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