Coproporphyrinogen Oxidase

**Definition:** An enzyme that catalyzes the oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX by the conversion of two propionate groups to two vinyl groups. It is the sixth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by CPO gene. Mutations of CPO gene result in HEREDITARY COPROPORPHYRIA.

**Tree numbers:** - D08.811.682.660.250

**Synonyms:** - Coproporphyrinogen III Oxidases - Coproporphyrinogenase