Uroporphyrinogen III is decarboxylated to form coproporphyrinogen III Authored: Jassal, B, D'Eustachio, P, 2007-01-24 10:19:49 Cytosolic uroporphyrinogen decarboxylase (UROD) catalyzes the sequntial removal of four carboxylic groups from the acetic acid side chains of uroporphyrinogen III (uro'gen III) to form coproporphyrinogen III (copro'gen III) (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). EC Number: 4.1.1.37 Edited: Jassal, B, D'Eustachio, P, 2007-01-24 10:19:49 Pubmed6822570 Pubmed8644733 Pubmed9564029 Reactome Database ID Release 43189425 Reactome, http://www.reactome.org ReactomeREACT_9422 Reviewed: Sassa, S, 2007-01-24 10:18:36 has a Stoichiometric coefficient of 4