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Description
Category: Aliphatics, Isotope Labelled Compounds, Pharmaceuticals, Intermediates and Fine Chemicals /// Appearance: Beige to Pale Brown Solid /// Application Notes: Succinylacetone-13C7 is a labelled analogue of Succinylacetone (S692060). Succinylacetone (SA) is a specific marker for the inherited metabolic disease, hepatorenal tyrosinemia. It was developed a stable-isotope for the determination of SA in dried blood spots (DBS) and liquid urine using a 13C4-SA as internal standard. The method was applied retrospectively and prospectively for the diagnosis of hepatorenal tyrosinemia and for follow-up of patients under treatment. /// References: Kvittingen, E., et al.: Inher. Metab. Dis., 14, 554 (1991), Lindstedt, S., et al.: Lancet, 340, 813
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