Navarro JA, Botella JA, Metzendorf C, Lind MI, Schneuwly S. Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich׳s ataxia. Free Radic Biol Med. 2015 Apr 2. pii: S0891-5849(15)00126-4. PMID: 25841783.
From the abstract: "Friedreich׳s ataxia is the most important recessive ataxia in the Caucasian population. Loss of frataxin expression affects the production of iron-sulfur clusters and, therefore, mitochondrial energy production. One of the pathological consequences is an increase of iron transport into the mitochondrial compartment leading to a toxic accumulation of reactive iron. ... frataxin-deficient flies were hypersensitive toward dietary iron and developed an iron-dependent decay of mitochondrial functions. ... mitoferrin downregulation improved many of the frataxin-deficient conditions ... whereas mitoferrin overexpression exacerbated most of them. ... demonstrates the crucial role of mitoferrin dysfunction in the etiology of Friedreich׳s ataxia and provides evidence that impairment of mitochondrial iron transport could be an effective treatment of the disease."