Modification of proteins with ubiquitin (UBB; MIM 191339) or ubiquitin-like proteins controls many signaling networks and requires a ubiquitin-activating enzyme (E1), a ubiquitin conjugating enzyme (E2), and a ubiquitin protein ligase (E3). UBA6/UBE1L2 is an E1 enzyme that initiates the activation and conjugation of ubiquitin-like proteins.
