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

Three steps

• oxalic acidReach 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 oxalate (cytosol to extracellular)

  Catalysed by ASL, SLC6A14, SLC26A6cytosol, extracellular spaceOne of 2 routes

• 4-maleylacetoacetateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
  3. 4-maleylacetoacetate = 4-fumarylacetoacetate

  Catalysed by ASL, FAH, GSTZ1cytosol

• L-glutamic 5-semialdehydeReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by ASL, ARG1, OATcytosol, mitochondrial matrix

• anilineReach 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:arylamine N-acetyltransferase

  Catalysed by ASS1, NAT8L, NAT2, NAT1mitochondrial matrix, cytosol

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

  Catalysed by ASS1, NAT8Lmitochondrial matrix, cytosolOne of 2 routes

• D-aspartic acidReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. transport of aspartate (cytosol to extracellular)
  3. transport of D-aspartate (cytosol to extracellular)

  Catalysed by ASS1, SLC1A7, SLC1A3, SLC1A2 and 2 moreextracellular space, cytosol

• CHEBI:17380Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by ASL, SLC7A6, SLC7A5cytosol, extracellular spaceOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by ASL, SLC7A6, PRODH2cytosol, extracellular spaceOne of 3 routes

• enol-oxaloacetateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by ASL, FH, SDHB, SDHAcytosolOne of 3 routes

• AH2Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by ASL, SLC7A6, MOCOScytosol, extracellular spaceOne of 4 routes

• S-methyl-5'-thioadenosineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by ASL, SLC3A2, SLC7A7, SRMcytosol, extracellular spaceOne of 2 routes

• 5-aminolevulinic acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by ASL, SLC7A6, ALAS1, ALAS2 and 1 morecytosol, extracellular space, mitochondrial matrix

• nitrateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

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

• indole-3-pyruvateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by ASL, SLC7A6, IL4I1cytosol, extracellular spaceOne of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Steryl-Sulfatase

  Catalysed by ASL, SLC25A10, STScytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-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. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by ASS1, NAT8L, HADHA, ACADVL and 1 moremitochondrial matrix

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

  Catalysed by ASL, SLC7A6, AARS1cytosol, extracellular space

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

  Catalysed by ASL, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 3 routes

• CHEBI:17769Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. Putrescine:oxygen oxidoreductase (deaminating)

  Catalysed by ASL, SLC3A2, SLC7A7, AOC2 and 2 morecytosol, extracellular spaceOne of 2 routes

• trans-urocanateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by ASL, SLC7A5, SLC7A6, HALcytosol, extracellular spaceOne of 2 routes

• urateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. Peroxidase

  Catalysed by ASL, ARG1, MPOcytosol, nucleusOne of 3 routes

• 5-hydroxy-L-tryptophanReach 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 space

• 4-methyl-2-oxopentanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by ASL, SLC3A2, SLC7A6, SLC7A7 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 4 routes

• 3-oxopropanoic acidReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. L-aspartate 1-carboxy-lyase (beta-alanine-forming)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by ASS1, GAD2, ABATcytosol, mitochondrial matrix

• ubiquinolReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. succinate:quinone oxidoreductase
  3. thioredoxin-disulfide reductase (NADPH) (ubiquinone)

  Catalysed by ASL, SDHD, SDHB, SDHA and 3 morecytosol, mitochondrial matrixOne of 6 routes

• 3-phenylpyruvateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by ASL, SLC7A6, GOT2, TATcytosol, extracellular space, mitochondrial matrixOne of 5 routes

• thiocyanateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by ASL, SLC25A10, MPST, TSTcytosol, mitochondrial matrixOne of 2 routes

• CHEBI:18095Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by ASL, SLC7A6, SLC1A4cytosol, extracellular spaceOne of 5 routes

• N-carbamoyl-beta-alanineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by ASL, SLC6A14cytosol, extracellular spaceOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by ASL, SLC7A6, GARTcytosol, extracellular space

• 2-phenylethylamineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by ASL, SLC7A6, DDCcytosol, extracellular space

• hydrogen cyanideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfite (cytosol to mitochondria)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by ASL, SLC25A10, MPST, TSTcytosol, mitochondrial matrixOne of 2 routes

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by ASL, SLC7A6, PFAScytosol, extracellular space

• ophthalmateReach 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-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by ASL, SLC7A6, GSScytosol, extracellular space

• S-methylmercury-L-cysteineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and methionine (cytosol to extracellular)
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by ASL, SLC7A6, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 6 routes

• 2-aminoisobutanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by ASL, SLC7A6, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• biocytinReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-lysine(out) + L-arginine(in) = L-lysine(in) + L-arginine(out)
  3. biocytin + H2O = biotin + L-lysine

  Catalysed by ASL, SLC7A9, SLC25A29, SLC7A6 and 1 morecytosol, inner side of the membrane, outer side of the membrane

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  3. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by ASL, SLC25A2, AGXT2cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by ASL, SLC7A6, AGXT2cytosol, extracellular spaceOne of 2 routes

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. N(omega)-methyl-L-arginine(in) + L-arginine(out) = N(omega)-methyl-L-arginine(out) + L-arginine(in)
  3. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by ASL, SLC25A29, AGXT2cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by ASL, SLC7A6, SHMT1cytosol, extracellular spaceOne of 2 routes

• S-disulfanyl-L-cysteineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by ASL, SLC7A6, CARS2cytosol, extracellular space

• (5Z)-dodecenoyl-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-oxomyrist-7-enoyl-CoA)

  Catalysed by ASS1, NAT8L, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach 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. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (7Z)-tetradecenoyl-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. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-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. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

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

• (7Z,10Z)-hexadecadienoyl-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. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by ASS1, NAT8L, HADHA, ACADVL and 1 moremitochondrial matrix

• (4Z,7Z)-decadienoyl-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. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by ASS1, NAT8L, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-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. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by ASS1, NAT8L, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• CHEBI:240943Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by ASL, SLC7A6, GLYAT, GLYATL2 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 3 routes