Uroporphyrinogen decarboxylase HemE (IPR006361)

Short name: Uroporphyrinogen_deCO2ase_HemE

Overlapping homologous superfamilies

Family relationships



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 [PMID: 9564029]. 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) [PMID: 7592567].

GO terms

Biological Process

GO:0006779 porphyrin-containing compound biosynthetic process

Molecular Function

GO:0004853 uroporphyrinogen decarboxylase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.