This gene encodes the fifth enzyme of the heme biosynthetic pathway. This enzyme is responsible for catalyzing the conversion of uroporphyrinogen to coproporphyrinogen through the removal of four carboxymethyl side chains. Mutations and deficiency in this enzyme are known to cause familial porphyria cutanea tarda and hepatoerythropoetic porphyria.
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Protein Aliases: porphyrinogen carboxylase; UPD; URO D; URO-D; Uroporphyrinogen decarboxylase; uroporphyrinogen III decarboxylase
Gene Aliases: AI323803; PCT; porphyrinogen carboxy-lyase; UPD; Uro-d; UROD