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

One step

acetoacetateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
Catalysed by FAHcytosolOne of 2 routes
4-maleylacetoacetateReach from here →
1. 4-maleylacetoacetate = 4-fumarylacetoacetate
Catalysed by GSTZ1
fumaric acidReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
Catalysed by FAHcytosolOne of 2 routes

Two steps

(S)-3-hydroxybutanoateReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
Catalysed by FAH, ADHFE1cytosol
2-hydroxybutyric acidReach from here →
1. 4-fumarylacetoacetate fumarylhydrolase
2. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 1 morecytosol, extracellular spaceOne of 2 routes

Three steps

CHEBI:1146900Reach from here →
Catalysed by FAH, AACS, HMGCS1cytosolOne of 3 routes
pregnenolone sulfateReach from here →
Catalysed by FAH

a quinone

Reach from here →

Catalysed by FAH, SDHBcytosol

CHEBI:15345Reach from here →

Catalysed by FAH, AACScytosolOne of 3 routes

acetoneReach from here →

Catalysed by FAHcytosol, mitochondrial matrix

pyruvateReach from here →

Catalysed by FAHcytosol, mitochondrial matrixOne of 4 routes

all-trans-retinoic acidReach from here →

Catalysed by FAH, SLC16A4, SLC16A5cytosol, extracellular spaceOne of 2 routes

(S)-malateReach from here →

Catalysed by FAH, FHcytosolOne of 5 routes

sulfateReach from here →

Catalysed by FAH, SLC25A10cytosol, mitochondrial matrixOne of 2 routes

ubiquinonesReach from here →

Catalysed by FAH, SDHD, SDHB, SDHA and 1 morecytosol, mitochondrial matrix

3-fumarylpyruvateReach from here →

Catalysed by FAH, FAHD1cytosol

(R)-3-hydroxybutyric acidReach from here →

Catalysed by FAH, BDH1, BDH2cytosol, mitochondrial matrixOne of 4 routes

sulfiteReach from here →

Catalysed by FAH, SLC25A10cytosol, mitochondrial matrixOne of 2 routes

acetateReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A6 and 2 morecytosol, extracellular spaceOne of 2 routes

formic acidReach from here →

Catalysed by FAH, SLC16A4, SLC16A5cytosol, extracellular spaceOne of 2 routes

propionic acidReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 2 morecytosol, extracellular spaceOne of 2 routes

butyric acidReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 2 morecytosol, extracellular spaceOne of 2 routes

thiosulfateReach from here →

Catalysed by FAH, SLC25A10cytosol, mitochondrial matrixOne of 2 routes

(S)-lactic acidReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 2 morecytosol, extracellular spaceOne of 2 routes

(3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →

Catalysed by FAH, HMGCL, HMGCLL1cytosol

acetoacetyl-CoAReach from here →

Catalysed by FAH, AACScytosolOne of 2 routes

2-(N(omega)-L-arginino)succinateReach from here →

Catalysed by FAH, ASLcytosolOne of 2 routes

N(6)-(1,2-dicarboxyethyl)-AMPReach from here →

Catalysed by FAH, ADSL, TNRC6BcytosolOne of 2 routes

(2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →

Catalysed by FAH, ADSLcytosolOne of 2 routes

, SLC25A10

, STS

cytosol, mitochondrial matrix, Golgi apparatus

One of 2 routes

androsterone 3alpha-sulfateReach from here →

Catalysed by FAH, SLC25A10, STScytosol, mitochondrial matrixOne of 3 routes

prostaglandin E3Reach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 2 morecytosol, extracellular spaceOne of 2 routes

prostaglandin A2Reach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 2 morecytosol, extracellular spaceOne of 2 routes

prostaglandin J2Reach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 2 morecytosol, extracellular spaceOne of 2 routes

15-oxoprostaglandin F2alphaReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 2 morecytosol, extracellular spaceOne of 2 routes

all-trans-4-hydroxyretinoateReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, CYP26A1cytosol, extracellular spaceOne of 3 routes

a 1,4-benzosemiquinoneReach from here →

Catalysed by FAH, SDHB, CRYZ, TP53I3 and 2 morecytosol

N-acetylcadaverineReach from here →

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

cholesterol sulfateReach from here →

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

5alpha-dihydrotestosterone sulfateReach from here →

Catalysed by FAH, SLC25A10, STScytosol, mitochondrial matrixOne of 3 routes

all-trans-retinoyl-1-O-(beta-D-glucuronate)Reach from here →

Catalysed by FAH, SLC16A4, SLC16A5, UGT1A1 and 2 morecytosol, extracellular space, endoplasmic reticulum

all-trans-18-hydroxyretinoateReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, CYP1A1 and 24 morecytosol, extracellular spaceOne of 2 routes

a phenyl acetateReach from here →

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

an aryl sulfateReach from here →

Catalysed by FAH, SLC25A10, ARSL, ARSF and 4 morecytosol, mitochondrial matrix

acetylpyruvateReach from here →

Catalysed by FAH, FAHD1cytosol, mitochondrial matrixOne of 2 routes

CHEBI:15467Reach from here →

Catalysed by FAH, AACS, HMGCS1cytosolOne of 3 routes

propionyl-CoAReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, SLC16A7 and 5 morecytosol, extracellular spaceOne of 3 routes

10-formyltetrahydrofolic acidReach from here →

Catalysed by FAH, SLC16A4, SLC16A5, MTHFD1cytosol, extracellular spaceOne of 2 routes

malonateReach from here →

Catalysed by FAH, FH, SLC25A10cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

(R)-2-hydroxyglutarateReach from here →

Catalysed by FAH, ADHFE1cytosol, mitochondrial matrixOne of 2 routes

beta-nicotinamide D-ribosideReach from here →

Catalysed by FAH, SDHB, NQO2cytosol