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Description

Protoporphyrinogen oxidase (UniProt: P50336, also known as EC: 1.3.3.4, PPO, PPOX) is encoded by the PPOX gene (Gene ID: 5498) in human. Protoporphyrinogen oxidase is a mitochondrial inner membrane enzyme that catalyzes the 6-electron oxidation of protoporphyrinogen-IX to form protoporphyrin-IX. It is involved in step one of the subpathway that synthesizes protoporphyrin-IX from protoporphyrinogen-IX. It is expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney, and pancreas and may be present as a monomer or active homodimeric form. Defects in PPOX are linked to variegate porphyria that lead to severe deficiency of this enzyme that result in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver.