carbamoyl phosphate

Three steps

• N-carbamoyl-beta-alanineReach 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 3-Ureidopropionate

  Catalysed by CAD, SLC6A14extracellular space, cytosolOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

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

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

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

• ophthalmateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

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

  Catalysed by OTC, SLC7A6, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

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

  Catalysed by OTC, SLC7A6, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-isoleucineReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. D-serine(in) + L-glutamine(out) + Na(+)(out) = D-serine(out) + L-glutamine(in) + Na(+)(in)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by CAD, SLC1A5, SLC7A10inner side of the membrane, outer side of the membrane

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by OTC, SLC7A6, AGXT2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

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

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

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

• (5Z)-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-7-enoyl-CoA)

  Catalysed by CAD, HADHA, ACAA2, HADHBcytosol, mitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach 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 (cis-cetoleic acid)

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

• (7Z)-tetradecenoyl-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 ((7Z)-tetradecenoic acid)

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

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-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. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

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

• (7Z,10Z)-hexadecadienoyl-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. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by CAD, HADHA, ACADVL, HADHBcytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-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. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by CAD, ECHS1, ACAA2, HADHcytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-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. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by CAD, ECHS1, ACAA2, HADHcytosol, mitochondrial matrixOne of 2 routes

• CHEBI:240943Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

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

• a quinolReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. (S)-dihydroorotate amidohydrolase
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

  Catalysed by CAD, DHODHcytosol

• hypochlorous acidReach 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. Peroxidase

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

• potassium atomReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. transport of aspartate (cytosol to extracellular)

  Catalysed by CAD, SLC1A7, SLC1A3, SLC1A2 and 2 morecytosol, extracellular spaceOne of 3 routes

• phenylacetylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

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

• CHEBI:27540Reach 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 (hexanoic acid)

  Catalysed by CAD, ACSM4, ACSM1, ACSM2A and 3 morecytosolOne of 6 routes

• elaidic acidReach 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 (elaidate)

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

• 5-hydroxytryptophanReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)

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

• CHEBI:28666Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. LTD4 hydrolysis

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

• CHEBI:28701Reach 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. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by CAD, ACAA1, SCP2, MYO5Bcytosol, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

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

• N-acetyl-L-cysteineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

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

• CHEBI:29052Reach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-glucose-6-phosphate aldose-ketose-isomerase
  3. sedoheptulose-7-phosphate:D-glyceraldehyde-3-phosphate glyceronetransferase

  Catalysed by G6PC2, GPI, TALDO1endoplasmic reticulum, cytosol

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

  Catalysed by OTC, SLC7A6, SLC1A1mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• Cys-tRNA(Cys)Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by OTC, SLC7A6, CARS1mitochondrial matrix, extracellular space, cytosol

• Phe-tRNA(Phe)Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. L-Phenylalanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by OTC, SLC7A6, FARSB, FARSAmitochondrial matrix, extracellular space, cytosol

• Gly-tRNA(Gly)Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• Asp-tRNA(Asp)Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by CAD, DARS1cytosol

• Trp-tRNA(Trp)Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. L-Tryptophan -tRNA(Trp) ligase (AMP-forming)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• Ile-tRNA(Ile)Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and isoleucine (cytosol to extracellular)
  3. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)

  Catalysed by OTC, SLC7A6, IARS1mitochondrial matrix, extracellular space, cytosol

• Asn-tRNA(Asn)Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of asparagine and citrulline (cytosol to extracellular)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• glycocholateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

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

• hydrogen sulfideReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

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

• CHEBI:30249Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

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

• formic acidReach 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 chloride and formate (cytosol to extracellular)

  Catalysed by CAD, SLC6A14, SLC26A4extracellular space, cytosolOne of 2 routes

• malonic acidReach 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. Malonate Transport, Antiport with Chloride

  Catalysed by CAD, SLC6A14extracellular space, cytosolOne of 2 routes

• orotateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. (S)-dihydroorotate amidohydrolase
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

  Catalysed by CAD, DHODHcytosol

• L-lysineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. L-ornithine(out) + L-lysine(in) = L-ornithine(in) + L-lysine(out)

  Catalysed by OTC, SLC7A9, SLC25A2, SLC25A15mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• D-lysineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by OTC, SLC7A9, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• putrescineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. L-ornithine carboxy-lyase (putrescine-forming)

  Catalysed by OTC, SLC7A9, ODC1mitochondrial matrix, extracellular space, cytosolOne of 4 routes

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

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

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

• di-mu-sulfido-diiron(2+)Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by OTC, SLC7A6, NDUFAB1, FDXR and 5 moremitochondrial matrix, extracellular space, cytosol