Mitochondrial function in the presence of an import defect. Mitochondrial respiration during both state II (complex I driven) and state III (maximal) respiratory states (A) and the respiratory control ratios (B). ROS production under the same respiratory conditions, expressed per μg of mitochondria (C) and corrected for oxygen consumption (D). *P < 0.05, main effect of KD on absolute ROS emission. Representative blot for protein carbonylation in whole muscle lysates and the corresponding Ponceau stain for loading (E). (n = 4–6.) AFU, arbitrary fluorescence units; CTL, control intermyofibrillar mitochondria; KD, knockdown intermyofibrillar mitochondria; ROS, reactive oxygen species.