2-(N(omega)-L-arginino)succinate

Three steps

• CHEBI:15345Reach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by ASS1, NAT8L, AACSmitochondrial matrix, cytosolOne of 2 routes

• cholineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. transport of chloride and choline (cytosol to extracellular)

  Catalysed by ASL, SLC6A14, SLC5A7cytosol, extracellular spaceOne of 2 routes

• pyruvateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by ASL, SLC7A6, AGXT2cytosol, extracellular space, mitochondrial matrixOne of 29 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by ASL, SLC7A6, TPH1, TPH2cytosol, extracellular spaceOne of 3 routes

• CHEBI:15380Reach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by ASS1, NAT8L, ALAS1, CPNE7mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by ASS1, NAT8L, GLYAT, GLYATL2 and 1 moremitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Production of Tiglylcarnitine

  Catalysed by ASS1, NAT8L, CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by ASS1, NAT8L, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by ASS1, NAT8L, GLYATmitochondrial matrix

• isovaleryl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by ASS1, NAT8L, GLYAT, GLYATL2 and 1 moremitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. propanoyl-CoA C-acyltransferase

  Catalysed by ASS1, NAT8L, ACAA1, SCP2 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Palmitate:CoA ligase (AMP-forming)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by ASS1, NAT8L, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Phenylacetate:CoA ligase

  Catalysed by ASS1, NAT8Lmitochondrial matrix, cytosolOne of 3 routes

• propionyl-CoAReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Propanoate:CoA ligase (AMP-forming)

  Catalysed by ASS1, NAT8L, ACSS3, ACSS2 and 1 moremitochondrial matrix, cytosolOne of 43 routes

• CHEBI:15570Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6, SLC3A2, SLC7A10cytosol, extracellular spaceOne of 5 routes

• CHEBI:15611Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by ASL, SLC7A6, GNMTcytosol, extracellular space

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine 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 ASL, SLC7A6, TPH1, TPH2cytosol, extracellular spaceOne of 3 routes

• CHEBI:15650Reach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by ASS1, NAT8L, ZDHHC11B, NAA10 and 30 moremitochondrial matrix, cytosolOne of 2 routes

• trimethylenediamineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by ASL, SLC3A2, SLC7A5, SLC7A6 and 1 morecytosol, extracellular space

• CHEBI:15727Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and histidine (cytosol to extracellular)
  3. L-histidine:beta-alanine ligase (ADP-forming)

  Catalysed by ASL, SLC7A5, SLC7A6, CARNS1cytosol, extracellular spaceOne of 3 routes

• CHEBI:15746Reach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by ASS1, NAT8L, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15765Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by ASL, SLC7A6, THcytosol, extracellular spaceOne of 5 routes

• malonateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. malonate(out) + (S)-malate(in) = malonate(in) + (S)-malate(out)

  Catalysed by ASL, FH, SLC25A10cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by ASL, SLC7A6, GFPT2, GFPT1cytosol, extracellular space

• beta-nicotinamide D-ribosideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. a quinone + succinate = fumarate + a quinol
  3. 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamide + a quinone + H(+) = beta-nicotinamide D-riboside + a quinol

  Catalysed by ASL, SDHB, NQO2cytosol

• N-acetyl-beta-D-glucosaminylamineReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  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 ASS1, AGA

• thioredoxin dithiolReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. L-Aspartate:tRNA(Asp) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by ASS1, DARS1cytosolOne of 2 routes

• nitriteReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. 4 nitric oxide + O2 + 2 H2O = 4 nitrite + 4 H(+)

  Catalysed by ASL, NOS3, NOS1, NOS2 and 1 morecytosol

• 11-cis-retinolReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by ASS1, NAT8L, DGAT1, LRAT and 3 moremitochondrial matrix, cytosol

• (R)-3-aminoisobutyric acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by ASL, SLC7A6, AGXT2cytosol, extracellular space, mitochondrial matrix

• iodideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by ASL, SLC7A6, TPOcytosol, extracellular spaceOne of 3 routes

• CHEBI:16523Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6, SLC3A2, SLC7A10cytosol, extracellular spaceOne of 4 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by ASL, ARG1, ACY1cytosol

• 2-phenylacetamideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by ASL, SLC7A6, EPXcytosol, extracellular space

• CHEBI:16633Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by ASL, SLC7A6, SCLYcytosol, extracellular space

• Met-tRNA(Met)Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by ASL, SLC7A6, MARS1cytosol, extracellular space

• CHEBI:16668Reach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by ASS1, SLC25A12, SLC25A13, GAD1 and 1 moremitochondrial matrix, cytosol

• quinolinic acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Transport of Quinolinic Acid

  Catalysed by ASL, SLC25A10, SLC22A6cytosol, mitochondrial matrix, extracellular spaceOne of 2 routes

• cyanamideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. urea = cyanamide + H2O

  Catalysed by ASL, ARG1, CA1, CA2cytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by ASL, SLC7A6, AADATcytosol, extracellular space

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by ASL, SLC7A6, CNDP2cytosol, extracellular space

• 2-oxobutanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and threonine (cytosol to extracellular)
  3. L-threonine ammonia-lyase (2-oxobutanoate-forming)

  Catalysed by ASL, SLC7A6, SDS, SDSLcytosol, extracellular space

• tryptamineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by ASL, SLC7A6, DDCcytosol, extracellular space

• 2-oxoglutaramateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by ASL, SLC7A6, KYAT1cytosol, extracellular spaceOne of 3 routes

• CHEBI:16856Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by ASL, SLC7A6, GSScytosol, extracellular spaceOne of 2 routes

• glyoxylic acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by ASL, SLC7A6, DAOcytosol, extracellular space, peroxisome

• creatineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by ASL, GATM, GAMTcytosolOne of 2 routes

• ferrocytochrome cReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfite (cytosol to mitochondria)
  3. sulfite:ferricytochrome-c oxidoreductase

  Catalysed by ASL, SLC25A10, SUOXcytosol, mitochondrial matrix