Reactome | UROD decarboxylates URO1 to COPRO1

UROD decarboxylates URO1 to COPRO1

Stable Identifier

R-HSA-190182

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

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

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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).

Literature References

PubMed ID	Title	Journal	Year
8644733	Uroporphyrinogen decarboxylase: complete human gene sequence and molecular study of three families with hepatoerythropoietic porphyria Romana, M, Taieb, A, Topi, G, Moran-Jimenez, MJ, Ged, C, D'Alessandro, L, Enriquez De Salamanca, R, 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. Sassa, S, de Verneuil, H, Kappas, A	J Biol Chem	1983