uroporphyrinogen decarboxylase

Uroporphyrinogen III decarboxylase (uroporphyrinogen decarboxylase, or UROD) is an enzyme that in humans is encoded by the UROD gene. Function Uroporphyrinogen III decarboxylase is a homodimeric enzyme that catalyzes the fifth step in heme biosynthesis, which corresponds to the elimination of carboxyl groups from the four acetate side chains of uroporphyrinogen III to yield coproporphyrinogen III: uroporphyrinogen III coproporphyrinogen III + 4 CO2 Clinical significance