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OMIM 612386) Ferrochelatase, the terminal enzyme of the heme biosynthesis pathways, resides in the inner mitochondrial membrane and catalyzes the insertion of ferrous iron into PP-IX to produce protoheme IX (heme).[67,68] Ferrochelatase is a homodimer with each subunit possessing a (2Fe-2S) cluster necessary for enzyme activity.[28] Heterogeneity of the molecular defects in the FECH gene is well established with more than 190 different mutations described, ranging from nonsense, missense, and splicing, to frameshift mutations.[69–71] Inherited deficiency of ferrochelatase in humans causes the disease erythropoietic protoporphyria (EPP) (OMIM #17700), wherein overproduction of free protoporphyrin in the bone marrow results in abnormally low levels (10%–30%) of normal FECH activity.[68,72] Protoporphyria is an autosomal dominant disease clinically characterized by photosensitivity and hepatobiliary disease, coexistent biochemically elevated protoporphyrin levels