Mitochondria-penetrating peptides conjugated to desferrioxamine as chelators for mitochondrial labile iron

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.


 Mitochondria-penetrating peptides conjugated to desferrioxamine as chelators for mitochondrial labile iron