Identification of 4,6-dioxoheptanoic acid (succinylacetone), 3,5-dioxooctanedioic acid (succinylacetoacetate) and 4-Oxo-6-hydroxyheptanoic acid in the urine from patients with hereditary tyrosinemia.
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.
D000090:Acetoacetates / Q000652:urine*; D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine*; D008401:Gas Chromatography-Mass Spectrometry; D006537:Heptanoates / Q000652:urine*; D006538:Heptanoic Acids / Q000652:urine*; D006801:Humans; D014443:Tyrosine / Q000378:metabolism*
D000090:Acetoacetates; D006537:Heptanoates; D006538:Heptanoic Acids; D014443:Tyrosine; C020804:succinylacetone; C030734:succinylacetoacetate; C036555:4-oxo-6-hydroxyheptanoic acid