Journal de pathologie clinique et expérimentale

Abstrait

Mitochondrial ABC Transporters and Iron Metabolism

Alexandra Seguin and Diane McVey Ward

Mitochondrial are a key organelle in iron metabolism and many metabolic processes involved in iron homeostasis occur in the mitochondria. Eukaryotic cells have developed different transport mechanisms to deal with coordinating movement of iron and iron-related molecules across membranes. Some of those transport mechanisms involve ATP-binding cassette (ABC) transporters. There are four mitochondrial ABC transporters Abcb6, Abcb7, Abcb8 and Abcb10. Abcb6 is localized to the outer membrane of mitochondria where it is involved in porphyrin transport. Abcb7, Abcb8 and Abcb10 are localized to the inner mitochondrial membrane and the exact molecule transported by each is still unclear. Here, we provide a brief review of what is known about each transporter and its role in mitochondrial iron homeostasis. We describe the human diseases associated with known mutations in the genes encoding these proteins and discuss the possible importance of these transporters in immune cell function.