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Urea cycle
Compartment-spanning conversion of nitrogen into urea through ornithine, citrulline and arginine intermediates. 17 reactions, from Reactome R-HSA-70635.
- arginine + H2O => ornithine + urea [ARG2]L-Arg + H2O → L-Orn + Urea
- glutamate + acetyl CoA => N-acetyl glutamate + CoAAc-CoA + L-Glu → CoA-SH + NAcGlu + H+
- 2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]HCO3- + NH4+ + 2 ATP → CAP + Pi + 2 ADP + 2 H+
- carbamoyl phosphate + ornithine => citrulline + orthophosphateCAP + L-Orn → L-Cit + Pi + H+
- arginine + H2O => ornithine + urea [ARG1]L-Arg + H2O → L-Orn + Urea
- argininosuccinate fumarate + arginineARSUA → L-Arg + FUMA
- ASS1 tetramer:NMRAL1 dimer:NADPH transforms L-Asp and L-Cit to ARSUAATP + L-Asp + L-Cit → ARSUA + AMP + PPi + H+
- ornithine (cytosolic) + citrulline (mitochondrial) => ornithine (mitochondrial) + citrulline (cytosolic)L-Cit + L-Orn + H+ → L-Cit + L-Orn + H+
- SIRT5 deglutarylates CPS1N6-glutaryl lysine CPS1 + 8 NAD+ + 8 H2O → CPS1 + 8 2''-O-glutaryl-ADP-D-ribose(3-) + 8 NAM
- SIRT5 desuccinylates CPS1NAD+ + H2O + N6-succinyl K1291 CPS1 → NAM + 2''-O-succinyl-ADP-D-ribose(3-) + CPS1
- OTC precursor is targeted to the mitochondrial matrix and processedOTC precursor → OTC trimer
- TP53 binds ARG1 geneTP53 Tetramer + ARG1 gene → TP53 tetramer:ARG1 gene
- TP53 binds CPS1 geneTP53 Tetramer + CPS1 gene → TP53 tetramer:CPS1 gene
- TP53 binds OTC geneTP53 Tetramer + OTC gene → TP53 tetramer:OTC gene
- ARG1 gene expressionARG1 gene → ARG1 trimer
- CPS1 gene expressionCPS1 gene → CPS1
- OTC gene expressionOTC gene → OTC precursor