The ADAT3 gene encodes a protein that forms a heterodimer with the ADAT2 protein. The ADAT3-ADAT2 heterodimer functions as an enzyme that is responsible for the hydrolytic deamination of adenosine to inosine on t-RNA. This occurs at the position 34 of the anticodon stem-loop motif of t-RNA, also known as the wobble position. The ADAT3 protein is hence involved in a highly specific t-RNA modification. While the exact significance of this process is yet to be fully elucidated, it is speculated to affect the t-RNA stability and structure, while also improving its fidelity in translation.
Mutations in the gene have been associated with Mental Retardation, Autosomal Recessive 36 (MRT36), a disorder characterized by intellectual disability and esotropia.