This gene encodes an arginyltransferase, an enzyme that is involved in posttranslational conjugation of arginine to N-terminal aspartate or glutamate residues. Conjugation of arginine to the N-terminal aspartate or glutamate targets proteins for ubiquitin-dependent degradation. Alternative splicing results in multiple transcript variants. ATE1 (Arginyltransferase 1) is a Protein Coding gene. Diseases associated with ATE1 include Chromosome 10Q26 Deletion Syndrome and Beare-Stevenson Cutis Gyrata Syndrome. Gene Ontology (GO) annotations related to this gene include arginyltransferase activity. Involved in the post-translational conjugation of arginine to the N-terminal aspartate or glutamate of a protein. This arginylation is required for degradation of the protein via the ubiquitin pathway. Does not arginylate cysteine residues.