Posttranslational modification of proteins by the addition of the small protein SUMO (see SUMO1, MIM 601912), or sumoylation, regulates protein structure and intracellular localization. SAE1 (MIM 613294) and UBA2 form a heterodimer that functions as a SUMO-activating enzyme for the sumoylation of proteins (Okuma et al., 1999 [PubMed 9920803]).[supplied by OMIM, Mar 2010],UBA2,ARX,HRIHFB2115,SAE2,Cell Biology & Developmental Biology,Ubiquitin,Ubiquitin-Proteasome Signaling Pathway,UBA2