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In the urine from patients with hereditary tyrosinemia, three characteristic compounds have been found. They have been identified as 4,6-dioxoheptanoic acid (succinylacetone), 3,5-dioxooctanedioic acid (succinyl-acetoacetate) and 4-oxo-6-hydroxyheptanoic acid. The identities have been established by mass spectrometry of several derivatives, and by comparison with a synthetic sample of 4,6-dioxoheptanoic acid.
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Lindblad et al. (1982) studied this question.
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