3-fumarylpyruvate

Three steps

• orotateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. a quinone + succinate = fumarate + a quinol
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

  Catalysed by FAHD1, SDHB, DHODH

• isocitric acidReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. pyruvate kinase (dTDP)
  3. transport of isocitrate and PEP (cytosol to mitochondria)

  Catalysed by FAHD1, PKM, PKLR, SLC25A1cytosol, mitochondrial matrixOne of 5 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by FAHD1, PC, GOT2mitochondrial matrix

• (2R,3R)-tartrateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by FAHD1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by FAHD1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by FAHD1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by FAHD1, SLC25A10, SLC25A14, SLC25A30mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• cis-aconitic acidReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. pyruvate kinase (dTDP)
  3. transport of cis-aconitate and PEP (cytosol to mitochondria)

  Catalysed by FAHD1, PKM, PKLR, SLC25A1cytosol, mitochondrial matrixOne of 5 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by FAHD1, TYW1B, TYW1, IL4I1cytosol

• 2-oxohexanoateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by FAHD1, AGXT2

• D-cysteineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by FAHD1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-alpha-aminobutyric acidReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by FAHD1, AGXT2, GPTcytosol

• glyoxylateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)

  Catalysed by FAHD1, ME2, CLYBLOne of 8 routes

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA

  Catalysed by FAHD1, HMGCL, HMGCLL1mitochondrial matrix, cytosol

• 13-cis-retinalReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinal dehydrogenase (13-cis-retinal)

  Catalysed by FAHD1, SLC16A4, SLC16A5, SLC16A3 and 5 moreextracellular space, cytosol, endoplasmic reticulum

• L-ornithineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and ornithine (cytosol to extracellular)

  Catalysed by FAHD1, AGXT2, SLC7A6mitochondrial matrix, extracellular space, cytosolOne of 7 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

  Catalysed by FAHD1, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosol

• gamma-Glu-AlaReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. gamma-glutamyltransferase (alanine)

  Catalysed by FAHD1, AGXT2, GGT1, GGT5 and 4 moremitochondrial matrix, extracellular space, cytosol

• acetoacetyl-CoAReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate

  Catalysed by FAHD1, AACSmitochondrial matrix, cytosolOne of 2 routes

• glycineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-Alanine:glyoxylate aminotransferase

  Catalysed by FAHD1, AGXT2, AGXTmitochondrial matrixOne of 14 routes

• (S)-malyl-CoAReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by FAHD1, ME2, SUCLG1, SUCLA2

• (R)-methylmalonyl-CoAReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by FAHD1, AGXT2, ALDH6A1mitochondrial matrix

• butanoyl-CoAReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of butyrate and pyruvate (cytosol to extracellular)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by FAHD1, SLC16A4, SLC16A5, SLC16A3 and 9 moreextracellular space, cytosolOne of 2 routes

• propanoyl-CoAReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine
  3. 2-methyl-3-oxopropanoate + NAD(+) + CoA + H2O = propanoyl-CoA + hydrogencarbonate + NADH + H(+)

  Catalysed by FAHD1, AGXT2, ALDH6A1

• prostaglandin E1Reach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin E1(out) + 2 (S)-lactate(in) = prostaglandin E1(in) + 2 (S)-lactate(out)

  Catalysed by FAHD1, LDHA, LDHB, LDHC and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin F2alpha(out) + 2 (S)-lactate(in) = prostaglandin F2alpha(in) + 2 (S)-lactate(out)

  Catalysed by FAHD1, LDHA, LDHB, LDHC and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin H2Reach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin H2(out) + 2 (S)-lactate(in) = prostaglandin H2(in) + 2 (S)-lactate(out)

  Catalysed by FAHD1, LDHA, LDHB, LDHC and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin D2Reach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin D2(out) + 2 (S)-lactate(in) = prostaglandin D2(in) + 2 (S)-lactate(out)

  Catalysed by FAHD1, LDHA, LDHB, LDHC and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• D-alanineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by FAHD1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate

  Catalysed by FAHD1, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosol

• dihydroxyacetone phosphateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. a quinone + succinate = fumarate + a quinol
  3. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol

  Catalysed by FAHD1, SDHB, GPD2

• D-threonineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by FAHD1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• CMP-N-acetyl-beta-neuraminateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CTP:N-acetylneuraminate cytidylyltransferase

  Catalysed by FAHD1, NPL, CMAScytosol, nucleusOne of 2 routes

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

  Catalysed by FAHD1, SLC25A10, SGSHmitochondrial matrix, cytosol

• dehydroepiandrosterone 3-sulfateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by FAHD1, SLC25A10, STSmitochondrial matrix, cytosol

• L-tryptophanReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and tryptophan (cytosol to extracellular)

  Catalysed by FAHD1, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 8 routes

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

  Catalysed by FAHD1, SLC25A10, TSTD1mitochondrial matrix, cytosolOne of 2 routes

• L-threonineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by FAHD1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 5 routes

• L-kynurenineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by FAHD1, AGXT2, KYNUmitochondrial matrix

• beta-alanineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by FAHD1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-hydroxy-3-oxopropanoateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate aldose-ketose-isomerase

  Catalysed by FAHD1, AGXT, HYIcytosolOne of 2 routes

• L-asparagineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and asparagine (cytosol to extracellular)

  Catalysed by FAHD1, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 9 routes

• IMPReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)
  3. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)

  Catalysed by FAHD1, ADSL, TNRC6B, ADSS2 and 1 morecytosol

• L-phenylalanineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and phenylalanine (cytosol to extracellular)

  Catalysed by FAHD1, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 12 routes

• 3-hydroxy-L-kynurenineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by FAHD1, AGXT2, KYNUmitochondrial matrix

• L-homocysteineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by FAHD1, TYW1B, TYW1, BHMT2

• adenosine 5'-phosphosulfateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. ATP:sulfate adenylyltransferase

  Catalysed by FAHD1, SLC25A10, PAPSS1, PAPSS2mitochondrial matrix, cytosolOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by FAHD1, TYW1B, TYW1, BHMT2

• N-acetylputrescineReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetylputrescine + H2O = putrescine + acetate

  Catalysed by FAHD1, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosol

• (2R)-2-phosphoglycerateReach from here →
  1. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)
  2. pyruvate kinase (dTDP)
  3. 2-phospho-D-glycerate hydro-lyase (phosphoenolpyruvate-forming)

  Catalysed by FAHD1, PKM, PKLR, ENO1 and 2 morecytosol