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| Description: | Dibenzyl 2-Acetyl-3-oxohexanedioate is an intermediate formed in the synthesis of Succinylacetone (S692060), 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. |
| Molecular Weight: | 382.41 |
| Molecular Formula: | C22H22O6 |
| Solubility: | DCM, Methanol, Ethyl Acetate |
| Appearance: | oil |
| Storage: | Refrigerator |
| References: | Kvittingen, E., et al. Inher. Metab. Dis., 14, 554 (1991); Lindstedt, S., et al. Lancet, 340, 813 (1992); Schulze, A., et al. Clin. Chem., 47, 1424 (2001); Allard, P., et al. Clin. Biochem., 37, 1010 (2004). |
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