Reactome | UROD decarboxylates URO3 to COPRO3

UROD decarboxylates URO3 to COPRO3

Stable Identifier

R-HSA-189425

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

4 H(+) + uroporphyrinogen III => 4 CO2 + coproporphyrinogen III, Uroporphyrinogen III is decarboxylated to form coproporphyrinogen III

ReviewStatus

5/5

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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).

Literature References

PubMed ID	Title	Journal	Year
8644733	Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria Moran-Jimenez, MJ, Ged, C, Romana, M, Enriquez De Salamanca, R, Taieb, A, Topi, G, D'Alessandro, L, de Verneuil, H	Am J Hum Genet	1996
6822570	Purification and properties of uroporphyrinogen decarboxylase from human erythrocytes. A single enzyme catalyzing the four sequential decarboxylations of uroporphyrinogens I and III. de Verneuil, H, Sassa, S, Kappas, A	J Biol Chem	1983