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uroporphyrin the porphyrin (q.v.) produced by oxidation of the methylene bridges in uroporphyrinogen. Excessive amounts of uroporphyrin I are excreted in congenital erythropoietic porphyria, and both types I and III are excreted in porphyria cutanea tarda. See uroporphyrinogen.
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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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