Fumarylacetoacetate hydrolase (FAH)
Cofactors used
Clinical / pharmacological. Tyrosinaemia type I: liver failure, renal Fanconi, cabbage odour; treated with nitisinone
What it does, reaction by reaction
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples) Amino Acid & Nitrogen Metabolism · Cytosol + mitochondrion
Fumarylacetoacetate + H₂O → fumarate + acetoacetate
Converts Fumarylacetoacetate H2O into Fumarate Acetoacetate
› Notes
Fumarylacetoacetate hydrolase (FAH) yields one glucogenic product (fumarate) and one ketogenic product (acetoacetate). This establishes phenylalanine and tyrosine as both glucogenic and ketogenic.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
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