succinylacetoacetate

succinylacetoacetate
suc·ci·nyl·ac·e·to·ac·e·tate (suk″sin-əl-ə-se″to-asґə-tāt) a compound formed by reduction of fumarylacetoacetate; it occurs at elevated levels in tyrosinemia type I, due to the deficiency of fumarylacetoacetase. It is readily decarboxylated to succinylacetone (q.v.); both compounds can cause hepatorenal damage.

Medical dictionary. 2011.

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  • succinylacetone — A minor metabolite that is elevated in individuals with tyrosinemia IA. * * * suc·ci·nyl·ac·e·tone (suk″sin əl asґə tōn) the compound formed by decarboxylation of succinylacetoacetate, occurring at elevated levels in… …   Medical dictionary

  • tyrosinemia type I — an autosomal recessive disorder due to mutation in the FAH gene (locus: 15q23 q25), which encodes fumarylacetoacetase, an enzyme important in tyrosine catabolism. It is characterized by accumulation of succinylacetoacetate and succinylacetone,… …   Medical dictionary

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