Coproporphyrinogen oxidase (EC 220.127.116.11) is the sixth enzyme of the heme biosynthetic pathway. This soluble protein is localized in the intermembrane space of mitochondria and catalyzes the conversion of 2 propionate groups at positions 2 and 4 of coproporphyrinogen III to 2 vinyl groups of protoporphyrinogen IX.[supplied by OMIM]
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