Frataxin deficiency results in increased oxidative stress and impaired recruitment of antioxidant defences.34 In line with previous studies YG8R mice had reduced protein levels of PGC‐1a and Nrf2 in either the spinal cord and/or cerebellum.35, 36 This was associated with global reductions in expression of antioxidant enzymes and enzymatic activity of the iron‐sulphur protein aconitase within the cerebellum28 (Table). This evidence concerns the gene PPARGC1A and hyperinsulinemic hypoglycemia, familial, 4.