Whilst the mechanisms of altered lipid metabolism in FRDA are not completely understood it has been suggested that reduced PPARγ expression and its transcriptional regulator PGC-1α, could lead to reduced breakdown of lipids, specifically PUFAs, making them more readily available to undergo lipid peroxidation (Tamarit et al., 2016; Gaschler and Stockwell, 2017; Su et al., 2019). This evidence concerns the gene PPARGC1A and Friedreich ataxia.