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J Bioenerg Biomembr. 1995 Apr;27(2):207-14.

Uroporphyrinogen decarboxylase.

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Department of Medical Biochemistry, University of Wales College of Medicine, Cardiff, England.


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.

[Indexed for MEDLINE]

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