An enzyme that catalyzes the decarboxylation of UROPORPHYRINOGEN III to coproporphyrinogen III by the conversion of four acetate groups to four methyl groups. It is the fifth enzyme in the 8-enzyme biosynthetic pathway of HEME. Several forms of cutaneous PORPHYRIAS are results of this enzyme deficiency as in PORPHYRIA CUTANEA TARDA; and HEPATOERYTHROPOIETIC PORPHYRIA.

Annotation

/defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC PORPHYRIA

DUI

D014575 MeSH Browser

CUI

M0022406

CAS

Uroporphyrinogen-III carboxy-lyase

Previous indexing

Porphyrinogens (1975); Carboxy-Lyases (1974-1975); Porphyrins (1974)

History note

91(76); was see under CARBOXY-LYASES 1976-90

Public note

91; was see under CARBOXY-LYASES 1976-90