Title : Biochemical studies on the enzymatic deficiencies in hereditary tyrosinemia.

Pub. Date : 1983 Oct 31

PMID : 6652907






3 Functional Relationships(s)
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1 Experiments are described on the effects of succinylacetone and fumarylacetoacetate on delta-aminolevulinic acid dehydratase, methionine adenosyltransferase and p-OH-phenylpyruvate dioxygenase. succinylacetone aminolevulinate dehydratase Homo sapiens
2 delta-Aminolevulinic acid dehydratase from human erythrocytes is inhibited non-competitively by succinylacetone (Ki 0.03 mumol/l) and by fumarylacetoacetate (Ki 0.06 mumol/l). succinylacetone aminolevulinate dehydratase Homo sapiens
3 It is concluded that secondary enzyme deficiencies observed in hereditary tyrosinemia (delta-aminolevulinic acid dehydratase, methionine adenosyl transferase) are the result of inhibition by succinylacetone and fumarylacetoacetate, accumulating as a result of a primary deficiency of fumarylacetoacetase. succinylacetone aminolevulinate dehydratase Homo sapiens