fumaric acid

Three steps

• guanidinoacetateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-Arginine:glycine amidinotransferase

  Catalysed by ASL, GATMcytosolOne of 3 routes

• 3',5'-cyclic GMPReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3',5'-cyclic GMP(out) + a dicarboxylate(in) = 3',5'-cyclic GMP(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 12 routes

• adenosine 5'-phosphoramidateReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. ATP:sulfate adenylyltransferase
  3. adenosine 5'-phosphosulfate + NH4(+) = adenosine 5'-phosphoramidate + sulfate + 2 H(+)

  Catalysed by SLC25A10, PAPSS1, PAPSS2, FHITmitochondrial matrix, cytosol

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

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 7 routes

• beta-alanineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  3. L-aspartate 1-carboxy-lyase (beta-alanine-forming)

  Catalysed by ASL, ASS1, GAD2cytosolOne of 4 routes

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

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 26 routes

• leukotriene C4Reach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. leukotriene C4 = leukotriene A4 + glutathione

  Catalysed by SLC25A10, TSTD1, MGST3, GSTM4 and 2 moremitochondrial matrix, cytosol

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

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 7 routes

• taurocyamineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by ASL, GATMcytosol

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by ASL, ASS1, AGAcytosol

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

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 10 routes

• 3',5'-cyclic AMPReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3',5'-cyclic AMP(out) + a dicarboxylate(in) = 3',5'-cyclic AMP(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• carbamoyl phosphateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  3. Carbamoyl-phosphate:L-ornithine carbamoyltransferase

  Catalysed by ASL, ASS1, OTCcytosol, mitochondrial matrixOne of 2 routes

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol
  3. dihydroxyacetone phosphate + hexadecanoyl-CoA = 1-hexadecanoylglycerone 3-phosphate + CoA

  Catalysed by SDHB, GPD2, GNPAT

• L-tyrosineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  3. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)

  Catalysed by ASL, ASS1, SLC7A13cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)

  Catalysed by ASL, SLC25A2cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H(+)

  Catalysed by FH, SDHB, SDHA, PRODH2cytosolOne of 2 routes

• 5-oxo-L-prolineReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. glutathione = L-cysteinylglycine + 5-oxo-L-proline

  Catalysed by SLC25A10, TSTD1, CHAC2, CHAC1mitochondrial matrix, cytosol

• kynurenateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. L-kynurenine + glyoxylate = kynurenate + glycine + H2O

  Catalysed by FH, CLYBL, KYAT3, AADATcytosolOne of 3 routes

• 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  3. (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

  Catalysed by ADSL, ATICcytosolOne of 2 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by ASL, IL4I1cytosol

• (3S)-citramalyl-CoAReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by FH, ME2, ME3, CLYBLcytosol, mitochondrial matrix

• phosphoenolpyruvateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. Citrate Transport, Mitochondrial
  3. phosphoenolpyruvate(in) + citrate(out) = phosphoenolpyruvate(out) + citrate(in)

  Catalysed by FH, SLC25A1cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 3 routes

• D-glucosamineReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by SLC25A10, SGSHmitochondrial matrix, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by FH, ME2, ME3, AGXT2cytosol, mitochondrial matrixOne of 2 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-glyceraldehyde 3-phosphateReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol
  3. D-glyceraldehyde 3-phosphate = dihydroxyacetone phosphate

  Catalysed by SDHB, GPD2, TPI1

• 4-aminobutanoateReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by SLC25A10, SLC26A6, SLC6A12mitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• L-prolineReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. L-proline + a quinone = (S)-1-pyrroline-5-carboxylate + a quinol + H(+)

  Catalysed by FH, SDHB, SDHA, PRODH and 1 morecytosolOne of 4 routes

• prostaglandin E2Reach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. prostaglandin E2(out) + a dicarboxylate(in) = prostaglandin E2(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-benzoylglycineReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. N-benzoylglycine(out) + a dicarboxylate(in) = N-benzoylglycine(in) + a dicarboxylate(out)

  Catalysed by SLC25A10, SLC25A35, SLC25A34, SLC22A6 and 1 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  3. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by ASL, ASS1, SLC25A12, SLC25A13cytosol, mitochondrial matrixOne of 4 routes

• dIMPReach from here →
  1. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  2. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)
  3. dIMP + inosine = 2'-deoxyinosine + IMP

  Catalysed by ADSL, TNRC6B, ADSS2, ADSS1 and 1 morecytosol

• L-cysteinylglycineReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. glutathione + H2O = L-cysteinylglycine + L-glutamate

  Catalysed by SLC25A10, TSTD1, GGT3P, GGT1 and 3 moremitochondrial matrix, cytosolOne of 2 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by FH, ME2, ME3, HOGA1cytosol, mitochondrial matrixOne of 2 routes

• 3-demethylubiquinol-10Reach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate
  3. 3-demethylubiquinol-10 + S-adenosyl-L-methionine = ubiquinol-10 + S-adenosyl-L-homocysteine + H(+)

  Catalysed by SLC25A10, SQOR, COQ3mitochondrial matrix, cytosol

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by ASL, NOS3, NOS1, NOS2cytosol

• xanthurenateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O

  Catalysed by FH, CLYBL, KYAT3, AADATcytosol

• 3',3'-cGAMPReach from here →
  1. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  2. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
  3. 3',3'-cGAMP(out) + 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(in) = 3',3'-cGAMP(in) + 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(out)

  Catalysed by ADSL, SLC19A1cytosol, inner side of the membrane, outer side of the membrane

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by FH, ME2, ME3, HOGA1cytosol, mitochondrial matrixOne of 2 routes

• beta-citrylglutamateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. Citrate Transport, Mitochondrial
  3. citrate + L-glutamate + ATP = beta-citrylglutamate + ADP + phosphate + H(+)

  Catalysed by FH, SLC25A1, RIMKLBcytosol, mitochondrial matrix

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (9S)-hydroperoxy-(10E,12Z)-octadecadienoate + 2 glutathione = (9S)-hydroxy-(10E,12Z)-octadecadienoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX1, GPX4mitochondrial matrix, cytosol

• glycodeoxycholateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. glycodeoxycholate(in) + glutathione(in) + ATP + H2O = glycodeoxycholate(out) + glutathione(out) + ADP + phosphate + H(+)

  Catalysed by SLC25A10, TSTD1, ABCC4mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX1, GPX2 and 1 moremitochondrial matrix, cytosol

• 16alpha-hydroxydehydroepiandrosterone 3-sulfateReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)
  3. dehydroepiandrosterone 3-sulfate + reduced [NADPH--hemoprotein reductase] + O2 = 16alpha-hydroxydehydroepiandrosterone 3-sulfate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by SLC25A10, STS, CYP3A7mitochondrial matrix, cytosol

• 3-methylmalateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)

  Catalysed by FH, CLYBLcytosol

• (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, PTGES, MGST3 and 4 moremitochondrial matrix, cytosol

• (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX1, GPX4mitochondrial matrix, cytosol

• an S-substituted glutathioneReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by SLC25A10, TSTD1, GSTT4, GSTA4 and 21 moremitochondrial matrix, cytosolOne of 2 routes