Reaction: FECH binds Fe2+ to PRIN9 to form heme
- in pathway: Heme biosynthesis
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).
Reaction - small molecule participants:
H+ [mitochondrial matrix]
heme [mitochondrial matrix]
PRIN9 [mitochondrial matrix]
Fe2+ [mitochondrial matrix]
Reactome.org reaction link: R-HSA-189465
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Reaction input - small molecules:
protoporphyrin
iron(2+)
Reaction output - small molecules:
hydron
ferroheme b
Reactome.org link: R-HSA-189465