While it is apparent that the key components of the Angelman syndrome phenotype are caused by loss of function of the maternally expressed UBE3A gene in the brain [12, 13], it is still unclear how loss of UBE3A can directly lead to the observed neurological defects including synaptic plasticity problems, ataxia, seizures, and intellectual disability [14]. Here, UBE3A is linked to cerebellar ataxia.