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Ferrochelatase (FECH)

Target: FECH
Molecular classification: Enzyme

Overview

Ferrochelatase is an essential mitochondrial enzyme that completes the biosynthetic pathway for heme by inserting ferrous iron into protoporphyrin IX. Its structure includes specialized domains for substrate handling and catalysis—including a vital [2Fe–2S] cluster—and its function integrates tightly with cellular metabolism through regulatory protein interactions. Deficiency or mutation leads directly to human disease due to impaired hemoglobin production.

Mechanism of action

Inhibiting ferrochelatase leads to accumulation of protoporphyrin IX

Biological functions

Heme biosynthesisIron insertionRegulation of heme synthesis

Disease associations

Erythropoietic protoporphyria

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