Coproporphyrinogen III oxidase

Coproporphyrinogen-III oxidase, mitochondrial is an is_associated_with::enzyme that in humans is encoded by the CPOX is_associated_with::gene. A genetic defect in the enzyme results in a reduced production of is_associated_with::heme in animals. The medical condition associated with this enzyme defect is called is_associated_with::hereditary coproporphyria.

It is an enzyme involved in the sixth step of is_associated_with::porphyrin metabolism it catalyses the oxidative is_associated_with::decarboxylation of is_associated_with::coproporphyrinogen III to proto-porphyrinogen IX in the is_associated_with::haem and is_associated_with::chlorophyll biosynthetic pathways. The is_associated_with::protein is a homodimer containing two internally bound is_associated_with::iron is_associated_with::atoms per is_associated_with::molecule of native protein. The is_associated_with::enzyme is active in the presence of molecular oxygen that acts as an is_associated_with::electron acceptor. The enzyme is widely distributed having been found in a variety of is_associated_with::eukaryotic and is_associated_with::prokaryotic sources.