4-fumarylacetoacetate

362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API

143 compounds within 3 steps: 3 in 1, 24 in 2, 116 in 3

Three steps

3-oxopropanoateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate
Catalysed by FAH, AGXT2cytosol, mitochondrial matrix
D-serineReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. D-serine = pyruvate + NH4(+)
Catalysed by FAH, SRRcytosol, mitochondrial matrix
(R)-mevalonateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
3. (R)-mevalonate + 2 NADP(+) + CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + 2 NADPH + 2 H(+)
Catalysed by FAH, HMGCL, HMGCLL1, HMGCRcytosol
glyoxylateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. (S)-malate hydro-lyase (fumarate-forming)
3. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
Catalysed by FAH, FH, CLYBLcytosol
(R)-lactic acidReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. (R)-Lactate:NAD+ oxidoreductase
Catalysed by FAH, LDHDcytosol, mitochondrial matrix
2-iminopropanoateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. 2-iminopropanoate + H2O = pyruvate + NH4(+)
Catalysed by FAH, RIDAcytosol, mitochondrial matrix
phosphoenolpyruvic acidReach 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
13-cis-retinalReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and retinoate (cytosol to extracellular)
3. retinal dehydrogenase (13-cis-retinal)
Catalysed by FAH, SLC16A4, SLC16A5, ALDH1A2 and 1 morecytosol, extracellular space, endoplasmic reticulum
N-acetyl-beta-neuraminic acidReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
Catalysed by FAH, NPLcytosol, mitochondrial matrix
N-(acetyl)-sphing-4-enineReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space
a 3-acylpyruvateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)
Catalysed by FAH, FAHD1cytosol, mitochondrial matrix
succinyl-CoAReach 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, SUGCTcytosol
(3S)-3-hydroxybutanoyl-CoAReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate
3. (3S)-3-hydroxybutanoyl-CoA + NAD(+) = acetoacetyl-CoA + NADH + H(+)
Catalysed by FAH, AACS, HADH, HSD17B10cytosol
(S)-malyl-CoAReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. (S)-malate hydro-lyase (fumarate-forming)
3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate
Catalysed by FAH, FH, SUCLG1, SUCLA2cytosol
butanoyl-CoAReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and butyrate (cytosol to extracellular)
3. Butanoate:CoA ligase (AMP-forming)
Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 8 morecytosol, extracellular spaceOne of 2 routes
glutaryl-CoAReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate
Catalysed by FAH, HMGCL, HMGCLL1, SUGCTcytosol
N-acetyl-D-glucosamine 6-phosphateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate
Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space
dihydroxyacetone phosphateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. a quinone + succinate = fumarate + a quinol
3. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol
Catalysed by FAH, SDHB, GPD2cytosol
2-oxo-3-sulfanylpropanoateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
Catalysed by FAH, MPSTcytosol, mitochondrial matrix
2-methyl-3-oxopropanoateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine
Catalysed by FAH, AGXT2cytosol, mitochondrial matrix
L-methionineReach 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
N-sulfo-D-glucosamineReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and sulfate (cytosol to mitochondria)
3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate
Catalysed by FAH, SLC25A10, SGSHcytosol, mitochondrial matrix
dehydroepiandrosterone 3-sulfateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and sulfate (cytosol to mitochondria)
3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)
Catalysed by FAH, SLC25A10, STScytosol, mitochondrial matrix
glutathioneReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and sulfite (cytosol to mitochondria)
3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
Catalysed by FAH, SLC25A10, TSTD1cytosol, mitochondrial matrixOne of 2 routes
L-alanineReach 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 matrixOne of 18 routes
IMPReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
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 FAH, ADSL, TNRC6B, ADSS2 and 1 morecytosol
adenosine 5'-phosphosulfateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and sulfate (cytosol to mitochondria)
3. ATP:sulfate adenylyltransferase
Catalysed by FAH, SLC25A10, PAPSS1, PAPSS2cytosol, mitochondrial matrixOne of 4 routes
N-acetylputrescineReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. N-acetylputrescine + H2O = putrescine + acetate
Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space
glutathione disulfideReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and thiosulfate (cytosol to mitochondria)
3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
Catalysed by FAH, SLC25A10, TSTD1cytosol, mitochondrial matrix
5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase
Catalysed by FAH, ADSLcytosolOne of 2 routes
N(8)-acetylspermidineReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. N(8)-acetylspermidine + H2O = spermidine + acetate
Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space
(3S)-citramalyl-CoAReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA
Catalysed by FAH, CLYBLcytosol, mitochondrial matrix
an N-acetyl-L-cysteine-S-conjugateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetate and acetoacetate (cytosol to extracellular)
3. an N-acetyl-L-cysteine-S-conjugate + H2O = an S-substituted L-cysteine + acetate
Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 3 morecytosol, extracellular space
5-amino-2-oxopentanoateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine
Catalysed by FAH, AGXT2cytosol, mitochondrial matrix
S-sulfanylglutathioneReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and thiosulfate (cytosol to mitochondria)
3. thiosulfate + glutathione = S-sulfanylglutathione + sulfite + H(+)
Catalysed by FAH, SLC25A10, TSTD1cytosol, mitochondrial matrixOne of 3 routes
estrone 3-sulfateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and sulfate (cytosol to mitochondria)
3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)
Catalysed by FAH, SLC25A10, STScytosol, mitochondrial matrix
(4R)-4-hydroxy-2-oxoglutarateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate
Catalysed by FAH, HOGA1cytosol, mitochondrial matrix
(3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. a quinone + succinate = fumarate + a quinol
3. trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H(+)
Catalysed by FAH, SDHB, PRODH2cytosol
(5-hydroxyindol-3-yl)acetateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and sulfate (cytosol to mitochondria)
3. Transport of 5-Hydroxyindoleacetate
Catalysed by FAH, SLC25A10, SLC4A1cytosol, mitochondrial matrix, extracellular spaceOne of 2 routes
ubiquinol-10Reach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of fumarate and sulfite (cytosol to mitochondria)
3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate
Catalysed by FAH, SLC25A10, SQORcytosol, mitochondrial matrix
(4S)-4-hydroxy-2-oxoglutarateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate
Catalysed by FAH, HOGA1cytosol, mitochondrial matrix
O-3-oxobutanoyl-(R)-carnitineReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate
3. acetoacetyl-CoA + (R)-carnitine = O-3-oxobutanoyl-(R)-carnitine + CoA
Catalysed by FAH, AACS, CRATcytosol
2-(1-hydroxyethyl)thiamine diphosphateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
3. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)
Catalysed by FAH, PDHA1, PDHB, PDHA2cytosol, mitochondrial matrix