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

Three steps

• leukotriene C4Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by ASL, SLC7A6cytosol, extracellular space

• dimethylamineReach 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. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by ASL, SLC25A2, DDAH1cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• IMPReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  3. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)

  Catalysed by ASL, ADSL, TNRC6B, ADSS2 and 1 morecytosol

• L-glutamate 5-semialdehydeReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by ASL, ARG1, OATcytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by ASL, SLC7A6, KYNUcytosol, extracellular space

• L,L-cystathionineReach 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-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by ASS1, NAT8L, NAGSmitochondrial matrix

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

  Catalysed by ASL, SLC7A6, GCLC, GCLMcytosol, extracellular spaceOne of 2 routes

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

  Catalysed by ASL, SLC3A2, SLC7A5, SLC7A6cytosol, extracellular space, peroxisome

• L-homocysteineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by ASL, SLC7A6, BHMT2cytosol, extracellular space

• adenosine 5'-phosphosulfateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. ATP:sulfate adenylyltransferase

  Catalysed by ASL, SLC25A10, PAPSS1, PAPSS2cytosol, mitochondrial matrixOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by ASL, SLC7A6, BHMT2cytosol, extracellular space

• N-acetylputrescineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. acetyl-CoA:putrescine N-acetyltransferase

  Catalysed by ASL, SLC3A2, SLC7A7, SAT1 and 1 morecytosol, extracellular spaceOne of 2 routes

• 4-aminobutanalReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. putrescine + O2 + H2O = 4-aminobutanal + H2O2 + NH4(+)

  Catalysed by ASL, SLC3A2, SLC7A7, AOC1cytosol, extracellular space

• glutathione disulfideReach 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. cystine to cysteine conversion

  Catalysed by ASL, SLC7A9cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(1)-acetylspermidineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2

  Catalysed by ASL, SLC3A2, SLC7A7, PAOXcytosol, extracellular space

• an acyl-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. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by ASS1, NAT8L, ASAH1, ACER2 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  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 ASL, SLC7A6, SLC1A4cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

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

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

• L-2-aminopentanoateReach 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. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

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

• L-2-aminohexanoateReach 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. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

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

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

  Catalysed by ASL, ADSLcytosolOne of 2 routes

• (3Z)-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-5-enoyl-CoA)

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

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

  Catalysed by ASL, SLC7A6, CARS2cytosol, extracellular space

• N-formyl-L-kynurenineReach 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 = N-formyl-L-kynurenine

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

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-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. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by ASS1, NAT8L, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glutamine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

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

• phosphoenolpyruvateReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by ASS1, GOT2, PCK1, PCK2mitochondrial matrix

• 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. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

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

• L-methionine (S)-S-oxideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by ASL, SLC7A6, MSRAcytosol, extracellular space

• L-methionine (R)-S-oxideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by ASL, SLC7A6, MSRB3, MSRB1 and 1 morecytosol, extracellular space

• 5-amino-2-oxopentanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-Arginine amidinohydrolase
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by ASL, ARG1, AGXT2cytosolOne of 2 routes

• deoxycholoyl-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. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by ASS1, NAT8L, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. thiosulfate + glutathione = S-sulfanylglutathione + sulfite + H(+)

  Catalysed by ASL, SLC25A10, TSTD1cytosol, mitochondrial matrixOne of 3 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by ASL, SLC7A6, GLYATL2cytosol, extracellular spaceOne of 2 routes

• methylamineReach 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 + H2O = L-citrulline + methylamine

  Catalysed by ASL, SLC25A29, DDAH1cytosol, inner side of the membrane, outer side of the membrane

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

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

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  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 ASS1, SLC7A13, PAHinner side of the membrane, outer side of the membrane

• 4-aminobutanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by ASL, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 5 routes

• 3-iodo-L-tyrosineReach 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 2 routes

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

  Catalysed by ASL, SLC7A6, SLC1A4cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• estrone 3-sulfateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by ASL, SLC25A10, STScytosol, mitochondrial matrix

• N-benzoylglycineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by ASL, SLC7A6cytosol, extracellular space, mitochondrial matrixOne of 2 routes

• decanoyl-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. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by ASS1, NAT8L, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-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. Carnitine O-Palmitoyltransferase

  Catalysed by ASS1, NAT8L, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-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 (palmitolate)

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

• L-cysteinylglycineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of alanine and arginine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by ASL, SLC7A6, GGTLC3, GGT1 and 4 morecytosol, extracellular spaceOne of 2 routes

• 3-hydroxyisovaleryl-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. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by ASS1, NAT8L, CPT1A, CPT1Bmitochondrial matrix, cytosol

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. a quinone + succinate = fumarate + a quinol
  3. trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H(+)

  Catalysed by ASL, SDHB, PRODH2cytosol

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by ASL, SLC25A10, SLC4A1cytosol, mitochondrial matrix, extracellular spaceOne of 2 routes

• CHEBI:63544Reach 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 (adrenic acid)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes