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    Hereditary tyrosinemia - fumarylacetoacetase deficiency

    Succinylacetone which has been isolated from urine from patients with hereditary tyrosinemia inhibits porphobilinogen synthase (EC 4.2.1.24) both in liver and erythrocytes. It accumulates together with succiny...

    S -P Fällström, B Lindblad, S Lindstedt, G Steen in Pediatric Research (1979)