| hydroxymethylbilane synthase |
[EC 4.3.1.8] an enzyme of the lyase class that catalyzes the deamination and condensation of four molecules of porphobilinogen to form a linear tetrapyrrole intermediate, hydroxymethylbilane, which cyclizes to form uroporphyrinogen. Alone, the enzyme produces uroporphyrinogen I; in the presence of uroporphyrinogen synthase it produces uroporphyrinogen III, the physiologically important isomer. Deficiency of the enzyme, an autosomal dominant trait, results in acute intermittent porphyria. Called also porphobilinogen deaminase and uroporphyrinogen I synthase.
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