Reactome | FECH binds Fe2+ to PRIN9 to form heme

FECH binds Fe2+ to PRIN9 to form heme

Stable Identifier

R-HSA-189465

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

Fe(2+) + protoporphyrin IX => 2 H(+) + heme b, Ferrous iron is inserted into protoporphyrin IX to form heme

ReviewStatus

5/5

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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