Ferrochelatase (FECH)
Cofactors used
Clinical / pharmacological. Erythropoietic protoporphyria; also inhibited by lead
What it does, reaction by reaction
Heme Biosynthesis (Porphyrin Pathway) Amino Acid & Nitrogen Metabolism · Mitochondrion + cytosol
Protoporphyrin IX + Fe²⁺ → heme (protoheme IX) + 2 H⁺
Converts Protoporphyrin IX Fe2+ into Heme (protoheme IX) H+
› Notes
Ferrochelatase (FECH) inserts ferrous iron into protoporphyrin IX on the matrix side of the inner mitochondrial membrane. This final step requires bioavailable Fe²⁺ and is effectively irreversible; lead also inhibits ferrochelatase.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
Heme Biosynthesis (Porphyrin Pathway)
Listed there as: Ferrochelatase
Ferrous iron availability; intact mitochondrial iron delivery
Lead; iron deficiency; FECH pathogenic variants
Indirectly coupled to erythropoiesis and iron-regulatory signaling
Recent literature
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