Journal of Bioenergetics and Biomembranes

, Volume 27, Issue 2, pp 207–214 | Cite as

Uroporphyrinogen decarboxylase

  • G. H. Elder
  • A. G. Roberts


Uroporphyrinogen decarboxylase (EC catalyzes the decarboxylation of uroporphyrinogen III to coproporphyrinogen III. The amino acid sequences, kinetic properties, and physicochemical characteristics of enzymes from different sources (mammals, yeast, bacteria) are similar, but little is known about the structure/function relationships of uroporphyrinogen decarboxylases. Halogenated and other aromatic hydrocarbons cause hepatic uroporphyria by decreasing hepatic uroporphyrinogen decarboxylase activity. Two related human porphyrias, porphyria cutanea tarda and hepatoerythropoietic porphyria, also result from deficiency of this enzyme. The roles of inherited and acquired factors, including iron, in the pathogenesis of human and experimental uroporphyrias are reviewed.

Key words

Uroporphyrinogen decarboxylase porphyria iron heme biosynthesis uroporphyrinogen cytochromc P450IA hepatoerythropoietic porphyria porphyria cutanea tarda 


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Copyright information

© Plenum Publishing Corporation 1995

Authors and Affiliations

  • G. H. Elder
    • 1
  • A. G. Roberts
    • 1
  1. 1.Department of Medical BiochemistryUniversity of Wales College of MedicineCardiffEngland

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