Fumarylacetoacetate

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Fumarylacetoacetate

HOOCCH=CHCOCH2COCH2COOH;an intermediate in phenylalanine and tyrosine catabolism; elevated in tyrosinaemia IA.

fumarylacetoacetate hydrolase, an enzyme that catalyses the hydrolysis of fumarylacetoacetate to fumarate and acetoacetate; a deficiency indicates tyrosinaemia IA.