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.
Also Known As:
Uroporphyrinogen III Decarboxylase; Carboxy-Lyase, Uroporphyrinogen; Decarboxylase, Uroporphyrinogen; Decarboxylase, Uroporphyrinogen III; Uroporphyrinogen Carboxy Lyase; Uroporphyrinogen Carboxy-Lyase; Uroporphyrinogen-III carboxy-lyase