Anti-PPOX antibody [EPR10400] - BSA and Azide free

Supplementary info

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

Activity summary

PPOX also known as protoporphyrinogen oxidase is an important enzyme in heme biosynthesis. It catalyzes the oxidation of protoporphyrinogen IX to protoporphyrin IX. The mass of PPOX is about 50 kDa and it is expressed in the mitochondria of cells across various tissues including the liver and bone marrow. PPOX's activity is important for producing heme which is an important component of hemoglobin.

Biological function summary

The enzyme PPOX contributes to heme production working within the mitochondrial membrane. It is part of the heme biosynthetic pathway and functions in converting protoporphyrinogen IX the last precursor to heme. This enzymatic action is important for producing heme groups required for hemoglobin myoglobin cytochromes and other heme-containing proteins. Without PPOX cells could not efficiently synthesize these important proteins.

Pathways

PPOX plays a role in the protoporphyrin IX biosynthesis pathway which forms part of the larger heme biosynthesis pathway. It interacts with other enzymes like coproporphyrinogen oxidase (CPOX) and ferrochelatase (FECH) which work sequentially in the pathway. These interactions ensure the completed heme molecule for functions like oxygen transport and electron transfer.

Associated diseases and disorders

Defects in PPOX are linked to variegate porphyria a type of porphyria causing skin and neurological symptoms. Mutations in the PPOX gene reduce enzyme activity leading to the accumulation of protoporphyrinogen IX. This disease connects PPOX with other enzymes in the heme pathway such as hydroxymethylbilane synthase (HMBS) which is involved in acute intermittent porphyria. Proper understanding of PPOX and its pathway interactions helps in managing and diagnosing such conditions.