| uroporphyrinogen |
a porphyrinogen (q.v.) in which each pyrrole ring has one acetate side chain and one propionate side chain; it is formed by condensation of four molecules of porphobilinogen. Four isomers are possible but only two exist naturally, types I and III; the latter is a functional intermediate in heme biosynthesis while the former is produced in an abortive side reaction.
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| uroporphyrinogen decarboxylase |
[EC 4.1.1.37] an enzyme of the lyase class that catalyzes the decarboxylation of uroporphyrinogen III to coproporphyrinogen III in the biosynthesis of heme. Decreased enzyme activity is associated with porphyria cutanea tarda and the variant hepatoerythropoietic porphyria.
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| uroporphyrinogen I synthase |
hydroxymethylbilane synthase.
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| uroporphyrinogen III cosynthase |
uroporphyrinogen-III synthase.
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| uroporphyrinogen-III synthase |
[EC 4.2.1.75] an enzyme of the lyase class that acts concertedly with hydroxymethylbilane synthase to convert porphobilinogen to uroporphyrinogen III in the biosynthesis of porphyrins and heme. Deficiency of the enzyme, an autosomal recessive trait, causes congenital erythropoietic porphyria. Called also uroporphyrinogen III cosynthase.
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