N-carbamoyl-L-aspartic acid

Three steps

• sphing-4-enineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by CAD, NAT8L, ASAH1, ACER2 and 2 morecytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• D-threonineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by CAD, AGXT2, SLC7A10cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• spermidineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by CAD, NAT8L, SAT1, SAT2cytosol, mitochondrial matrixOne of 3 routes

• hypotaurineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by CAD, SLC25A12, SLC25A13, GADL1 and 1 morecytosol, mitochondrial matrix

• N-sulfo-D-glucosamineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by CAD, SLC25A14, SLC25A30, SGSHcytosol, inner side of the membrane, outer side of the membrane

• dehydroepiandrosterone 3-sulfateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by CAD, SLC25A14, SLC25A30, STScytosol, inner side of the membrane, outer side of the membrane

• L-dopaquinoneReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  3. L-tyrosine + O2 = L-dopaquinone + H2O

  Catalysed by CAD, SLC7A13, TYRcytosol, inner side of the membrane, outer side of the membrane

• L-threonineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)

  Catalysed by CAD, SLC7A13, SLC1A5cytosol, inner side of the membrane, outer side of the membraneOne of 8 routes

• 6-phospho-D-glucono-1,5-lactoneReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. glucose-6-phosphatase (carbamoyl-phosphate)
  3. D-glucose-6-phosphate:NADP+ 1-oxidoreductase

  Catalysed by CAD, G6PC2, G6PDcytosol, endoplasmic reticulumOne of 3 routes

• L-kynurenineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  3. Transport of L-Kynurenine

  Catalysed by CAD, SLC7A13, SLC43A1cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 5 routes

• dimethylamineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by CAD, AGXT2, DDAH1cytosolOne of 2 routes

• IMPReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. IMP:L-aspartate ligase (GDP-forming)
  3. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)

  Catalysed by CAD, ADSS2, ADSS1cytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by CAD, AGXT2, KYNUcytosol

• L,L-cystathionineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by CAD, NAT8L, NAGScytosol, mitochondrial matrix

• L-homocysteineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by CAD, SLC7A13, BHMT2cytosol, inner side of the membrane, outer side of the membrane

• adenosine 5'-phosphosulfateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. ATP:sulfate adenylyltransferase

  Catalysed by CAD, SLC25A14, SLC25A30, PAPSS1 and 1 morecytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by CAD, SLC7A13, BHMT2cytosol, inner side of the membrane, outer side of the membrane

• glutathione disulfideReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  3. cystine to cysteine conversion

  Catalysed by CAD, SLC7A13cytosol, inner side of the membrane, outer side of the membrane

• an acyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by CAD, NAT8L, ASAH1, ACER2 and 2 morecytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  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 CAD, AGXT2, SLC1A4cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• 1D-myo-inositol 3-phosphateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. glucose-6-phosphatase (carbamoyl-phosphate)
  3. 1D-myo-inositol-3-phosphate lyase (isomerizing)

  Catalysed by CAD, G6PC2, ISYNA1cytosol, endoplasmic reticulum

• L-2-aminopentanoateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by CAD, AGXT2cytosol

• L-2-aminohexanoateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by CAD, AGXT2cytosol

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)
  3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

  Catalysed by CAD, PAICS, ADSLcytosolOne of 2 routes

• (3Z)-dodecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by CAD, NAT8L, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• N-formyl-L-kynurenineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-tryptophan(out) + L-aspartate(in) = L-tryptophan(in) + L-aspartate(out)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by CAD, SLC7A13, IDO1, TDO2 and 1 morecytosol, inner side of the membrane, outer side of the membrane

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by CAD, NAT8L, SLC27A2, SLC27A5cytosol, mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by CAD, SLC7A13, PPATcytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• phosphoenolpyruvateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by CAD, GOT2, PCK1, PCK2cytosol, mitochondrial matrix

• D-glucosamine 6-phosphateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by CAD, SLC7A13, GFPT2, GFPT1cytosol, inner side of the membrane, outer side of the membrane

• L-methionine (S)-S-oxideReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by CAD, SLC7A13, MSRAcytosol, inner side of the membrane, outer side of the membrane

• L-methionine (R)-S-oxideReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by CAD, SLC7A13, MSRB3, MSRB1 and 1 morecytosol, inner side of the membrane, outer side of the membrane

• deoxycholoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by CAD, NAT8L, SLC27A2, SLC27A5cytosol, mitochondrial matrixOne of 4 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  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 CAD, SLC7A13, PAHcytosol, inner side of the membrane, outer side of the membrane

• L-prolineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by CAD, AGXT2, SLC1A4cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• estrone 3-sulfateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by CAD, SLC25A14, SLC25A30, STScytosol, inner side of the membrane, outer side of the membrane

• decanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by CAD, NAT8L, ACSM4, ACSM1 and 4 morecytosol, mitochondrial matrixOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Carnitine O-Palmitoyltransferase

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 11 routes

• L-cysteinylglycineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. gamma-glutamyltransferase (alanine)

  Catalysed by CAD, AGXT2, GGTLC3, GGT1 and 4 morecytosolOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by CAD, NAT8L, CPT1A, CPT1Bcytosol, mitochondrial matrix

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by CAD, SLC25A14, SLC25A30, SLC4A1cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 2 routes

• CHEBI:63544Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 10 routes

• docosanoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 9 routes

• Mo-molybdopterinReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by CAD, AGXT2, MOCOScytosol

• (2E)-octadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Carnitine O-Palmitoyltransferase

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 8 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by CAD, NAT8L, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 9 routes