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Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

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Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
Cofactors and co-substrates are written beside the reaction when you zoom in
Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
Compartment transfer of the molecule it is linked to
Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

FAH cleaves 4FAA

4FAA + H2O → FUMA + ACA + H+

Reactome: R-HSA-71181
Compartment: cytosol
Rhea: RHEA:10245

Inputs

4FAACHEBI:18034, C8H6O6, cytosol
H2OCHEBI:15377, H2O, cytosol

Outputs

FUMACHEBI:29806, C4H2O4, cytosol
ACACHEBI:13705, C4H5O3, cytosol
H+CHEBI:15378, H, cytosol

Catalysed by

fumarylacetoacetase activity of FAH dimer [cytosol]EC 3.7.1.2

Literature

Characterization of the human fumarylacetoacetate hydrolase gene and identification of a missense mutation abolishing enzymatic activity. Hum Mol Genet, 1993
Purification and properties of a diketo acid hydrolase from beef liver Biochemistry, 1972
Hereditary tyrosinaemia type I: from basics to progress in treatment Ann Med, 2000
The genetic tyrosinemias Am J Med Genet C Semin Med Genet, 2006