Fig. 4From: The PPAR pan-agonist bezafibrate ameliorates cardiomyopathy in a mouse model of Barth syndromeEffects of BF on mitochondria. a Mitochondrial DNA (mtDNA) copy numbers were analyzed with qPCR of total DNA from cardiac tissues and normalized to nuclear DNA (nDNA) content. b Citrate synthase (CS) activities were measured in cardiac homogenates and normalized to protein concentrations. Data are depicted as fold changes relative to WT controls. c Enzymatic activity of RC segment I-III in mitochondria from 7 month-old WT and TazKD mice with and without BF treatment. Values are normalized to CS activity and are shown as fold-changes relative to WT controls. d Western blot. 20 μg protein samples from 7 month-old untreated WT, untreated TazKD, and BF-treated TazKD mouse hearts were analyzed. A cocktail of monoclonal antibodies specific to selected polypeptides of the RC complexes were used. Signal intensities were normalized relative to mitochondrial malate dehydrogenase (mMDH) and plotted for each markerBack to article page