4-fumarylacetoacetate

Three steps

• 2-methyl-3-oxopropanoic acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by FAH, AGXT2cytosol, mitochondrial matrix

• L-citrullineReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)

  Catalysed by FAH, ASL, ASS1cytosol

• oxaloacetateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. Pyruvate:carbon-dioxide ligase (ADP-forming)

  Catalysed by FAH, PCcytosol, mitochondrial matrixOne of 8 routes

• L-arginineReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by FAH, ASLcytosolOne of 2 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space

• (S)-lactateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by FAH, LDHA, LDHB, LDHC and 2 morecytosol, mitochondrial matrix

• quinolinic acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Transport of Quinolinic Acid

  Catalysed by FAH, SLC25A10, SLC22A6cytosol, mitochondrial matrix, extracellular spaceOne of 2 routes

• 2-oxobutanoateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by FAH, AGXT2cytosol, mitochondrial matrixOne of 2 routes

• 2-oxoglutaramateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by FAH, KYAT1cytosol, mitochondrial matrixOne of 2 routes

• ferrocytochrome cReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and L-lactate (cytosol to extracellular)
  3. (S)-Lactate:ferricytochrome-c 2-oxidoreductase

  Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 3 morecytosol, extracellular space, mitochondrial matrixOne of 2 routes

• oxalic acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. transport of oxalate and sulfate (cytosol to extracellular)

  Catalysed by FAH, SLC25A10, SLC26A6cytosol, mitochondrial matrix, extracellular space

• 3-hydroxypyruvateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by FAH, AGXTcytosol, mitochondrial matrix

• 17alpha-hydroxyprogesteroneReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxyprogesterone)

  Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space, endoplasmic reticulum

• aceneuramateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by FAH, NPLcytosol, mitochondrial matrix

• 5'-deoxyadenosineReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  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 FAH, TYW1B, TYW1cytosol, mitochondrial matrix

• 3-hydroxy-3-methylglutarateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate

  Catalysed by FAH, HMGCL, HMGCLL1, SUGCTcytosolOne of 3 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. a quinone + succinate = fumarate + a quinol
  3. L-proline + a quinone = (S)-1-pyrroline-5-carboxylate + a quinol + H(+)

  Catalysed by FAH, SDHB, PRODH, PRODH2cytosol

• enol-oxaloacetateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by FAH, FH, SDHB, SDHAcytosolOne of 3 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Steryl-Sulfatase

  Catalysed by FAH, SLC25A10, STScytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• 4-methylumbelliferyl acetateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)

  Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 5 morecytosol, extracellular space

• urateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and L-lactate (cytosol to extracellular)
  3. transport of L-lactate and urate (cytosol to extracellular)

  Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 3 morecytosol, extracellular space

• all-trans-retinalReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and retinoate (cytosol to extracellular)
  3. retinoate reduction

  Catalysed by FAH, SLC16A4, SLC16A5cytosol, extracellular space

• ubiquinolReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. succinate:quinone oxidoreductase
  3. thioredoxin-disulfide reductase (NADPH) (ubiquinone)

  Catalysed by FAH, SDHD, SDHB, SDHA and 3 morecytosol, mitochondrial matrixOne of 6 routes

• thiocyanateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by FAH, SLC25A10, MPST, TSTcytosol, mitochondrial matrixOne of 2 routes

• dTDPReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. pyruvate kinase (dTDP)

  Catalysed by FAH, PKM, PKLRcytosol, mitochondrial matrix

• hydrogen cyanideReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfite (cytosol to mitochondria)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by FAH, SLC25A10, MPST, TSTcytosol, mitochondrial matrixOne of 2 routes

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by FAH, AGXT2cytosol, mitochondrial matrix

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by FAH, AGXT2cytosol, mitochondrial matrix

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by FAH, AGXT2cytosol, mitochondrial matrix

• a quinolReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by FAH, FH, SDHB, SDHAcytosolOne of 13 routes

• folic acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. Folate Transport by Oats

  Catalysed by FAH, SLC25A10, SLC22A8, SLC22A7cytosol, mitochondrial matrix, extracellular spaceOne of 2 routes

• hydroxyacetoneReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. Acetoacetate carboxy-lyase (acetone-forming)
  3. cytochrome P450 (acetone)

  Catalysed by FAH, CYP2E1cytosol, mitochondrial matrix

• 17alpha-hydroxypregnenoloneReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetate and acetoacetate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxypregnenolone)

  Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space, endoplasmic reticulum

• a dicarboxylateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A35, SLC25A34cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• chlorine(.)Reach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and formate (cytosol to extracellular)
  3. transport of chloride and formate (cytosol to extracellular)

  Catalysed by FAH, SLC16A4, SLC16A5, SLC26A4cytosol, extracellular spaceOne of 3 routes

• hydrogen sulfideReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. 3alpha-hydroxysteroid 3-dehydrogenase (Si-specific) (H2S2O3)

  Catalysed by FAH, SLC25A10cytosol, mitochondrial matrixOne of 2 routes

• D-aspartateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. D-aspartate(in) + sulfate(out) = D-aspartate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-aspartateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)

  Catalysed by FAH, ASL, ASS1cytosolOne of 4 routes

• oxalateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A14, SLC26A2 and 3 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• citric acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Citrate Transport, Mitochondrial

  Catalysed by FAH, FH, SLC25A1cytosol, mitochondrial matrixOne of 5 routes

• maleateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• malonic acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfite (cytosol to mitochondria)
  3. transport of malonate and sulfite (cytosol to mitochondria)

  Catalysed by FAH, SLC25A10cytosol, mitochondrial matrixOne of 4 routes

• orotateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. a quinone + succinate = fumarate + a quinol
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

  Catalysed by FAH, SDHB, DHODHcytosol

• 3-hydroxypyruvic acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. L-Serine:pyruvate aminotransferase

  Catalysed by FAH, AGXTcytosol, mitochondrial matrixOne of 2 routes

• isocitric acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by FAH, FH, SLC25A1cytosol, mitochondrial matrixOne of 3 routes

• (2R,3R)-tartrateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by FAH, SLC25A10, SLC25A14, SLC25A30cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• cis-aconitic acidReach from here →
  1. 4-fumarylacetoacetate fumarylhydrolase
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by FAH, FH, SLC25A1cytosol, mitochondrial matrixOne of 3 routes