fumaric acid

Three steps

• indole-3-pyruvateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. L-tryptophan + glyoxylate = indole-3-pyruvate + glycine

  Catalysed by FH, CLYBL, AADATcytosol

• 3,3'-diiodo-L-thyronineReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. Steryl-Sulfatase
  3. Steryl-Sulfatase

  Catalysed by SLC25A10, STSmitochondrial matrix, cytosol, Golgi apparatusOne of 3 routes

• glycine betaineReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by SLC25A10, SLC26A6, SLC6A12mitochondrial matrix, cytosol, extracellular spaceOne of 3 routes

• urateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by SLC25A10, TSTD1, SLC22A13mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• methylarsonous acidReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GSTO1, GSTO2mitochondrial matrix, cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine

  Catalysed by FH, CLYBL, AADATcytosol

• CHEBI:17895Reach 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 tyrosine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 6 routes

• CHEBI:17927Reach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by ASL, SLC3A2, SLC7A6cytosol, extracellular space

• butanoateReach 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(in) + butanoate(out) = dehydroepiandrosterone 3-sulfate(out) + butanoate(in)

  Catalysed by SLC25A10, STS, SLC22A9mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

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

  Catalysed by FH, CLYBL, AADATcytosol

• CHEBI:18019Reach 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 lysine (cytosol to extracellular)

  Catalysed by ASL, SLC3A2, SLC7A5, SLC7A6cytosol, extracellular spaceOne of 9 routes

• L-glutamineReach 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 glutamine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 10 routes

• dTDPReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. pyruvate kinase (dTDP)

  Catalysed by FH, ME2, ME3, PKM and 1 morecytosol, mitochondrial matrix

• CHEBI:18095Reach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of trans-4-hydroxy-L-proline (cytosol to extracellular)

  Catalysed by SLC25A10, SLC26A6, CLTRN, SLC6A20mitochondrial matrix, cytosol, extracellular space

• N-carbamoyl-beta-alanineReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by SLC25A10, SLC26A6mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• arsenic triglutathioneReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. arsenic triglutathione + 3 [thioredoxin]-dithiol + 3 S-adenosyl-L-methionine = trimethylarsine + 3 [thioredoxin]-disulfide + 3 glutathione + 3 S-adenosyl-L-homocysteine + 3 H(+)

  Catalysed by SLC25A10, TSTD1, AS3MTmitochondrial matrix, cytosolOne of 3 routes

• CHEBI:18381Reach 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. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by ADSL, ATICcytosol

• (6S)-5-methyl-5,6,7,8-tetrahydrofolateReach 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. 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)

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

• 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathioneReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. 15-deoxy-Delta(12,14)-prostaglandin J2 + glutathione = 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathione

  Catalysed by SLC25A10, TSTD1, MGST3mitochondrial matrix, cytosol

• 3-carboxy-4-methyl-5-propyl-2-furanpropanoateReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(out) + a dicarboxylate(in) = 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(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

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

• (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach 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,17Z)-eicosapentaenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

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

• (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoateReach 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,17Z)-eicosapentaenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

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

• (15S)-hydroxy-(11Z,13E)-eicosadienoateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (15S)-hydroperoxy-(11Z,13E)-eicosadienoate + 2 glutathione = (15S)-hydroxy-(11Z,13E)-eicosadienoate + glutathione disulfide + H2O

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

• (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (17S)-hydroperoxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + 2 glutathione = (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + glutathione disulfide + H2O

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

• a hydroxy-1,2-diacyl-glycero-3-phosphocholineReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. a hydroperoxy-1,2-diacyl-glycero-3-phosphocholine + 2 glutathione = a hydroxy-1,2-diacyl-glycero-3-phosphocholine + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, GPX4mitochondrial matrix, cytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. transport of malate and OAA (cytosol to mitochondria)
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by FH, AGXT2cytosol, mitochondrial matrixOne of 3 routes

