Automated description from the Alliance of Genome Resources (Release 7.0.0)
Enables carbamoyl-phosphate synthase (ammonia) activity. Predicted to be involved in several processes, including alpha-amino acid metabolic process; carbamoyl phosphate biosynthetic process; and triglyceride catabolic process. Located in mitochondrion. Is expressed in alimentary system; intestine; liver; and midgut. 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. Orthologous to human CPS1 (carbamoyl-phosphate synthase 1).