Methylation is increased in FRDA patients directly upstream of the GAA repeat and further upstream at a CpG island encompassing the promoter region, first exon and first intron of the FXN gene (Greene et al., 2007; Al-Mahdawi et al., 2008, 2013; Castaldo et al., 2008; vans-Galea et al., 2012; Quesada et al., 2015). The gene discussed is FXN; the disease is Friedreich ataxia.