CONICET | Buscador de Institutos y Recursos Humanos

Frataxin is a nuclear-encoded mitochondrial protein highlyconserved in prokaryotes and eukaryotes. Its deficiency wasinitially described as the phenotype of Friedreichs ataxia, anautosomal recessive disease in humans. Although several functionshave been described for frataxin, that is, involvement inFe-S cluster and heme synthesis, energy conversion and oxidativephosphorylation, iron handling and response to oxidativedamage, its precise function remains unclear. Although there isa general consensus on the participation of frataxin in themaintenance of cellular iron homeostasis and in iron metabolism,this protein may have other specific functions in differenttissues and organisms.