(2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinate

Three steps

• a quinolReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by ADSL, FH, SDHB, SDHAcytosolOne of 13 routes

• folic acidReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. 10-formyldihydrofolate + 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide = 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide + 7,8-dihydrofolate
  3. dihydrofolate:NAD+ oxidoreductase

  Catalysed by ADSL, ATIC, DHFR, DHFR2cytosolOne of 4 routes

• a dicarboxylateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)

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

• chlorine(.)Reach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. transport of chloride and sulfate (cytosol to extracellular)

  Catalysed by ADSL, SLC25A10, SLC26A6cytosol, mitochondrial matrix, extracellular space

• hydrogen sulfideReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. 3alpha-hydroxysteroid 3-dehydrogenase (Si-specific) (H2S2O3)

  Catalysed by ADSL, SLC25A10cytosol, mitochondrial matrixOne of 2 routes

• D-aspartateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. D-aspartate(in) + sulfate(out) = D-aspartate(out) + sulfate(in)

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

• L-aspartateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)

  Catalysed by ADSL, ASL, ASS1cytosolOne of 4 routes

• oxalateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

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

• citric acidReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Citrate Transport, Mitochondrial

  Catalysed by ADSL, FH, SLC25A1cytosol, mitochondrial matrixOne of 5 routes

• maleateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

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

• malonic acidReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfite (cytosol to mitochondria)
  3. transport of malonate and sulfite (cytosol to mitochondria)

  Catalysed by ADSL, SLC25A10cytosol, mitochondrial matrixOne of 4 routes

• orotateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. a quinone + succinate = fumarate + a quinol
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

  Catalysed by ADSL, SDHB, DHODHcytosol

• isocitric acidReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

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

• (2R,3R)-tartrateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

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

• (S,S)-tartrateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

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

• (3R)-citramalateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

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

• (3S)-citramalateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

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

• cis-aconitic acidReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

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

• glyoxylateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)

  Catalysed by ADSL, FH, CLYBLcytosol

• glycineReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by ADSL, ATIC, SHMT1cytosolOne of 4 routes

• (S)-malyl-CoAReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by ADSL, FH, SUCLG1, SUCLA2cytosol

• 5-formimidoyltetrahydrofolateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by ADSL, ATIC, FTCDcytosolOne of 2 routes

• dihydroxyacetone phosphateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. a quinone + succinate = fumarate + a quinol
  3. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol

  Catalysed by ADSL, SDHB, GPD2cytosol

• L-methionineReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(in) + (6S)-5-methyl-5,6,7,8-tetrahydrofolate(out) = 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(out) + (6S)-5-methyl-5,6,7,8-tetrahydrofolate(in)
  3. (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine

  Catalysed by ADSL, SLC19A1, MTRcytosol, inner side of the membrane, outer side of the membrane

• N-sulfo-D-glucosamineReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by ADSL, SLC25A10, SGSHcytosol, mitochondrial matrix

• dehydroepiandrosterone 3-sulfateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by ADSL, SLC25A10, STScytosol, mitochondrial matrix

• glutathioneReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfite (cytosol to mitochondria)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by ADSL, SLC25A10, TSTD1cytosol, mitochondrial matrixOne of 2 routes

• IMPReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. (6R)-10-formyltetrahydrofolate + 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide = 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide + (6S)-5,6,7,8-tetrahydrofolate
  3. IMP + H2O = 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide

  Catalysed by ADSL, ATICcytosolOne of 2 routes

• adenosine 5'-phosphosulfateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. ATP:sulfate adenylyltransferase

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

• glutathione disulfideReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by ADSL, SLC25A10, TSTD1cytosol, mitochondrial matrix

• S-sulfanylglutathioneReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. thiosulfate + glutathione = S-sulfanylglutathione + sulfite + H(+)

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

• estrone 3-sulfateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by ADSL, SLC25A10, STScytosol, mitochondrial matrix

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  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 ADSL, SDHB, PRODH2cytosol

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Transport of 5-Hydroxyindoleacetate

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

• ubiquinol-10Reach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. transport of fumarate and sulfite (cytosol to mitochondria)
  3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate

  Catalysed by ADSL, SLC25A10, SQORcytosol, mitochondrial matrix