Taken together, the lower activity of the ETC and deficient muscle mitochondrial respiration [9] , [55] , elevated Nrf2 protein content [28] and a strong trend for COX activity to be lower [30] in MCD patients, combined with elevated MnSOD protein content and activity in the current study, suggest that mitochondria are an important source of ROS generation in skeletal muscle of MCD patients ( Fig. 3 ).
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