Compared with healthy individuals, both frataxin and OXCT1 levels were significantly decreased in the skeletal muscle homogenates of FRDA patients (Fig. 5A and B; 69% and 57% decrease for frataxin and OXCT1, respectively, n = 5,**P < 0.01), providing further evidence that frataxin deficiency decreases OXCT1 levels. The gene discussed is FXN; the disease is Friedreich ataxia.