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Fig. 4 | Orphanet Journal of Rare Diseases

Fig. 4

From: The PPAR pan-agonist bezafibrate ameliorates cardiomyopathy in a mouse model of Barth syndrome

Fig. 4

Effects 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 marker

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