Emphasizing the need for the precise regulation of mitochondrial iron, increased expression of mitoferrin-1 has also been linked to neuronal disorders such as Friedreic ataxia (FRDA), a disease characterized by neurodegeneration and cardiomyopathy caused by the loss of function of frataxin, a protein involved in generation of Fe/S clusters in mitochondria. This evidence concerns the gene FXN and Friedreich ataxia.