Automated description from the Alliance of Genome Resources (Release 3.0)
Exhibits carbamoyl-phosphate synthase (ammonia) activity. Predicted to contribute to carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity. Predicted to be involved in several processes, including alpha-amino acid metabolic process; carbamoyl phosphate biosynthetic process; and cellular response to ammonia. Localizes to mitochondrion. Used to study carbamoyl phosphate synthetase I deficiency disease. Human ortholog(s) of this gene implicated in carbamoyl phosphate synthetase I deficiency disease; persistent fetal circulation syndrome; and vascular disease. Is expressed in alimentary system; intestine; liver; and midgut. Orthologous to human CPS1 (carbamoyl-phosphate synthase 1).