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Recent advances in FRDA research have revealed the presence of several epigenetic modifications that are either directly or indirectly involved in this<jats:italic>FXN<\/jats:italic>gene silencing. Although epigenetic marks may be inherited from one generation to the next, modifications of DNA and histones can be reversed, indicating that they are suitable targets for epigenetic-based therapy. Unlike other trinucleotide repeat disorders, such as Huntington disease, the large expansions of GAA\u00b7TTC repeats in FRDA do not produce a change in the frataxin amino acid sequence, but they produce reduced levels of normal frataxin. Therefore, transcriptional reactivation of the<jats:italic>FXN<\/jats:italic>gene provides a good therapeutic option. 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