carbamoyl phosphate

Three steps

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by OTC, SLC7A6, BCAT2, BCAT1mitochondrial matrix, extracellular space, cytosol

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

• 5-aminolevulinateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

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

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

  Catalysed by OTC, SLC7A6, BCAT1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• alpha-lactoseReach from here →
  1. glucose-6-phosphatase (carbamoyl-phosphate)
  2. D-Glucose-6-phosphate phosphohydrolase
  3. UDP-alpha-D-galactose:D-glucose 4-beta-D-galactosyltransferase

  Catalysed by G6PC2, G6PC1, G6PC3, B4GALT2 and 2 moreendoplasmic reticulum, Golgi apparatus

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by OTC, SLC7A6, GOT2, GOT1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

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

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

• glycochenodeoxycholic acidReach 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

• glyoxylateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by OTC, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 11 routes

• glycolithocholic acidReach 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 (glycine)

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

• L-2-amino-3-oxobutanoic acidReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

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

• gamma-Glu-AlaReach 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, GGT1, GGT5 and 4 moremitochondrial matrix, extracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

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

• taurineReach 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. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by CAD, SLC6A14, SLC6A6extracellular space, cytosolOne of 4 routes

• (R)-methylmalonyl-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. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by CAD, ALDH6A1cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-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. (S)-methylmalonyl-CoA hydrolase

  Catalysed by CADcytosol, mitochondrial matrix

• 3'-dephospho-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. acid phosphatase 2, lysosomal (CoA)

  Catalysed by CAD, ACP2cytosol, lysosomeOne of 2 routes

• (2E)-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. (2E)-dodecenoyl-CoA formation

  Catalysed by CADcytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-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. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

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

• (6Z,9Z,12Z)-octadecatrienoyl-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 (gamma-linolenate)

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

• butanoyl-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. Butanoate:CoA ligase (AMP-forming)

  Catalysed by CAD, ACSM4, ACSM1, ACSM2A and 3 morecytosolOne of 9 routes

• dodecanoyl-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 (lauric acid)

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

• glutaryl-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 Glutaryl Carnitine

  Catalysed by CAD, CPT1A, CPT1C, CPT1BcytosolOne of 2 routes

• eicosanoyl-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 (eicosanoate)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 12 routes

• itaconyl-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. Itaconate:CoA ligase (GDP-forming)

  Catalysed by CAD, SUCLG1, SUCLG2, SUCLA2cytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-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 ((6Z,9Z)-octadecadienoic acid)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 13 routes

• tetradecanoyl-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 (myristic acid)

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

• octanoyl-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. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by CAD, CROTcytosol, mitochondrial matrixOne of 20 routes

• phytanoyl-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. phytanate-CoA ligase

  Catalysed by CADcytosolOne of 5 routes

• octadecanoyl-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 (stearate)

  Catalysed by CAD, SLC27A2, ACSL4, ACSL3 and 4 morecytosolOne of 15 routes

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

• sarcosineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

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

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

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

• D-sedoheptulose 7-phosphateReach 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

• 4-guanidinobutanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of arginine and citrulline (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

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

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

  Catalysed by OTC, SLC7A6, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. acetyl-CoA:D-glucosamine-6-phosphate N-acetyltransferase

  Catalysed by CAD, GFPT2, GFPT1, GNPNAT1cytosolOne of 3 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by CAD, ADSS2, ADSS1cytosolOne of 2 routes

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

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

• L-histidineReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  3. L-histidine(in) + L-ornithine(out) + H(+)(in) = L-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 7 routes

• beta-D-fructose 6-phosphateReach from here →
  1. hydrogencarbonate + L-glutamine + 2 ATP + H2O = carbamoyl phosphate + L-glutamate + 2 ADP + phosphate + 2 H(+)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. alpha-D-glucosamine 6-phosphate + H2O = beta-D-fructose 6-phosphate + NH4(+)

  Catalysed by CAD, GFPT2, GFPT1, GNPDA1 and 1 morecytosol

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

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

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

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by OTC, SLC7A6, GOT2, GOT1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

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

  Catalysed by OTC, SLC7A5, SLC7A6, GATMmitochondrial matrix, extracellular space, cytosolOne of 5 routes

• sphing-4-enineReach 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 2 routes

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

• spermidineReach 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-acetylspermidine)

  Catalysed by CAD, SAT1, SAT2cytosolOne of 3 routes

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

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

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

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