ENTRY       EC 3.7.1.2
NAME        Fumarylacetoacetase
            beta-Diketonase
CLASS       Hydrolases
            Acting on carbon-carbon bonds
            In ketonic substances
SYSNAME     4-Fumarylacetoacetate fumarylhydrolase
REACTION    4-Fumarylacetoacetate + H2O = Acetoacetate + Fumarate
SUBSTRATE   4-Fumarylacetoacetate
            H2O
            3,5-Dioxo acid
            2,4-DIoxo acid
PRODUCT     Acetoacetate
            Fumarate
COMMENT     Also acts on other 3,5- and 2,4-dioxo acids.
PATHWAY     PATH: MAP00350  Tyrosine metabolism
DISEASE     MIM: 276700  Fumarylacetoacetase; Tyrosinemia, type I (3)
GENES       HSA: FAH(Hs.73875)
DBLINKS     University of Geneva ENZYME DATA BANK: 3.7.1.2
            WIT (What Is There) Metabolic Reconstruction: 3.7.1.2
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