Additionally, administration of 3–10 mg/kg DMF increased mRNA expression and protein level of frataxin in lymphoblasts derived from patients with FRDA, spleen of YG8 mice and cerebellum of FXN KIKO mice, suggesting that increased transcription initiation and elongation arises from the removal of R-loops and transcriptional silencing of the FXN locus [122]. The gene discussed is FXN; the disease is Friedreich ataxia.