UROD decarboxylates URO1 to COPRO1

Stable Identifier
R-HSA-190182
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Uroporphyrinogen I is decarboxylated to form coproporphyrinogen I
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Cytosolic uroporphyrinogen decarboxylase (UROD) catalyzes the sequential removal of four carboxylic groups from the acetic acid side chains of uroporphyrinogen I (URO1) to form coproporphyrinogen I (COPRO1). UROD catalyzes this reaction less efficiently than the decarboxylation of uroporphyrinogen III (de Verneuil et al. 1983).

Literature References
PubMed ID Title Journal Year
6822570 Purification and properties of uroporphyrinogen decarboxylase from human erythrocytes. A single enzyme catalyzing the four sequential decarboxylations of uroporphyrinogens I and III.

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

J Biol Chem 1983
8644733 Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria

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

Am J Hum Genet 1996
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
uroporphyrinogen decarboxylase activity of 2xUROD [cytosol]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created