porphobilinogen synthase


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por·pho·bi·lin·o·gen syn·thase

a liver enzyme catalyzing the formation of porphobilinogen and water from two molecules of δ-aminolevulinate, an important reaction in porphyrin biosynthesis; inhibited by lead in cases of lead poisoning; a deficiency of this enzyme results in elevated levels of δ-aminolevulinate and results in neurologic disturbances.
Synonym(s): δ-aminolevulinate dehydratase
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References in periodicals archive ?
New type of hepatic porphyria with porphobilinogen synthase defect and intermittent acute clinical manifestation.
Lead poisoning: a new biochemical perspective on the differentiation between acquired and hereditary neuroporphyria
Although aluminum may have a physiological role in the action of a few enzymes, such as succinic dehydrogenase and porphobilinogen synthase, it is typically monitored to evaluate toxicity in patients subjected to hemodialysis for renal failure (3, 4).
Effects of anticoagulants and contemporary blood collection containers on aluminum, copper, and zinc results
[delta]-aminolevulinic acid dehydrase (porphobilinogen synthase) in two families with inherited enzyme deficiency.
5-aminolevulinic acid dehydratase deficiency porphyria: a twenty-year clinical and biochemical follow-up

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