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

Three steps

• a quinolReach 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 13 routes

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

  Catalysed by ASL, SLC6A14, LPOcytosol, extracellular spaceOne of 4 routes

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

  Catalysed by ASL, SLC25A10, SLC22A8, SLC22A7cytosol, mitochondrial matrix, extracellular spaceOne of 2 routes

• phenylacetylglycineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

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

• CHEBI:27540Reach 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. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by ASS1, NAT8L, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• elaidic acidReach 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 (elaidate)

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

• 5-hydroxytryptophanReach 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 2 routes

• CHEBI:28666Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. LTD4 hydrolysis

  Catalysed by ASL, SLC7A6cytosol, extracellular space

• CHEBI:28701Reach 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:acetyl-CoA C-acyltransferase

  Catalysed by ASS1, NAT8L, ACAA1, SCP2 and 1 moremitochondrial matrix, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach 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

• 3-disulfanyl-L-alanineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  3. L-cystine thiocysteine-lyase (deaminating

  Catalysed by ASL, SLC7A9, CTHcytosol, inner side of the membrane, outer side of the membrane

• N-acetyl-L-cysteineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

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

• a dicarboxylateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A35, SLC25A34cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• Ca(2+)Reach 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 Ca2+ and K+ (cytosol to extracellular)

  Catalysed by ASS1, SLC1A7, SLC1A3, SLC1A2 and 7 moreextracellular space, cytosolOne of 3 routes

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

  Catalysed by ASL, SLC7A6, CARS1cytosol, extracellular space

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

  Catalysed by ASL, SLC7A6, FARSB, FARSAcytosol, extracellular space

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

  Catalysed by ASL, SLC7A6, GARS1cytosol, extracellular space

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

  Catalysed by ASL, SLC7A6, WARS1cytosol, extracellular space

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

  Catalysed by ASL, SLC7A6, IARS1cytosol, extracellular space

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

  Catalysed by ASL, SLC7A6, QARS1cytosol, extracellular space

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

  Catalysed by ASL, SLC7A6, NARS1cytosol, extracellular space

• glycocholateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by ASL, SLC7A6, BAATcytosol, extracellular space, peroxisomeOne of 3 routes

• hydrogen sulfideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

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

• D-aspartateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. D-aspartate(in) + sulfate(out) = D-aspartate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 4 routes

• acetateReach 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-L-aspartate amidohydrolase

  Catalysed by ASS1, NAT8L, ASPA, ACY3mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:30249Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by ASL, SLC7A6, IDO1, TDO2 and 1 morecytosol, extracellular space

• 3-acetamidopropanalReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. Transport by Y+Lat-2 into Extracellular Space
  3. N1-acetylspermidine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by ASL, SLC3A2, SLC7A6, PAOX and 1 morecytosol, extracellular spaceOne of 2 routes

• oxalateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A14, SLC26A2 and 3 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• formic 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 formate (cytosol to extracellular)

  Catalysed by ASL, SLC6A14, SLC26A4cytosol, extracellular spaceOne of 2 routes

• citric acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Citrate Transport, Mitochondrial

  Catalysed by ASL, FH, SLC25A1cytosol, mitochondrial matrixOne of 7 routes

• maleateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• malonic acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by ASL, SLC6A14cytosol, extracellular spaceOne of 6 routes

• orotateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. a quinone + succinate = fumarate + a quinol
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

  Catalysed by ASL, SDHB, DHODHcytosol

• (S)-dihydroorotateReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. (S)-dihydroorotate amidohydrolase

  Catalysed by ASS1, CADcytosolOne of 2 routes

• isocitric acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by ASL, FH, SLC25A1cytosol, mitochondrial matrixOne of 3 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by ASS1, GOT2mitochondrial matrix

• (2R,3R)-tartrateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by ASL, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-lysineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by ASL, ARG1, SLC25A2cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-arginineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by ASL, ARG1, SLC25A2cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• cis-aconitic acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by ASL, FH, SLC25A1cytosol, mitochondrial matrixOne of 3 routes

• 3-oxopropanoateReach 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. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by ASS1, GAD2, AGXT2cytosol

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

  Catalysed by ASL, SLC7A6, IL4I1cytosol, extracellular space

• di-mu-sulfido-diiron(2+)Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by ASL, SLC7A6, NDUFAB1, FDXR and 5 morecytosol, extracellular space, mitochondrial matrix

• serotoninReach 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 serotonin (cytosol to extracellular)

  Catalysed by ASS1, SLC1A7, SLC1A3, SLC1A2 and 3 moreextracellular space, cytosolOne of 2 routes

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by ASL, SLC7A6, BCAT2, BCAT1cytosol, extracellular space

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

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

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

  Catalysed by ASL, SLC7A6, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 10 routes