Alta RYP, Vitorino HA, Goswami D, Liria CW, Wisnovsky SP, Kelley SO, Machini MT, Espósito BP. (2017). PLoS ONE 12(2): e0171729. doi:10.1371/journal.pone.0171729
Diseases such as hereditary hemochromatosis or thalassemia are characterized by non-localized deposits of excess iron, however a number of conditions are known in which iron overload is circumscribed to a specific tissue or organelle (neurodegeneration with brain iron accumulation, hereditary X-linked sideroblastic anemia, anemia of chronic disease. Patients of FA typically have a decreased expression of the peptide frataxin, which assists the assembly of iron-sulfur clusters within the mitochondria.
In conclusion, the production of mtDFO is an interesting strategy to load a powerful, yet impermeant siderophore into either cytoplasm or mitochondria. This could contribute to the improvement of several iron overload conditions, where iron excess is either systemic or localized into specific tissues/organelles. Results indicate that mtDFO could be promising compounds for amelioration of the disease symptoms of iron overload in mitochondria.
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