown. We are using innovative technology developed in our group for the isolation, identification and validation of UBE3A ubiquitin substrates.
This project will open new horizons by its usage of the only available methodology to identify and validate, in the context of the whole organism, ubiquitination targets for a specific ubiquitin ligase.