CPOX Proteins and Enzymes

The protein encoded by the CPOX gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is solubleand found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation ofcoproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditarycoproporphyria (HCP).