7 nmol/mL, respectively; however, succinylacetone
was not detected in the urine.
NeoMass AAAC is used with Tandem Mass Spectrometry to detect concentrations of amino acids, free carnitine, acylcarnitines, succinylacetone
and argininosuccinic acid.
This reduced dosing frequency is approved for use in patients five years of age and older who have undetectable serum and urine succinylacetone
concentrations after a minimum of four weeks on a stable dosage of nitisinone.
As a result of the enzyme block, hepatotoxic intermediates such as maleyland fumarylacetoacetic acids accumulate and are ultimately metabolized to succinylacetone
(SUAC), a highly specific marker for TYR1 (1) (Fig.
Tenders are invited for 77 kit - Neobase Succinylacetone
Solution Pack to analyze newborn screening specimens properly for Tyrosenimia Type I which is required by law.
The accumulation of the toxic metabolites succinylacetone
and fumarylacetoacetate in the liver and kidneys leads to death if not treated.
is an inhibitor of the heme synthesis pathway, causing neurotoxicity that is similar to lead poisoning or aminolevulinic acid dehydratase (ALAD) deficiency porphyria (Wyllie & Hyams, 2006).
Eventually, by gas-liquid chromatography-mass-spectrometry, they identified succinylacetone
and succinylacetoacetate in the urine from these patients as the enzyme inhibitors.
Newborn screening for hepatorenal tyrosinemia: Tandem mass spectrometric quantification of succinylacetone
is a competitive inhibitor of 8-aminolevulinate dehydratase (enzyme-inhibitor dissociation constant [K.
In addition, succinylacetone
and succinylacetoacetate are found in the urine.