Reaction: UROD decarboxylates URO3 to COPRO3
- in pathway: Heme biosynthesis
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).
Reaction - small molecule participants:
COPRO3 [cytosol]
CO2 [cytosol]
URO3 [cytosol]
Reactome.org reaction link: R-HSA-189425
======
Reaction input - small molecules:
uroporphyrinogen III
Reaction output - small molecules:
coproporphyrinogen III
carbon dioxide
Reactome.org link: R-HSA-189425