Succinylacetone (4,6-dioxoheptanoic acid), is a result of tyrosine metabolism disorder. The synthesis of this medium-chain keto acid in the body is due to the reduced activity of the enzyme fumarylacetoacetate hydrolase. Succinylacetone is an acidogen, its high concentrations can lead to metabolic acidosis, which leads to damage to the liver, kidneys, heart, and nervous system. Succinylacetone appears to be an oncometabolite because patients with high levels of this compound often develop hepatocellular carcinoma.
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