| succinylacetone |
the compound formed by decarboxylation of succinylacetoacetate, occurring at elevated levels in tyrosinemia, type I, and believed to cause the characteristic hepatorenal damage. It competitively inhibits porphobilinogen synthase by being a structural analog of δ-aminolevulinic acid, secondarily inhibits other enzymes of tyrosine metabolism, and is structurally similar to maleic acid, an inhibitor of renal tubular function.
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