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).
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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