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Definition of Maleylacetoacetate
1. Noun. (biochemistry) An intermediate in the metabolism of tyrosine. ¹
¹ Source: wiktionary.com
Medical Definition of Maleylacetoacetate
1. An intermediate in l-phenylalanine and l-tyrosine catabolism; accumulates in certain inherited disorders of tyrosine metabolism. Maleylacetoacetate cis,trans-isomerase, an enzyme that catalyses the reversible conversion of maleylacetoacetate to 4-fumarylacetoacetate; an enzyme that participates in l-tyrosine catabolism; a deficiency of this enzyme is associated with tyrosinaemia type IB. Structure: -OOC-CH==CH-COCH2COCH2COO- (05 Mar 2000)