porphobilinogen synthase

(redirected from ALA dehydratase)
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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.

porphobilinogen synthase

References in periodicals archive ?
The enzyme ALA dehydratase, which catalyzes the condensation of 2 molecules of ALA to build PBG, is not a rate-limiting step in the heme biosynthetic pathway; therefore, an inhibitory effect of PBG on the enzyme has been hypothesized (4).
The increased ALA concentrations in AIP may reflect up-regulation of the biosynthetic pathway to compensate for heme deficiency and/or a possible inhibitory effect of PBG on ALA dehydratase (1, 4).
Molecular studies of the gene defect of ALA dehydratase deficiency porphyria: a new point mutation identified in a second German patient.