The PPOX gene provides instructions for making an enzyme known as protoporphyrinogen oxidase. This enzyme is involved in the production of a molecule called heme. Heme is vital for all of the body’s organs, although it is most abundant in the blood, bone marrow, and liver. Heme is an essential component of iron-containing proteins called hemoproteins, including hemoglobin (the protein that carries oxygen in the blood).
The production of heme is a multi-step process that requires eight different enzymes. Protoporphyrinogen oxidase is responsible for the seventh step in this process, in which two hydrogen atoms are removed from protoporphyrinogen IX (the product of the sixth step) to form protoporphyrin IX. In the final step, another enzyme modifies protoporphyrin IX by inserting an iron atom to produce heme.
Other Names
PPO; PPOX_HUMAN; protoporphyrinogen dehydrogenase; protoporphyrinogen IX oxidase; protoporphyrinogenase; protox
Related Genetics Topics
Identifiers
- NCBI Gene: 5498
- OMIM: 600923
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
