UBE3A-mediated PTPA ubiquitination and degradation regulate PP2A activity and dendritic spine morphology

Significance Deletion or loss-of-function mutations of the maternally inherited allele of UBE3A , which encodes an E3 ubiquitin ligase, lead to Angelman syndrome (AS), a developmental neurological disorder with severe intellectual disability. The consequences of UBE3A dysfunction are not well understood. Here, we demonstrate that UBE3A ubiquitinates PTPA, an activator of protein phosphatase 2A. Maternal loss of Ube3a in an AS mouse model leads...