Succinylacetone

Succinylacetone is an inhibitor of delta-aminolevulinic acid dehydratase (Ki = 300 nM for human erythrocyte enzyme). It inhibits heme biosynthesis and decreases the growth of murine erythroleukemia cells when used at concentrations of 0.1 and 1 mM. Succinylacetone is an abnormal metabolite of tyrosine that accumulates in hereditary tyrosinemia type I, a disorder characterized by a deficiency in fumarylacetoacetate hydrolase (FAH), the final enzyme in tyrosine catabolism. Without functional FAH, fumarylacetoacetate is converted into succinylacetone. In a rat model of hypertyrosinemia, succinylacetone (40 mg/kg twice daily) increases levels of delta-aminolevulinic acid in urine, decreases heme levels in liver, kidney, spleen, and vascular tissues, and reduces sensitivity of isolated rat aortic rings to endothelium-dependent and -independent vasodilation. Increased levels of succinylacetone has been used as a marker of tyrosinemia type 1.Formal Name: 4,6-dioxo-heptanoic acid. CAS Number: 51568-18-4. Synonyms: NSC 174804. Molecular Formula: C7H10O4. Formula Weight: 158.2. Purity: >98%. Formulation: (Request formulation change), A crystalline solid. Solubility: Water: 25 mg/ml. lambdamax: 272 nm. SMILES: CC(CC(CCC(O)=O)=O)=O. InChi Code: InChI=1S/C7H10O4/c1-5(8)4-6(9)2-3-7(10)11/h2-4H2,1H3,(H,10,11). InChi Key: WYEPBHZLDUPIOD-UHFFFAOYSA-N.