Definition: 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.
/defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC PORPHYRIA
Other names Uroporphyrinogen Carboxy-Lyase; Uroporphyrinogen Carboxy Lyase; Decarboxylase, Uroporphyrinogen III; Decarboxylase, Uroporphyrinogen; Carboxy-Lyase, Uroporphyrinogen; Uroporphyrinogen III Decarboxylase
