F
IPR006361

Uroporphyrinogen decarboxylase HemE

InterPro entry
Short nameUroporphyrinogen_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

Cross References

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