fumaric acid

Three steps

• (S)-dihydroorotateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

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

• an alcoholReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, CP, PRDX6mitochondrial matrix, cytosol

• glutarateReach 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(out) + glutarate(in) = dehydroepiandrosterone 3-sulfate(in) + glutarate(out)

  Catalysed by SLC25A10, STS, SLC22A24, SLC22A8mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. transport of malate and OAA (cytosol to mitochondria)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by FH, GOT2cytosol, mitochondrial matrix

• pentanoateReach 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. pentanoate(out) + dehydroepiandrosterone 3-sulfate(in) = pentanoate(in) + dehydroepiandrosterone 3-sulfate(out)

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

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

  Catalysed by SDHB, DHODH, SLC22A12inner side of the membrane, outer side of the membraneOne of 2 routes

• L-lysineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-lysine(out) + L-arginine(in) = L-lysine(in) + L-arginine(out)

  Catalysed by ASL, SLC7A9, SLC25A29, SLC7A6cytosol, inner side of the membrane, outer side of the membrane

• putrescineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by ASL, SLC3A2, SLC7A7cytosol, extracellular space

• N-carbamoyl-L-aspartateReach 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-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

  Catalysed by ASL, ASS1, CADcytosol

• beta-D-fructose 1,6-bisphosphateReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol
  3. beta-D-fructose 1,6-bisphosphate = D-glyceraldehyde 3-phosphate + dihydroxyacetone phosphate

  Catalysed by SDHB, GPD2, ALDOA, ALDOB and 1 more

• 3-oxopropanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by FH, ME2, ME3, AGXT2cytosol, mitochondrial matrix

• methylarsonateReach from here →
  1. transport of fumarate and thiosulfate (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

• a phenolReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. an aryl sulfate + H2O = a phenol + sulfate + H(+)
  3. an aryl sulfate + H2O = a phenol + sulfate + H(+)

  Catalysed by SLC25A10, ARSL, ARSF, ARSK and 3 moremitochondrial matrix, cytosol

• D-serineReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. D-serine = pyruvate + NH4(+)

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

• D-ribose 1-phosphateReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamide + a quinone + H(+) = beta-nicotinamide D-riboside + a quinol
  3. purine nucleoside phosphorylase (nicotinamide)

  Catalysed by SDHB, NQO2, PNPcytosol

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by FH, CLYBL, AADATcytosol

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

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

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

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

• (R)-lactic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (R)-Lactate:NAD+ oxidoreductase

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

• (S)-lactic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by FH, ME2, ME3, LDHB and 1 morecytosol, mitochondrial matrixOne of 7 routes

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

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
  2. 4-fumarylacetoacetate fumarylhydrolase
  3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA

  Catalysed by FAH, HMGCL, HMGCLL1cytosol

• 2-iminopropanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

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

• phosphoenolpyruvic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. Citrate Transport, Mitochondrial
  3. transport of citrate and PEP (cytosol to mitochondria)

  Catalysed by FH, SLC25A1cytosol, mitochondrial matrixOne of 9 routes

• N-acetyl-beta-neuraminic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

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

• tert-butanolReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by SLC25A10, TSTD1, CLIC2, GPX1 and 3 moremitochondrial matrix, cytosol

• L-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 amidinohydrolase

  Catalysed by ASL, ARG1cytosolOne of 25 routes

• (5-hydroxyindol-3-yl)acetaldehydeReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. Transport of 5-Hydroxyindoleacetate
  3. (5-hydroxyindol-3-yl)acetaldehyde + NAD(+) + H2O = (5-hydroxyindol-3-yl)acetate + NADH + 2 H(+)

  Catalysed by SLC25A10, SLC4A1, ALDH9A1mitochondrial matrix, cytosol, extracellular space

• taurineReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by SLC25A10, SLC26A6, SLC6A6mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamideReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate + a quinone = enol-oxaloacetate + a quinol
  3. 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamide + a quinone + H(+) = beta-nicotinamide D-riboside + a quinol

  Catalysed by FH, SDHB, SDHA, NQO2cytosolOne of 2 routes

• a 3-acylpyruvateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by FH, ME2, ME3, FAHD1cytosol, mitochondrial matrix

• acetoacetyl-CoAReach from here →
  1. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
  2. 4-fumarylacetoacetate fumarylhydrolase
  3. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate

  Catalysed by FAH, AACScytosolOne of 2 routes

• glycineReach 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 glycine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 21 routes

• prostaglandin F2alphaReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. prostaglandin F2alpha(out) + a dicarboxylate(in) = prostaglandin F2alpha(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

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach 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-(5Z,8Z,11Z,13E)-eicosatetraenoate + 2 glutathione = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + glutathione disulfide + H2O

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

• 7,8-dihydrofolateReach 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. 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

  Catalysed by ADSL, ATICcytosolOne of 4 routes

• (6S)-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. (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, ATICcytosol

• XMPReach 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. IMP + NAD(+) + H2O = XMP + NADH + H(+)

  Catalysed by ADSL, TNRC6B, ADSS2, ADSS1 and 2 morecytosol

• (13S)-hydroperoxy-(9Z,11E)-octadecadienoateReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. sulfite:ferricytochrome-c oxidoreductase
  3. 13(S)-HPODE to 13-oxy-radical-octadecadienoate conversion

  Catalysed by SLC25A10, SUOXmitochondrial matrix, cytosol

• (R)-S-lactoylglutathioneReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (R)-S-lactoylglutathione = methylglyoxal + glutathione

  Catalysed by SLC25A10, TSTD1, GLO1mitochondrial matrix, cytosol

• 4-guanidinobutanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by ASL, GATMcytosol

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

  Catalysed by FH, CLYBL, AADATcytosol

• a 1-acylglycerone 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 + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by SDHB, GPD2, GNPAT

• orotidine 5'-phosphateReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. (S)-dihydroorotate + a quinone = orotate + a quinol
  3. orotidine 5'-phosphate + diphosphate = orotate + 5-phospho-alpha-D-ribose 1-diphosphate

  Catalysed by SDHB, DHODH, UMPS

• 3-guanidinopropanoateReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by ASL, GATMcytosol

• L-histidineReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-histidine(out) + L-arginine(in) + Na(+)(out) = L-histidine(in) + L-arginine(out) + Na(+)(in)

  Catalysed by ASL, SLC7A6cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• dGMPReach 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. dGMP + inosine = 2'-deoxyguanosine + IMP

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

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

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

• S-formylglutathioneReach from here →
  1. transport of fumarate and sulfite (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. S-formylglutathione + H2O = formate + glutathione + H(+)

  Catalysed by SLC25A10, TSTD1, ESDmitochondrial matrix, cytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by FH, ME2, ME3, AGXT2cytosol, mitochondrial matrix