fumaric acid

Three steps

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

  Catalysed by SLC25A10, SLC26A6, SLC5A7mitochondrial matrix, cytosol, extracellular space

• acetylpyruvateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. acetylpyruvate + H2O = acetate + pyruvate + H(+)

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

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

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

• D-threo-isocitrateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. Citrate Transport, Mitochondrial
  3. citrate = D-threo-isocitrate

  Catalysed by FH, SLC25A1, ACO1, ACO2cytosol, mitochondrial matrixOne of 3 routes

• L-leucineReach 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 leucine (cytosol to extracellular)

  Catalysed by ASL, SLC3A2, SLC7A6, SLC7A7cytosol, extracellular spaceOne of 12 routes

• 15(S)-HPETEReach from here →
  1. transport of fumarate and thiosulfate (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by SLC25A10, TSTD1, GPX5, GPX1 and 7 moremitochondrial matrix, cytosol

• CHEBI:15699Reach 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 homoserine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 3 routes

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

  Catalysed by FH, SLC25A1cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• formateReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)
  3. oxalate(in) + formate(out) = oxalate(out) + formate(in)

  Catalysed by SLC25A10, SLC25A14, SLC26A2, SLC25A30 and 2 moremitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• nicotinic acid D-ribonucleotideReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. Transport of Quinolinic Acid
  3. Nicotinate-nucleotide:pyrophosphate phosphoribosyltransferase (carboxylating)

  Catalysed by SLC25A10, SLC22A6, QPRTmitochondrial matrix, cytosol, extracellular space

• N-carbamoyl-L-aspartic acidReach 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 carbamoyltransferase

  Catalysed by ASL, ASS1, CADcytosol

• CHEBI:15971Reach 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 histidine (cytosol to extracellular)

  Catalysed by ASL, SLC7A5, SLC7A6cytosol, extracellular spaceOne of 11 routes

• ferricytochrome cReach 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, cytosolOne of 4 routes

• methanethiolReach from here →
  1. transport of fumarate and thiosulfate (cytosol to mitochondria)
  2. 3alpha-hydroxysteroid 3-dehydrogenase (Si-specific) (H2S2O3)
  3. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

  Catalysed by SLC25A10, SELENBP1mitochondrial matrix, cytosol

• dihydroxyacetoneReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol
  3. dihydroxyacetone + ATP = dihydroxyacetone phosphate + ADP + H(+)

  Catalysed by SDHB, GPD2, TKFC

• androsteroneReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. steroid sulfatase (androsterone sulfate)
  3. steroid sulfatase (androsterone sulfate)

  Catalysed by SLC25A10, STSmitochondrial matrix, cytosolOne of 3 routes

• a halide anionReach 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, cytosol

• NMN zwitterionReach 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. ribosylnicotinamide kinase (nicotinamide ribonucleoside)

  Catalysed by SDHB, NQO2, NMRK2, NMRK1cytosolOne of 2 routes

• ureaReach 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 3 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

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

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

  Catalysed by ASL, SLC7A9cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• 17beta-hydroxy-5alpha-androstan-3-oneReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. steroid sulfatase (5-alpha-dihydrotestosterone)
  3. steroid sulfatase (5-alpha-dihydrotestosterone)

  Catalysed by SLC25A10, STSmitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by SLC25A10, SLC26A6, SLC26A4mitochondrial matrix, cytosol, extracellular space

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

  Catalysed by FH, SLC25A1cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-valineReach 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 valine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 7 routes

• nitric oxideReach 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 (Nitric-Oxide-Forming)

  Catalysed by ASL, NOS3, NOS1, NOS2cytosolOne of 2 routes

• protein polypeptide chainReach from here →
  1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. PROTEIN_BS

  Catalysed by ASLcytosolOne of 2 routes

• pregnenoloneReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. Steryl-Sulfatase
  3. Pregnenolone-sulfate sulfohydrolase

  Catalysed by SLC25A10, STSmitochondrial matrix, cytosol, Golgi apparatusOne of 4 routes

• (S)-lactateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by FH, ME2, ME3, LDHA and 4 morecytosol, mitochondrial matrix

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by FH, CLYBL, AADATcytosol

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

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

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

  Catalysed by FH, ME2, ME3, KYAT1cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:16856Reach from here →
  1. transport of fumarate and thiosulfate (cytosol to mitochondria)
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by SLC25A10, TSTD1, GPX5, GPX1 and 7 moremitochondrial matrix, cytosolOne of 8 routes

• CHEBI:16857Reach 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 threonine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 5 routes

• N-acetyl-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. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by ASL, ASS1, NAT8Lcytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by FH, ME2, ME3, SLC16A4 and 5 morecytosol, mitochondrial matrix, extracellular spaceOne of 6 routes

• nicotinamideReach 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

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

  Catalysed by SDHB, GPD2, TPI1

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

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

• L-isoleucineReach 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 isoleucine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 6 routes

• CHEBI:17203Reach 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 proline (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 11 routes

• L-glutamic 5-semialdehydeReach from here →
  1. a quinone + succinate = fumarate + a quinol
  2. L-proline + a quinone = (S)-1-pyrroline-5-carboxylate + a quinol + H(+)
  3. aldehyde dehydrogenase (1-pyrroline-5-carboxylate)

  Catalysed by SDHB, PRODH, PRODH2, ALDH4A1cytosolOne of 2 routes

• itaconateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  3. cis-aconitate carboxy-lyase

  Catalysed by FH, SLC25A1, ACOD1cytosol, mitochondrial matrix

• estroneReach from here →
  1. transport of fumarate and sulfate (cytosol to mitochondria)
  2. estrone 3-sulfate + H2O = estrone + sulfate + H(+)
  3. Estrone 3-sulfate sulfohydrolase

  Catalysed by SLC25A10, STSmitochondrial matrix, cytosolOne of 3 routes

• propanoateReach 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. propanoate(out) + dehydroepiandrosterone 3-sulfate(in) = propanoate(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

• aceneuramateReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

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

• 5'-deoxyadenosineReach from here →
  1. (S)-malate hydro-lyase (fumarate-forming)
  2. (S)-malate:NAD+ oxidoreductase (decarboxylating)
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by FH, ME2, ME3, TYW1B and 1 morecytosol, mitochondrial matrix

• agmatineReach 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 carboxy-lyase (agmatine-forming)

  Catalysed by ASLcytosol, mitochondrial matrix

• L-cysteineReach 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 cysteine (cytosol to extracellular)

  Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 10 routes

• inosineReach 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 + H2O = inosine + phosphate

  Catalysed by ADSL, TNRC6B, ADSS2, ADSS1 and 4 morecytosolOne of 7 routes