carbamoyl phosphate

Three steps

• CHEBI:16174Reach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-Glucose-6-phosphate phosphohydrolase
  3. dATP:D-glucose 6-phosphotransferase

  Catalysed by G6PC2, G6PC1, G6PC3, HK1 and 3 moreendoplasmic reticulum, cytosol

• sulfateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)

  Catalysed by CAD, SLC25A14, SLC25A30cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• ureaReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of arginine and citrulline (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by OTC, SLC7A5, SLC7A6, ARG1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• D-glucono-1,5-lactoneReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-Glucose-6-phosphate phosphohydrolase
  3. D-glucose + NAD(+) = D-glucono-1,5-lactone + NADH + H(+)

  Catalysed by G6PC2, G6PC1, G6PC3, H6PDendoplasmic reticulumOne of 2 routes

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

  Catalysed by OTC, SLC7A5, SLC7A6, SLC7A9mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 9 routes

• 11-cis-retinolReach 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. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by CAD, DGAT1, LRAT, AWAT1 and 2 morecytosol

• (R)-3-aminoisobutyric acidReach 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, cytosol

• CHEBI:16332Reach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-glucose-6-phosphate aldose-ketose-isomerase
  3. D-Fructose 6-phosphate:D-glyceraldehyde-3-phosphate glycolaldehyde transferase

  Catalysed by G6PC2, GPI, TKT, TKTL1 and 1 moreendoplasmic reticulum, cytosol

• iodideReach 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 3 routes

• oxaloacetateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. L-Aspartate:2-oxoglutarate aminotransferase

  Catalysed by CAD, GOT2cytosol, mitochondrial matrixOne of 6 routes

• nitric oxideReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of arginine and citrulline (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by OTC, SLC7A5, SLC7A6, NOS3 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by OTC, SLC7A9, ACY1mitochondrial matrix, extracellular space, cytosol

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

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

• D-alanyl-D-alanineReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. transport of D-alanine and glutamine (cytosol to extracellular)
  3. Formation of Alanylalanine

  Catalysed by CAD, SLC3A2, SLC7A10extracellular space, cytosol

• CHEBI:16633Reach 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

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

  Catalysed by OTC, SLC7A6, MARS1mitochondrial matrix, extracellular space, cytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

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

• orotic acidReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. (S)-dihydroorotate amidohydrolase
  3. (S)-dihydroorotate:NAD+ oxidoreductase

  Catalysed by CAD, DHODHcytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by OTC, SLC7A6, CNDP2mitochondrial matrix, extracellular space, cytosol

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

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

• tryptamineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

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

• CHEBI:16856Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. cystine to cysteine conversion

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

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

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

• D-erythrose 4-phosphateReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-glucose-6-phosphate aldose-ketose-isomerase
  3. D-Fructose 6-phosphate:D-glyceraldehyde-3-phosphate glycolaldehyde transferase

  Catalysed by G6PC2, GPI, TKT, TKTL1 and 1 moreendoplasmic reticulum, cytosol

• N-acetyl-L-aspartateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by CAD, NAT8Lcytosol, mitochondrial matrixOne of 2 routes

• oxalic 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 oxalate (cytosol to extracellular)

  Catalysed by CAD, SLC6A14, SLC26A6extracellular space, cytosol

• 3-hydroxypyruvateReach 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-serine + O2 + H2O = 3-hydroxypyruvate + H2O2 + NH4(+)

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

• L-glutamic 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-oxo-acid aminotransferase

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

• myo-inositolReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. 1D-myo-inositol-3-phosphate lyase (isomerizing)
  3. 1D-myo-inositol 3-phosphate phosphohydrolase

  Catalysed by G6PC2, ISYNA1, IMPA2, IMPA1endoplasmic reticulum, cytosolOne of 2 routes

• anilineReach 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:arylamine N-acetyltransferase

  Catalysed by CAD, NAT2, NAT1cytosol

• CHEBI:17312Reach 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. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by CADcytosolOne of 2 routes

• CHEBI:17380Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

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

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by OTC, SLC7A6, PRODH2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

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

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

• AH2Reach 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, cytosolOne of 4 routes

• 5-aminolevulinic acidReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

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

• indole-3-pyruvateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

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

• (7Z,10Z,13Z)-hexadecatrienoyl-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 (C18:3->C16:3), Mitochondrial

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

• lactoseReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-Glucose-6-phosphate phosphohydrolase
  3. D-glucose + UDP-alpha-D-galactose = lactose + UDP + H(+)

  Catalysed by G6PC2, G6PC1, G6PC3, B4GALT2 and 1 moreendoplasmic reticulum

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

  Catalysed by OTC, SLC7A6, AARS1mitochondrial matrix, extracellular space, cytosol

• glycine betaineReach 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 betaine and chloride (cytosol to extracellular)

  Catalysed by CAD, SLC6A14, SLC6A12extracellular space, cytosolOne of 3 routes

• trans-urocanateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

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

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

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

• CHEBI:17808Reach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-Glucose-6-phosphate phosphohydrolase
  3. ITP:D-glucose 6-phosphotransferase

  Catalysed by G6PC2, G6PC1, G6PC3, HK1 and 3 moreendoplasmic reticulum, cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by OTC, BCAT2mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• D-glucitolReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-Glucose-6-phosphate phosphohydrolase
  3. Aldehyde Reductase

  Catalysed by G6PC2, G6PC1, G6PC3endoplasmic reticulum, cytosolOne of 3 routes

• CHEBI:17927Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of arginine and citrulline (cytosol to extracellular)
  3. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by OTC, SLC7A5, SLC7A6, SLC3A2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-phenylpyruvateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by OTC, SLC7A6, GOT2, TATmitochondrial matrix, extracellular space, cytosolOne of 6 routes

• fumaric acidReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. L-Citrulline:L-aspartate ligase (AMP-forming)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by OTC, ASS1, ASLmitochondrial matrix, cytosolOne of 2 routes

• CHEBI:18095Reach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of alanine and citrulline (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by OTC, SLC7A6, SLC1A4mitochondrial matrix, extracellular space, cytosolOne of 5 routes