P22830

FECH (also designated Heme synthetase, Ferrochelatase) is the terminal enzyme of protoheme biosynthesis that catalyzes the ferrous form of iron insertion into protoporphyrin IX. Mature ferrochelatase is a homodimeric, mitochondrial membrane-associated protein translated downstream of an N-terminal 54-amino acid transit peptide. Ferrochelatase contains two nitric oxide (NO)-sensitive clusters and coordinated 2FE-2S clusters which may potentially serve as a nitric oxide sensor. Defects in the gene encoding the Ferrochelatase enzyme, FECH, cause erythropoietic protoporhyria (EPP), which is a dominantly inherited disease of porphyrin metabolism characterized by photosensitivity and hepatobiliary disease.