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).
Romana, M, Taieb, A, Topi, G, Moran-Jimenez, MJ, Ged, C, D'Alessandro, L, Enriquez De Salamanca, R, de Verneuil, H
Sassa, S, de Verneuil, H, Kappas, A
uroporphyrinogen decarboxylase activity of 2xUROD [cytosol]
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