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Frataxin-deficient models have increased total COX activity of cerebellar or B-lypmphocyte lysate. All FRDA models, KIKO, YG8 and patient B-lymphocytes showed 1.58-, 2.21- and 1.94-fold increase in COX activity respective to each model's control. Bars represent averages ± standard deviations (n = 3–4, P < 0.05*, P < 0.01**).
