Understanding the Pathogenesis of Angelman Syndrome through Animal Models

Table 2

Cellular functions regulated by UBE3A.

Identified substrates	Cellular functions
HHR 23A, Src family tyrosine kinase Blk, P53, P27, PML tumor suppressor [48–52].	Cell growth and differentiation
Steroid hormone receptors like androgen receptor, glucocorticoid receptor, mineralocorticoid receptor [45, 46, 84].	Coactivator of steroid hormone receptors
Arc, RhoA-GEF ephexin5, Rho-GEF Pbl/ECT2 [54–58].	Synaptic function and plasticity
Polyglutamine proteins, α-synuclein, misfolded proteins [59–61].	Cellular protein quality control

Number in the brackets indicates references.