This may lead to someone smarter than me digging a bit more re Friedrich's
Frataxin is a small, highly conserved protein, which is encoded in the nucleus, expressed in the cytoplasm as a precursor polypeptide, and then imported into the mitochondria. Early studies in yeast established a strong association between frataxin and mitochondrial iron, describing iron overload and susceptibility to oxidative stress when the frataxin homologue (Yfh1p) is deficient.31 In FRDA patients, endomyocardial biopsy analyses have revealed reduced activity of iron-sulphur cluster (ISC) containing subunits of the mitochondrial respiratory chain complexes I, II and II, and increased iron deposition.32 The ISCs are inorganic redox-active protein cofactors that are involved in a variety of cardinal functions within the cell, including oxidative phosphorylation, enzyme catalysis and gene regulation.
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