| succinyl coenzyme A |
the succinate monothioester of coenzyme A, a high energy intermediate formed in the tricarboxylic acid cycle (q.v.). It is also a precursor in the synthesis of porphyrins.
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| succinylacetoacetate |
a compound formed by reduction of fumarylacetoacetate; it occurs at elevated levels in tyrosinemia, type I, due to the deficiency of fumarylacetoacetase. It is readily decarboxylated to succinylacetone (q.v.); both compounds can cause hepatorenal damage.
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| 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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| succinyldihydrolipoamide |
succinyl bound to lipoamide, an intermediate in the reaction catalyzed by the α-ketoglutarate dehydrogenase complex.
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