Uroporphyrinogen Decarboxylase
Synonyms
Carboxy-Lyase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen III
Uroporphyrinogen Carboxy Lyase
Uroporphyrinogen Carboxy-Lyase
Uroporphyrinogen III Decarboxylase
Uroporphyrinogen-III carboxy-lyase
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.