Anti-CPOX antibody [EPR11927(B)]

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

CPOX or coproporphyrinogen oxidase catalyzes the oxidation of coproporphyrinogen III to protoporphyrinogen IX an important step in heme biosynthesis. CPOX possesses a molecular mass of approximately 37–40 kDa. Expression occurs primarily in the mitochondria of liver and bone marrow cells. This enzyme plays a role in maintaining the balance of porphyrin intermediates and prevents accumulation that may cause cellular toxicity.

Biological function summary

CPOX converts coproporphyrinogen III into protoporphyrinogen IX playing a role in the heme biosynthetic pathway. As part of the terminal steps in heme production this transformation is important for synthesizing heme an essential component of hemoglobin myoglobin and various cytochromes. CPOX is not part of any larger enzyme complex but interacts with adjacent enzymes in the metabolic pathway to ensure efficient heme synthesis.

Pathways

CPOX functions in the heme biosynthesis pathway. This pathway is vital for the production of heme enabling the synthesis of hemoglobin and electron transport chains. CPOX acts sequentially after coproporphyrinogen III oxidase and before protoporphyrinogen IX oxidase. The enzyme works in concert with proteins such as uroporphyrinogen III synthase (UROS) in earlier steps of the pathway affecting overall heme production efficiency.

Associated diseases and disorders

Mutations or defects in CPOX can lead to hereditary coproporphyria (HCP) a disorder characterized by neurovisceral attacks and photosensitivity. This condition results from the accumulation of porphyrins due to a dysfunctional heme synthesis pathway. Additionally CPOX-related dysfunction can have implications in liver disorders affecting the balance of heme precursors. Improved understanding of CPOX and its interactions with proteins like UROS can provide insight into therapeutic targets for these conditions.