51568-18-4

White Solid

Succinylacetone (SA) is a specific marker for the inherited metabolic disease, hepatorenal tyrosinemia. It was developed a stable-isotope quid urine using a 13C4-SA as internal standard.

ChEBI: A dioxo monocarboxylic acid that is heptanoic acid in which oxo groups replace the hydrogens at positions 4 and 6. It is an abnormal metabolite of the tyrosine metabolic pathway and a marker for type 1 tyrosinaemia.

4,6-Dioxoheptanoic acid is an inhibitor of heme biosynthesis. It is a catabolic byproduct of tyrosine breakdown and inhibits δ-aminolevulinic acid (ALA) dehydratase enzyme. It is a potential marker for detecting tyrosinemia type I and is measured in dried blood spot of new borns.

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