delta-aminolevulinic acid synthase

(redirected from d-aminolevulinic acid synthase)

δ-a·mi·no·lev·u·lin·ic ac·id syn·thase

an enzyme that catalyzes the reaction of succinyl-CoA with glycine to form δ-aminolevulinate, coenzyme A, and CO2. The committed step in porphyrin biosynthesis.
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Decreased PBGD activity can reduce the conversion of porphobilinogen (PBG) into hydroxymethylbilane, which in turn attenuates the feedback inhibition and increases the activity of d-aminolevulinic acid synthase (d-ALAS), thus increasing the levels of ALA and PBG and consequently urinary excretion thereof.
A novel mutation, IVS2-2AG, associated with acute intermittent porphyria in a Chinese family

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