Cps1 - carbamoyl-phosphate synthase 1

Enables several functions, including anion binding activity; calcium ion binding activity; and carbamoyl-phosphate synthase (ammonia) activity. Involved in several processes, including cellular response to cAMP; cellular response to oleic acid; and response to peptide hormone. Located in mitochondrial inner membrane. Part of protein-containing complex. Biomarker of fatty liver disease; hypertension; and obesity. 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). [provided by Alliance of Genome Resources, Apr 2022]