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

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

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

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

• 5,6,7,8-tetrahydrofolic acidReach 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. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by ADSL, ATICcytosol

• 3-(glutathion-S-yl)-4-hydroxynonanalReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (E)-4-hydroxynon-2-enal + glutathione = 3-(glutathion-S-yl)-4-hydroxynonanal

  Catalysed by SLC25A10, TSTD1, GSTP1mitochondrial matrix, cytosol

• CHEBI:240943Reach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. 2-Methylbutyrylglycine Transport, Extracellular

  Catalysed by SLC25A10, SLC26A6, SLC6A5mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• hypochlorous acidReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Peroxidase

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

• potassium atomReach 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 aspartate (cytosol to extracellular)

  Catalysed by ASL, ASS1, SLC1A7, SLC1A3 and 3 morecytosol, extracellular spaceOne of 3 routes

• 3beta-hydroxyandrost-5-en-17-oneReach 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. 3beta-Hydroxyandrost-5-en-17-one 3-sulfate sulfohydrolase

  Catalysed by SLC25A10, STSmitochondrial matrix, cytosolOne of 3 routes

• a carboxylateReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. (S)-dihydroorotate + a quinone = orotate + a quinol
  3. orotate(out) + a carboxylate(in) = orotate(in) + a carboxylate(out)

  Catalysed by SDHB, DHODH, SLC22A13inner side of the membrane, outer side of the membraneOne of 2 routes

• L-ascorbic acidReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. L-dehydroascorbate + 2 glutathione = glutathione disulfide + L-ascorbate

  Catalysed by SLC25A10, TSTD1, CLIC1, GSTO1 and 1 moremitochondrial matrix, cytosol

• K(+)Reach 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. K(+)(in) + L-aspartate(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-aspartate(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by ASL, ASS1, SLC1A7, SLC1A3 and 3 morecytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• Asp-tRNA(Asp)Reach 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:tRNA(Asp) ligase (AMP-forming)

  Catalysed by ASL, ASS1, DARS1cytosol

• isocyanateReach 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-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by ASL, ASS1, LACC1cytosol

• a thiolReach from here →
  1. transport of fumarate and thiosulfate (cytosol to mitochondria)
  2. 3alpha-hydroxysteroid 3-dehydrogenase (Si-specific) (H2S2O3)
  3. L-cysteinyl-[sulfatase] + 2 a thiol + O2 = an organic disulfide + 3-oxo-L-alanyl-[sulfatase] + hydrogen sulfide + H2O + H(+)

  Catalysed by SLC25A10, SUMF1mitochondrial matrix, cytosol

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

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

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

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

• glycolateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. glycolate + NADP(+) = glyoxylate + NADPH + H(+)

  Catalysed by FH, CLYBL, GRHPRcytosol

• acetateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. transport of acetate and pyruvate (cytosol to extracellular)

  Catalysed by FH, ME2, ME3, SLC16A4 and 4 morecytosol, mitochondrial matrix, extracellular spaceOne of 4 routes

• formic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. transport of formate and pyruvate (cytosol to extracellular)

  Catalysed by FH, ME2, ME3, SLC16A4 and 4 morecytosol, mitochondrial matrix, extracellular spaceOne of 6 routes

• propionic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. transport of propanoate and pyruvate (cytosol to extracellular)

  Catalysed by FH, ME2, ME3, SLC16A4 and 5 morecytosol, mitochondrial matrix, extracellular spaceOne of 4 routes

• butyric acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. transport of butyrate and pyruvate (cytosol to extracellular)

  Catalysed by FH, ME2, ME3, SLC16A4 and 5 morecytosol, mitochondrial matrix, extracellular spaceOne of 4 routes

• 3-hydroxypyruvic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. L-Serine:pyruvate aminotransferase

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

• (indol-3-yl)acetateReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. (indol-3-yl)acetate(out) + a dicarboxylate(in) = (indol-3-yl)acetate(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