carbamoyl phosphate

Three steps

• AReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (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

• glycoursodeoxycholateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. glycine to glycoursodeoxycholate conversion

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

• N-(9Z-octadecenoyl)glycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 2 moremitochondrial matrix, extracellular space, cytosol

• N-(9Z-octadecenoyl)-L-phenylalanineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O

  Catalysed by OTC, SLC7A6, PM20D1mitochondrial matrix, extracellular space, cytosol

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O

  Catalysed by OTC, SLC7A6, PM20D1mitochondrial matrix, extracellular space, cytosol

• N-(9Z-octadecenoyl)-L-leucineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and leucine (cytosol to extracellular)
  3. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate

  Catalysed by OTC, PM20D1mitochondrial matrix, extracellular space, cytosol

• N-octadecanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. octadecanoyl-CoA + glycine = N-octadecanoylglycine + CoA + H(+)

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

• N-(9Z-hexadecenoyl)-glycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. (9Z)-hexadecenoyl-CoA + glycine = N-(9Z-hexadecenoyl)-glycine + CoA + H(+)

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

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazoleReach 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(+)
  3. 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + ATP = 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole + ADP + phosphate + H(+)

  Catalysed by CAD, PFAS, GART

• N-hexadecanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

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

• N-dodecanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. dodecanoyl-CoA + glycine = N-dodecanoylglycine + CoA + H(+)

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

• N-decanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. decanoyl-CoA + glycine = N-decanoylglycine + CoA + H(+)

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

• N-octanoylglycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. octanoyl-CoA + glycine = N-octanoylglycine + CoA + H(+)

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

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

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

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

  Catalysed by OTC, SLC7A9, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-homocarnosineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and histidine (cytosol to extracellular)
  3. Formation of homocarnosine

  Catalysed by OTC, SLC7A5, SLC7A6, CARNS1mitochondrial matrix, extracellular space, cytosol

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

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

• L-cystine-bis-glycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 2 moremitochondrial matrix, extracellular space, cytosol

• 2-amino-2-deoxy-6-phospho-D-glucono-1,5-lactoneReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate
  3. D-glucosamine 6-phosphate + NADP(+) = 2-amino-2-deoxy-6-phospho-D-glucono-1,5-lactone + NADPH + 2 H(+)

  Catalysed by CAD, GFPT2, GFPT1, H6PD

• glycyldehydrophenylalanineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

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

• kinetensinReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. Chymase

  Catalysed by OTC, SLC7A6, CMA1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• an N-(fatty acyl)-glycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

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

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

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

• (7Z)-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 ((7Z)-octadecenoyl-CoA)

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

• acetaldehydeReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

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

• CHEBI:15345Reach 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. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by CAD, AACScytosolOne of 2 routes

• cholineReach 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 choline (cytosol to extracellular)

  Catalysed by CAD, SLC6A14, SLC5A7extracellular space, cytosolOne of 2 routes

• pyruvateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by OTC, SLC7A6, AGXT2mitochondrial matrix, extracellular space, cytosolOne of 18 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)

  Catalysed by OTC, SLC7A6, PAHmitochondrial matrix, extracellular space, cytosolOne of 3 routes

• CHEBI:15380Reach 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. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by CAD, ALAS1, CPNE7cytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach 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. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by CAD, GLYAT, GLYATL2, GLYATL1cytosol, mitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-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. Production of Tiglylcarnitine

  Catalysed by CAD, CPT1A, CPT1BcytosolOne of 2 routes

• isobutyryl-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-oxo-4-methyl-pentanoyl-CoA)

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

• 3-methylbut-2-enoyl-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. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by CAD, GLYATcytosol, mitochondrial matrix

• isovaleryl-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. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by CAD, GLYAT, GLYATL2, GLYATL1cytosol, mitochondrial matrixOne of 3 routes

• arachidonoyl-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. Arachidonate:CoA ligase (AMP-forming)

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

• CHEBI:15519Reach 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 C-acyltransferase

  Catalysed by CAD, ACAA1, SCP2, MYO5Bcytosol, peroxisomeOne of 2 routes

• palmitoyl-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. Palmitate:CoA ligase (AMP-forming)

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

• oleoyl-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. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 11 routes

• phenylacetyl-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. Phenylacetate:CoA ligase

  Catalysed by CADcytosolOne of 3 routes

• propionyl-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. Propanoate:CoA ligase (AMP-forming)

  Catalysed by CAD, ACSS3, ACSS2, ACSS1cytosolOne of 43 routes

• CHEBI:15611Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

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

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach 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 2 routes

• CHEBI:15650Reach 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-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by CAD, ZDHHC11B, NAA10, ESCO2 and 29 morecytosolOne of 2 routes

• trimethylenediamineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

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

• CHEBI:15727Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and histidine (cytosol to extracellular)
  3. L-histidine:beta-alanine ligase (ADP-forming)

  Catalysed by OTC, SLC7A5, SLC7A6, CARNS1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:15746Reach 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. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by CAD, SAT1, SAT2cytosolOne of 2 routes

• CHEBI:15765Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

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

• beta-D-glucoseReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-Glucose-6-phosphate phosphohydrolase
  3. D-glucose 1-epimerase

  Catalysed by G6PC2, G6PC1, G6PC3, GALMendoplasmic reticulum, cytosol

• thioredoxin dithiolReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. L-Glutamine:tRNA(Gln) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by CAD, QARS1cytosolOne of 2 routes