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carbamoyl phosphate

362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API

Inferred

Every route here is a hypothesis drawn from reference reactions (Rhea and Human-GEM), not a finding. Currency species such as water, ATP and NAD(H) never form a step. Evidence key

344 compounds within 3 steps: 5 in 1, 41 in 2, 298 in 3

Does it reach a compound?

One step

N-carbamoyl-L-aspartic acidReach from here →
1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
Catalysed by CADcytosol
L-citrullineReach from here →
1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
Catalysed by OTCmitochondrial matrix
L-glutamineReach from here →
1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
Catalysed by CAD
N-carbamoyl-L-aspartateReach from here →
1. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)
Catalysed by CAD
D-glucopyranose 6-phosphateReach from here →
1. glucose-6-phosphatase (carbamoyl-phosphate)
Catalysed by G6PC2endoplasmic reticulum

Two steps

CHEBI:1146900Reach from here →
1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
2. Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
Catalysed by CADcytosol
2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
2. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)
Catalysed by CAD, PFAS
CHEBI:15570Reach from here →

Three steps

beta-carbolineReach from here →
Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosol
(S)-carnitineReach from here →

Page 1 of 7

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
transport of D-alanine and glutamine (cytosol to extracellular)

Catalysed by CAD, SLC3A2, SLC7A10extracellular space, cytosolOne of 2 routes

L-leucineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and leucine (cytosol to extracellular)

Catalysed by OTCmitochondrial matrix, extracellular space, cytosolOne of 6 routes

CHEBI:15699Reach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and homoserine (cytosol to extracellular)

Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 4 routes

alpha-D-glucosamine 6-phosphateReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

Catalysed by CAD, GFPT2, GFPT1cytosol

keto-D-fructose 6-phosphateReach from here →

glucose-6-phosphatase (carbamoyl-phosphate)
D-glucose-6-phosphate aldose-ketose-isomerase

Catalysed by G6PC2, GPIendoplasmic reticulum, cytosol

CHEBI:15971Reach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and histidine (cytosol to extracellular)

Catalysed by OTC, SLC7A5, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

L-valineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and valine (cytosol to extracellular)

Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

L-arginineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of arginine and citrulline (cytosol to extracellular)

Catalysed by OTC, SLC7A5, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 9 routes

CHEBI:16523Reach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
transport of D-serine and glutamine (cytosol to extracellular)

Catalysed by CAD, SLC3A2, SLC7A10extracellular space, cytosolOne of 2 routes

protein polypeptide chainReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
PROTEIN_BS

Catalysed by CADcytosol

2-oxoglutaramateReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
L-Glutamine:pyruvate aminotransferase

Catalysed by CAD, KYAT1cytosolOne of 3 routes

CHEBI:16857Reach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and threonine (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

L-isoleucineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and isoleucine (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

CHEBI:17203Reach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and proline (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

L-cysteineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and cysteine (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

CHEBI:17895Reach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and tyrosine (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

CHEBI:18019Reach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and lysine (cytosol to extracellular)

Catalysed by OTC, SLC7A5, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 6 routes

2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

Catalysed by CAD, PFAScytosol

Gln-tRNA(Gln)Reach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
L-Glutamine:tRNA(Gln) ligase (AMP-forming)

Catalysed by CAD, QARS1cytosol

isocyanateReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
L-citrulline + H(+) = isocyanate + L-ornithine

Catalysed by OTC, LACC1mitochondrial matrix

chlorine(.)Reach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
Transport of L-Glutamine by Atb0 Transporter

Catalysed by CAD, SLC6A14extracellular space, cytosol

L-aspartateReach from here →

carbamoyl-phosphate:L-aspartate carbamoyltransferase
carbamoyl-phosphate:L-aspartate carbamoyltransferase

Catalysed by CADcytosolOne of 4 routes

(S)-dihydroorotateReach from here →

carbamoyl-phosphate:L-aspartate carbamoyltransferase
(S)-dihydroorotate amidohydrolase

Catalysed by CADcytosolOne of 2 routes

D-serineReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
D-serine(in) + L-glutamine(out) + Na(+)(out) = D-serine(out) + L-glutamine(in) + Na(+)(in)

Catalysed by CAD, SLC1A5inner side of the membrane, outer side of the membraneOne of 4 routes

D-glucoseReach from here →

glucose-6-phosphatase (carbamoyl-phosphate)
D-Glucose-6-phosphate phosphohydrolase

Catalysed by G6PC2, G6PC1, G6PC3endoplasmic reticulum

L-ornithineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15

Catalysed by OTC, SLC7A9mitochondrial matrix, extracellular space, cytosolOne of 13 routes

glycineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and glycine (cytosol to extracellular)

Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 5 routes

2-(N(omega)-L-arginino)succinateReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
L-Citrulline:L-aspartate ligase (AMP-forming)

Catalysed by OTC, ASS1mitochondrial matrix, cytosolOne of 2 routes

L-methionineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and methionine (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 9 routes

L-tryptophanReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and tryptophan (cytosol to extracellular)

Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 5 routes

L-threonineReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)

Catalysed by CAD, SLC1A5inner side of the membrane, outer side of the membraneOne of 4 routes

6-phospho-D-glucono-1,5-lactoneReach from here →

glucose-6-phosphatase (carbamoyl-phosphate)
D-glucose-6-phosphate:NADP+ 1-oxidoreductase

Catalysed by G6PC2, G6PDendoplasmic reticulumOne of 3 routes

L-alanineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of alanine and citrulline (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 10 routes

L-asparagineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of asparagine and citrulline (cytosol to extracellular)

Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 11 routes

L-phenylalanineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and phenylalanine (cytosol to extracellular)

Catalysed by OTC, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 9 routes

1D-myo-inositol 3-phosphateReach from here →

glucose-6-phosphatase (carbamoyl-phosphate)
1D-myo-inositol-3-phosphate lyase (isomerizing)

Catalysed by G6PC2, ISYNA1endoplasmic reticulum, cytosol

5-phospho-beta-D-ribosylamineReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
phosphoribosyl pyrophosphate amidotransferase (glutamine)

Catalysed by CAD, PPATcytosolOne of 2 routes

D-glucosamine 6-phosphateReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

Catalysed by CAD, GFPT2, GFPT1

an N(2)-acyl-L-glutamineReach from here →

hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

Catalysed by CAD, GLYATL1B, GLYATL1

Catalysed by CAD, SLC6A14

extracellular space, cytosol

One of 245 routes

N(omega)-methyl-L-arginineReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of arginine and citrulline (cytosol to extracellular)
N(omega)-methyl-L-arginine(in) + L-arginine(out) = N(omega)-methyl-L-arginine(out) + L-arginine(in)

Catalysed by OTC, SLC7A5, SLC7A6, SLC25A29mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

3-methyl-2-oxobutanoateReach from here →

Carbamoyl-phosphate:L-ornithine carbamoyltransferase
transport of citrulline and valine (cytosol to extracellular)
L-Valine:2-oxoglutarate aminotransferase

Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes