CPOX : coproporphyrinogen oxidase


Description

The CPOX (coproporphyrinogen oxidase) is a protein-coding gene located on chromosome 3.

The CPOX gene provides instructions for producing the enzyme coproporphyrinogen oxidase, which is crucial for heme production. Heme is essential for all bodily organs, with the highest concentrations found in blood, bone marrow, and liver. It's a critical component of iron-containing proteins called hemoproteins, including hemoglobin, which carries oxygen in the blood. Heme production is a complex multi-step process involving eight distinct enzymes. CPOX plays a key role in the sixth step of this process by removing carbon and oxygen atoms from coproporphyrinogen III, a product of the fifth step, to produce protoporphyrinogen IX. In subsequent steps, two other enzymes modify protoporphyrinogen IX and incorporate an iron atom to generate heme.

CPOX catalyzes the aerobic oxidative decarboxylation of propionate groups on rings A and B of coproporphyrinogen-III. This reaction yields the vinyl groups in protoporphyrinogen-IX, which is a crucial step in the sixth stage of the heme biosynthesis pathway.

CPOX is also known as COX, CPO, CPX, HARPO, HCP.

Associated Diseases


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