CPS1
UniProt:P31327 CPS1
NAGS
UniProt:Q8N159 NAGS
glutamate + acetyl CoA => N-acetyl glutamate + CoA
2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate
2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]
carbamoyl phosphate + ornithine => citrulline + orthophosphate
ATP + aspartate + citrulline <=> argininosuccinate + AMP + pyrophosphate
argininosuccinate <=> fumarate + arginine
arginine + H2O => ornithine + urea [ARG1]
arginine + H2O => ornithine + urea [ARG2]
ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic)
Urea cycle
Urea synthesis