InterPro domain: IPR006361

This entry represents uroporphyrinogen decarboxylase (URO-D or HemE), which catalyzes the fifth step in the haem biosynthetic pathway, converting uroporphyrinogen III to coproporphyrinogen III by decarboxylating the four acetate side chains of the substrate [ 1 ]. This step takes the pathway toward protoporphyrin IX, a common precursor of both haem and chlorophyll, rather than toward precorrin 2 and its products.

This activity is essential in all organisms, and subnormal activity of URO-D leads to the most common form of porphyria in humans, porphyria cutanea tarda (PCT) as well as hepatoerythropoietic porphyria (HEP) [ 2 ].

1. Crystal structure of human uroporphyrinogen decarboxylase. EMBO J. 17, 2463-71
2. Uroporphyrinogen decarboxylase. J. Bioenerg. Biomembr. 27, 207-14