FECH binds Fe2+ to PRIN9 to form heme

Stable Identifier
R-HSA-189465
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Ferrous iron is inserted into protoporphyrin IX to form heme
Locations in the PathwayBrowser
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Ferrochelatase (FECH) catalyzes the insertion of ferrous iron into protoporphyrin IX (PRIN9) to form heme. FECH is localized on the matrix surface of the inner mitochondrial membrane and this reaction takes place within the mitochondrial matrix. The enzyme functions as a homodimer with each monomer containing a nitric oxide-sensitive 2Fe-2S cluster. Enzyme deficiency is associated with erythropoietic protoporphyria in vivo, and inhibition of ferrochelatase activity is a clinically important consequence of lead poisoning (Piomelli et al. 1987).

Literature References
PubMed ID Title Journal Year
11175906 The 2.0 A structure of human ferrochelatase, the terminal enzyme of heme biosynthesis.

Wu, CK, Dailey, HA, Rose, JP, Burden, A, Sellers, VM, Wang, BC

Nat Struct Biol 2001
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
ferrochelatase activity of 2x(FECH:2Fe-2S cluster) [mitochondrial matrix]
Physical Entity
Activity
This event is regulated
Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created