UBA6 (Ubiquitin Like Modifier Activating Enzyme 6) is a Protein Coding gene. The UBA6 gene, located on 4q13.2, is conserved in chimpanzee, Rhesus monkey, dog, cow, mouse, rat, chicken, zebrafish, and frog. Uba6 is a homolog of the ubiquitin-activating enzyme, Uba1, and activates two ubiquitin-like proteins (UBLs), ubiquitin and FAT10. UBA6 is an alternative enzyme for ubiquitin activation in vertebrates that plays a pivotal role in early mouse development. UBA6 is widely expressed in the lymph node, appendix, and other tissues. Diseases associated with UBA6 include Ichthyosis, Congenital, Autosomal Recessive 4A, and Johanson-Blizzard Syndrome. Among its related pathways are the Metabolism of proteins and the Innate Immune System.