Ferrochelatase

Ferrochelatase (FECH, protoheme ferrolyase) is an enzyme that catalyses the terminal (eighth) step in the biosynthesis of is_associated_with::heme, converting is_associated_with::protoporphyrin IX into is_associated_with::heme. It catalyses the reaction:
 * protoporphyrin + Fe++ ↔ is_associated_with::protoheme + 2 H+.

Function
Ferrochelatase is localized to the mitochondrion where it catalyzes the insertion of the ferrous form of iron into protoporphyrin IX in the heme synthesis pathway. Two transcript variants encoding different isoforms have been found for this gene.



Structure
A ferrochelatase enzyme consists of 497 amino acid residues with a m.w. of 55.4 kDa.

Clinical significance
Defects in ferrochelatase are associated with is_associated_with::erythropoietic protoporphyria.

Interactions
Ferrochelatase has been shown to interact with is_associated_with::ABCB7.