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OMIM 613521) Uroporphyrinogen decarboxylase (UROD), a homodimeric housekeeping enzyme, is the fifth enzyme in the pathway and the final cytoplasmic enzyme in the synthesis of heme.[51] Unlike its predecessors, UROD contains no cofactors or metal ions and catalyzes the sequential removal or decarboxylation of the four-pyrrole acetic acid side chains of uroporphyrinogen to yield coproporphyrinogen III.[51] After these reactions coproporphyrinogen III is transported into the mitochondrial intermembrane space, while other partially decarboxylated intermediates are oxidized to the corresponding porphyrins and are eliminated from the cell into the plasma