Abstract
The final step in the biosynthesis of heme, i.e., the insertion of ferrous iron into the corresponding porphyrin, is catalyzed by the enzyme ferrochelatase (EC 4.99.1.1,), which is reported to be firmly bound to the inner phase of the mitochondrial inner membrane (2). The topography of the ferrochelatase means that the substrates (iron ions and porphyrinogens) as well as the products (hemes) have to penetrate the mitochondrial inner membrane. This contention raises the questions how the metabolites are transported across the mitochondrial membranes, and to what extent the transport interferes with the overall ferrochelatase reaction. To answer these questions, studies were undertaken on the ferrochelatase activity of ultrasonically treated rat liver mitochondria and of tightly coupled rat liver mitochondria.
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Romslo, I., Koller, M.E. (1978). The Regulatory Function of the Mitochondrial Inner Membrane in the Overall Ferrochelatase Reaction. In: Doss, M. (eds) Diagnosis and Therapy of Porphyrias and Lead Intoxication. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-67002-2_3
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DOI: https://doi.org/10.1007/978-3-642-67002-2_3
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