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Figure 2 mtDNA Amount and Expression of Genes Involved in Mitochondrial Biogenesis and FAO

(A) The mitochondrial deoxyribonucleic acid (mtDNA) amount is significantly increased in MIC compared with NF hearts. *p < 0.001 versus NF, DCM, and IHD. (B) The mRNA levels of PGC1-, NRF1, NRF2, and Tfam are coordinately up-regulated in MIC and down-regulated in DCM and IHD. *p < 0.001 versus NF; p < 0.05 versus NF. (C) Expression levels of PGC1-ß are slightly increased in MIC and IHD and decreased in DCM respect to NF. *Differences between each group and all the others are statistically significant (p < 0.001). (D) Both PPAR- and its targets mCPT-I, MCAD, and LCAD are up-regulated in MIC and down-regulated in DCM and IHD hearts. *p < 0.001 versus NF; p < 0.01 versus NF. Yellow = NF; red = MIC; blue = DCM; green = IHD. DCM = dilated cardiomyopathy; FAO = fatty acid oxidation; IHD = ischemic heart disease; LCAD = long-chain acyl-CoA dehydrogenasey; MCAD = medium-chain acyl-CoA dehydrogenase; mCPT-I = carnitine palmitoyl transferase muscle isoform; MIC = maternally inherited cardiomyopath; NF = nonfailing; NRF = nuclear respiratory factor; PGC = peroxisome proliferator activated receptor coactivator; PPAR = peroxisome proliferator activated receptor; Tfam = transcription and mitochondrial deoxyribonucleic acid maintenance factor.