Porphobilinogen synthase | definition of porphobilinogen synthase by Medical dictionary
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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.
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References in periodicals archive
New type of hepatic porphyria with porphobilinogen synthase
defect and intermittent acute clinical manifestation.
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).
[delta]-aminolevulinic acid dehydrase (porphobilinogen synthase
) in two families with inherited enzyme deficiency.