Coproporphyrinogen III oxidase

Coproporphyrinogen oxidase ( CPOX ) is called the enzyme that catalyzes the oxidation of coproporphyrinogen III to protoporphyrinogen IX, a partial reaction in the biosynthesis of porphyrins, which takes place in all living things. In eukaryotes, the substrate from the cytosol into the space between the inner and outer mitochondrial membrane is first transported, the transporter involved is still unknown. In humans, mutations in the gene can lead to CPOX CPOX deficiency and thus hereditary coproporphyria.

Catalyzed reaction

O2 2H ⇒ ⇒ 2CO2 2H2O

Coproporphyrinogen III is oxidized to protoporphyrinogen IX, with simultaneous carbon dioxide elimination. There will be one after the other instead of two oxidative decarboxylation. The enzyme requires no human metal ion as a cofactor.

Human Genome Organisation Pyruvate decarboxylation Digital Object Identifier PubMed Central
202166
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