succinylacetone

succinylacetone
A minor metabolite that is elevated in individuals with tyrosinemia IA.

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suc·ci·nyl·ac·e·tone (suk″sin-əl-asґə-tōn) the compound formed by decarboxylation of succinylacetoacetate, occurring at elevated levels in tyrosinemia type I, and believed to cause the characteristic hepatorenal damage. It competitively inhibits porphobilinogen synthase by being a structural analogue of δ-aminolevulinic acid, secondarily inhibits other enzymes of tyrosine metabolism, and is structurally similar to maleic acid, an inhibitor of renal tubular function.

Medical dictionary. 2011.

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