Succinyl<wbr/>acetone

Succinylacetone

CAT N°: 25501
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From 48.00 40.80

Succinylacetone is an inhibitor of ?-aminolevulinic acid dehydratase (Ki = 300 nM for human erythrocyte enzyme).{36832} It inhibits heme biosynthesis and decreases the growth of murine erythroleukemia cells when used at concentrations of 0.1 and 1 mM.{36833} 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.{36834} Without functional FAH, fumarylacetoacetate is converted into succinylacetone. In a rat model of hypertyrosinemia, succinylacetone (40 mg/kg twice daily) increases levels of ?-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.{36835}

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