IPR006361
Uroporphyrinogen decarboxylase HemE
InterPro entry
Short name | Uroporphyrinogen_deCO2ase_HemE |
Overlapping homologous superfamilies |
Description
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
[2]. 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)
[1].
References
1.Uroporphyrinogen decarboxylase. Elder GH, Roberts AG. J. Bioenerg. Biomembr. 27, 207-14, (1995). View articlePMID: 7592567
2.Crystal structure of human uroporphyrinogen decarboxylase. Whitby FG, Phillips JD, Kushner JP, Hill CP. EMBO J. 17, 2463-71, (1998). View articlePMID: 9564029
GO terms
biological process
molecular function
cellular component
- None
Cross References
ENZYME
Genome Properties
Representative structure
2eja: Crystal Structure Of Uroporphyrinogen Decarboxylase From Aquifex aeolicus