N-carbamoyl-L-aspartic acid

Three steps

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. 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(+)

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

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

  Catalysed by CAD, SLC7A13, CMA1cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 3 routes

• (7Z)-octadecenoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

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

• CHEBI:15345Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by CAD, NAT8L, AACScytosol, mitochondrial matrixOne of 2 routes

• cholineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by CAD, NAT8L, CHATcytosol, mitochondrial matrix, nucleus

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

  Catalysed by CAD, GOT2, FAHD1cytosol, mitochondrial matrixOne of 19 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)

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

• CHEBI:15380Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by CAD, NAT8L, ALAS1, CPNE7cytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by CAD, NAT8L, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Tiglylcarnitine

  Catalysed by CAD, NAT8L, CPT1A, CPT1Bcytosol, mitochondrial matrixOne of 2 routes

• isobutyryl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

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

• 3-methylbut-2-enoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by CAD, NAT8L, GLYATcytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by CAD, NAT8L, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Arachidonate:CoA ligase (AMP-forming)

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

• CHEBI:15519Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. propanoyl-CoA C-acyltransferase

  Catalysed by CAD, NAT8L, ACAA1, SCP2 and 1 morecytosol, mitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Palmitate:CoA ligase (AMP-forming)

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

• oleoyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by CAD, NAT8L, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Phenylacetate:CoA ligase

  Catalysed by CAD, NAT8Lcytosol, mitochondrial matrixOne of 3 routes

• propionyl-CoAReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Propanoate:CoA ligase (AMP-forming)

  Catalysed by CAD, NAT8L, ACSS3, ACSS2 and 1 morecytosol, mitochondrial matrixOne of 43 routes

• CHEBI:15570Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. transport of D-alanine and glutamine (cytosol to extracellular)

  Catalysed by CAD, SLC7A13, SLC3A2, SLC7A10cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 3 routes

• (S)-malateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. transport of malate and OAA (cytosol to mitochondria)

  Catalysed by CAD, GOT2cytosol, mitochondrial matrixOne of 9 routes

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  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 membraneOne of 3 routes

• CHEBI:15650Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by CAD, NAT8L, ZDHHC11B, NAA10 and 30 morecytosol, mitochondrial matrixOne of 2 routes

• CHEBI:15699Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate:L-glutamine amido-ligase (AMP-forming)
  3. transport of asparagine and homoserine (cytosol to extracellular)

  Catalysed by CAD, ASNS, SLC7A5cytosol, extracellular spaceOne of 30 routes

• CHEBI:15727Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate 1-carboxy-lyase (beta-alanine-forming)
  3. L-histidine:beta-alanine ligase (ADP-forming)

  Catalysed by CAD, GAD2, CARNS1cytosolOne of 2 routes

• CHEBI:15746Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by CAD, NAT8L, SAT1, SAT2cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:15765Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  3. solute carrier (L-dopa)

  Catalysed by CAD, SLC7A13, SLC3A2, SLC7A5cytosol, inner side of the membrane, outer side of the membrane, extracellular space

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

  Catalysed by CAD, SLC25A14, SLC25A30, SLC25A10cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• CHEBI:15842Reach from here →
  1. (S)-dihydroorotate amidohydrolase
  2. (S)-dihydroorotate:NAD+ oxidoreductase
  3. Orotidine-5'-phosphate:diphosphate phospho-alpha-D-ribosyl-transferase

  Catalysed by CAD, DHODH, UMPScytosol

• alpha-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. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

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

• keto-D-fructose 6-phosphateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. glucose-6-phosphatase (carbamoyl-phosphate)
  3. D-glucose-6-phosphate aldose-ketose-isomerase

  Catalysed by CAD, G6PC2, GPIcytosol, endoplasmic reticulum

• N-acetyl-beta-D-glucosaminylamineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by CAD, AGAcytosol

• thioredoxin dithiolReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-Aspartate:tRNA(Asp) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by CAD, DARS1cytosolOne of 2 routes

• CHEBI:15971Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate:L-glutamine amido-ligase (AMP-forming)
  3. transport of asparagine and histidine (cytosol to extracellular)

  Catalysed by CAD, ASNS, SLC7A6cytosol, extracellular spaceOne of 22 routes

• 11-cis-retinolReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by CAD, NAT8L, DGAT1, LRAT and 3 morecytosol, mitochondrial matrix

• (R)-3-aminoisobutyric acidReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by CAD, AGXT2cytosol, mitochondrial matrix

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

  Catalysed by CAD, ASS1, ASLcytosolOne of 40 routes

• CHEBI:16523Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. transport of D-serine and glutamine (cytosol to extracellular)

  Catalysed by CAD, SLC7A13, SLC3A2, SLC7A10cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 2 routes

• 2-phenylacetamideReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

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

• CHEBI:16633Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by CAD, AGXT2, SCLYcytosol

• Met-tRNA(Met)Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by CAD, SLC7A13, MARS1cytosol, inner side of the membrane, outer side of the membrane

• CHEBI:16668Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by CAD, SLC25A12, SLC25A13, GAD1 and 1 morecytosol, mitochondrial matrix

• quinolinic acidReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. Transport of Quinolinic Acid

  Catalysed by CAD, SLC25A14, SLC25A30, SLC22A6cytosol, inner side of the membrane, outer side of the membrane, extracellular spaceOne of 2 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

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

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by CAD, SLC7A13, CNDP2cytosol, inner side of the membrane, outer side of the membrane

• tryptamineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-tryptophan(out) + L-aspartate(in) = L-tryptophan(in) + L-aspartate(out)
  3. L-tryptophan decarboxy-lyase

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

• 2-oxoglutaramateReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by CAD, SLC7A13, KYAT1cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• CHEBI:16857Reach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate:L-glutamine amido-ligase (AMP-forming)
  3. transport of asparagine and threonine (cytosol to extracellular)

  Catalysed by CAD, ASNS, SLC7A5cytosol, extracellular spaceOne of 36 routes

• oxalic acidReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. transport of oxalate and sulfate (cytosol to extracellular)

  Catalysed by CAD, SLC25A14, SLC25A30, SLC26A6cytosol, inner side of the membrane, outer side of the membrane, extracellular space

• L-isoleucineReach from here →
  1. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  2. L-aspartate:L-glutamine amido-ligase (AMP-forming)
  3. transport of asparagine and isoleucine (cytosol to extracellular)

  Catalysed by CAD, ASNS, SLC7A5cytosol, extracellular spaceOne of 36 routes