Mutation of UBE3B, an E3 ubiquitin ligase, has been found to lead to Blepharophimosis-Ptosis-Intellectual-Disability Syndrome (BPID) in human infants, indicating potential involvement of UBE3B in the regulation of neuronal signaling in the brain (Basel-Vanagaite et al. 2012). Here, UBE3B is linked to syndromic intellectual disability.