UROD decarboxylates URO3 to COPRO3

Stable Identifier
R-HSA-189425
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
4 H(+) + uroporphyrinogen III => 4 CO2 + coproporphyrinogen III, Uroporphyrinogen III is decarboxylated to form coproporphyrinogen III
ReviewStatus
5/5
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Cytosolic uroporphyrinogen decarboxylase (UROD) catalyzes the sequntial removal of four carboxylic groups from the acetic acid side chains of uroporphyrinogen III (URO3) to form coproporphyrinogen III (COPRO3) (de Verneuil et al. 1983). Human UROD is a dimer (Whitby et al. 1998). Heterogenous and homogenous deficiencies of UROD are associated with porphyria cutanea tarda and hepatoerythropoietic porphyria respectively in vivo (Moran-Jiminez et al. 1996).
Literature References
PubMed ID Title Journal Year
8644733 Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria

Romana, M, Taieb, A, Topi, G, Moran-Jimenez, MJ, Ged, C, D'Alessandro, L, Enriquez De Salamanca, R, de Verneuil, H

Am J Hum Genet 1996
6822570 Purification and properties of uroporphyrinogen decarboxylase from human erythrocytes. A single enzyme catalyzing the four sequential decarboxylations of uroporphyrinogens I and III.

Sassa, S, de Verneuil, H, Kappas, A

J Biol Chem 1983
Participants
Participates
Catalyst Activity

carbon-carbon lyase activity of 2xUROD [cytosol]

Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created
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