Description
Succinylacetone (4,6-dioxoheptanoic acid), is a result of tyrosine metabolism disorder. The synthesis of this medium-chain keto acid in the body is due to the reduced activity of the enzyme fumarylacetoacetate hydrolase. Succinylacetone is an acidogen, its high concentrations can lead to metabolic acidosis, which leads to damage to the liver, kidneys, heart, and nervous system. Succinylacetone appears to be an oncometabolite because patients with high levels of this compound often develop hepatocellular carcinoma.
Succinylacetone is widely used in research as an inhibitor of the enzyme 5-aminolevulinic acid dehydratase (ALAD), which plays a key role in heme biosynthesis.
The product is used primarily as a control for MS/MS.
Chemical Properties
White Solid
Uses
4,6-Dioxoheptanoic acid has been used:
- as component of dendritic cell conditioning medium and in the inhibition of ALA dehydratase (ALAD) in dendritic cells(50)
- as an inhibitor of heme biosynthesis in human mononuclear cells(51) and HeLa cells(52)
- as a standard for the quantification of succinylacetone from Plasmodium falciparum cultures(53)
Uses
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.
Definition
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.
Biochem/physiol Actions
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.
IC 50
Human Endogenous Metabolite
