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

Three steps

• 5-aminolevulinateReach 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 + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by ASL, SLC7A6, ALAS1, ALAS2cytosol, extracellular space

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

  Catalysed by ASL, SLC7A6, BCAT1cytosol, extracellular spaceOne of 2 routes

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

  Catalysed by ASL, SLC7A6, GOT2, GOT1 and 1 morecytosol, extracellular spaceOne of 6 routes

• glycochenodeoxycholateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by ASL, SLC7A6, BAATcytosol, extracellular space

• glycochenodeoxycholic acidReach 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

• glyoxylateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by ASL, SLC7A6, AADATcytosol, extracellular spaceOne of 12 routes

• glycolithocholic acidReach 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 (glycine)

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

• L-2-amino-3-oxobutanoic acidReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

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

• D-glucopyranose 6-phosphateReach from here →
  1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  3. glucose-6-phosphatase (carbamoyl-phosphate)

  Catalysed by ASS1, OTC, G6PC2mitochondrial matrix, endoplasmic reticulum

• phosphoenolpyruvic 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. GTP:oxaloacetate carboxy-lyase (adding GTP

  Catalysed by ASS1, GOT2, PCK1mitochondrial matrix, cytosolOne of 2 routes

• gamma-Glu-AlaReach 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, GGT1, GGT5 and 4 morecytosol, extracellular space

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by ASL, SLC7A6, GLDC, GCSHcytosol, extracellular space, mitochondrial matrix

• taurineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by ASL, SLC6A14, SLC6A6cytosol, extracellular spaceOne of 5 routes

• (S)-malyl-CoAReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by ASL, FH, SUCLG1, SUCLA2cytosol

• (R)-methylmalonyl-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. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by ASS1, NAT8L, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-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. (S)-methylmalonyl-CoA hydrolase

  Catalysed by ASS1, NAT8Lmitochondrial matrix

• 3'-dephospho-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. acid phosphatase 2, lysosomal (CoA)

  Catalysed by ASS1, NAT8L, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-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. (2E)-dodecenoyl-CoA formation

  Catalysed by ASS1, NAT8Lmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-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. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ASS1, NAT8L, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-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 (gamma-linolenate)

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

• butanoyl-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. Butanoate:CoA ligase (AMP-forming)

  Catalysed by ASS1, NAT8L, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-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 (lauric acid)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• glutaryl-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 Glutaryl Carnitine

  Catalysed by ASS1, NAT8L, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 2 routes

• eicosanoyl-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 (eicosanoate)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-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. Itaconate:CoA ligase (GDP-forming)

  Catalysed by ASS1, NAT8L, SUCLG1, SUCLG2 and 1 moremitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-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 ((6Z,9Z)-octadecadienoic acid)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-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 (myristic acid)

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

• octanoyl-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. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ASS1, NAT8L, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-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. phytanate-CoA ligase

  Catalysed by ASS1, NAT8Lmitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-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 (stearate)

  Catalysed by ASS1, NAT8L, SLC27A2, ACSL4 and 5 moremitochondrial matrix, cytosolOne of 15 routes

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

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

• sarcosineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

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

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by ASL, SLC7A6, SHMT1, SHMT2cytosol, extracellular space

• carnosineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-histidine(out) + L-arginine(in) + Na(+)(out) = L-histidine(in) + L-arginine(out) + Na(+)(in)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by ASL, SLC7A6, CARNS1cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by ASL, SLC7A6, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 3 routes

• dihydroxyacetone phosphateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. a quinone + succinate = fumarate + a quinol
  3. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol

  Catalysed by ASL, SDHB, GPD2cytosol

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

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

• an N-acylglycineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by ASL, SLC7A6, GLYATL3, GLYAT and 1 morecytosol, extracellular space

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by ASL, SLC7A6, GOT2, GOT1cytosol, extracellular spaceOne of 3 routes

• sphing-4-enineReach 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

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

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

• spermidineReach 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 7 routes

• hypotaurineReach 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 + H(+) = hypotaurine + CO2

  Catalysed by ASS1, SLC25A12, SLC25A13, GADL1 and 1 moremitochondrial matrix, cytosol

• N-sulfo-D-glucosamineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by ASL, SLC25A10, SGSHcytosol, mitochondrial matrix

• dehydroepiandrosterone 3-sulfateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by ASL, SLC25A10, STScytosol, mitochondrial matrix

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

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

• glutathioneReach 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 + ATP = glutathione + ADP + phosphate + H(+)

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

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

  Catalysed by ASL, SLC7A6, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. transport of arginine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by ASL, SLC3A2, SLC7A5, SLC7A6 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 2 routes

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

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