carbamoyl phosphate

Three steps

• L-saccharopineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by OTC, SLC7A5, SLC7A6, AASSmitochondrial matrix, extracellular space, cytosol

• L-kynurenineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by OTC, SLC7A6, KYNUmitochondrial matrix, extracellular space, cytosolOne of 5 routes

• beta-alanineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by OTC, SLC7A6, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• leukotriene C4Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosol

• taurocyamineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of arginine and citrulline (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by OTC, SLC7A5, SLC7A6, GATMmitochondrial matrix, extracellular space, cytosol

• L-glutamate 5-semialdehydeReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by OTC, SLC7A9, OATmitochondrial matrix, extracellular space, cytosol

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by CAD, AGAcytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by OTC, SLC7A6, KYNUmitochondrial matrix, extracellular space, cytosol

• L,L-cystathionineReach 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
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by CAD, NAGScytosol, mitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by OTC, SLC7A6, GCLC, GCLMmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by OTC, SLC7A5, SLC7A6mitochondrial matrix, extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by OTC, SLC7A6, BHMT2mitochondrial matrix, extracellular space, cytosol

• S-methyl-L-methionineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by OTC, SLC7A6, BHMT2mitochondrial matrix, extracellular space, cytosol

• L-tyrosineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

  Catalysed by OTC, SLC7A6, PAHmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. N(omega),N(omega)-dimethyl-L-arginine(in) + L-ornithine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-ornithine(in)

  Catalysed by OTC, SLC7A9, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• an acyl-CoAReach 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
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by CAD, ASAH1, ACER2, ACER1 and 1 morecytosol, Golgi apparatusOne of 3 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by OTC, SLC7A6, SLC1A4mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• histamineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and histidine (cytosol to extracellular)
  3. L-histidine carboxy-lyase (histamine-forming)

  Catalysed by OTC, SLC7A5, SLC7A6, HDCmitochondrial matrix, extracellular space, cytosol

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

  Catalysed by CAD, PAICScytosolOne of 2 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of arginine and citrulline (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by OTC, SLC7A5, SLC7A6, IL4I1mitochondrial matrix, extracellular space, cytosol

• (3Z)-dodecenoyl-CoAReach 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
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by CAD, HADHA, ACAA2, HADHBcytosol, mitochondrial matrixOne of 3 routes

• S-sulfanyl-L-cysteineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by OTC, SLC7A6, CARS2mitochondrial matrix, extracellular space, cytosol

• N-formyl-L-kynurenineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 3 moremitochondrial matrix, extracellular space, cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach 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
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by CAD, SLC27A2, SLC27A5cytosol, endoplasmic reticulumOne of 3 routes

• L-methionine (S)-S-oxideReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by OTC, SLC7A6, MSRAmitochondrial matrix, extracellular space, cytosol

• L-methionine (R)-S-oxideReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by OTC, SLC7A6, MSRB3, MSRB1 and 1 moremitochondrial matrix, extracellular space, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by OTC, SLC7A9, AGXT2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• deoxycholoyl-CoAReach 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
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by CAD, SLC27A2, SLC27A5cytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by OTC, SLC7A6, PPCSmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 4-aminobutanoateReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. Transport of L-Glutamine by Atb0 Transporter
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by CAD, SLC6A14, SLC6A12extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by OTC, SLC7A6, TPOmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• L-prolineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by OTC, SLC7A6, SLC1A4mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-benzoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by OTC, SLC7A6, CDO1mitochondrial matrix, extracellular space, cytosolOne of 6 routes

• decanoyl-CoAReach 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
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by CAD, ACSM4, ACSM1, ACSM2A and 3 morecytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach 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
  3. Carnitine O-Palmitoyltransferase

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach 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
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 11 routes

• D-glucose 6-phosphateReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-glucose-6-phosphate:NADP+ 1-oxidoreductase
  3. NAADP(+) + D-glucose 6-phosphate = NAADPH + 6-phospho-D-glucono-1,5-lactone + H(+)

  Catalysed by G6PC2, G6PDendoplasmic reticulumOne of 4 routes

• L-cysteinylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by OTC, SLC7A6, GGTLC3, GGT1 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach 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
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by CAD, CPT1A, CPT1Bcytosol

• CHEBI:63544Reach 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
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• hexacosanoyl-CoAReach 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
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 5 routes

• tetracosanoyl-CoAReach 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
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 10 routes

• docosanoyl-CoAReach 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
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 9 routes

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of arginine and citrulline (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by OTC, SLC7A5, SLC7A6, NOS3 and 2 moremitochondrial matrix, extracellular space, cytosol

• Mo-molybdopterinReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by OTC, SLC7A6, MOCOSmitochondrial matrix, extracellular space, cytosol

• (2E)-octadecenoyl-CoAReach 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
  3. Carnitine O-Palmitoyltransferase

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach 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
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 8 routes

• L-threonylcarbamoyladenylateReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

  Catalysed by CAD, SLC1A5, YRDCinner side of the membrane, outer side of the membrane