Anti-carbamoyl-phosphate synthase 1, mitochondrial antibodies enable researchers to detect and measure the carbamoyl-phosphate synthase 1, mitochondrial antigen in biological samples. This target is a reported synonym of the CPS1 gene, which encodes carbamoyl-phosphate synthase 1. This protein is known to function in hepatocyte differentiation, among other biological roles. The human version of carbamoyl-phosphate synthase 1, mitochondrial has a canonical amino acid length of 1500 residues and a protein mass of 164.9 kilodaltons, although 3 isoforms have been identified. It is reported to be localized in the nucleus, mitochondria, and cell membrane of cells and notably expressed in the small intestine, liver, and duodenum. Other names for this target antigen include CPSASE1, GATD6, and PHN.