Figure 5: Respiration Is Normal, but Gene Expression Is Altered in Mitochondria Isolated From

(A) Respiration and mitochondrial electron transport chain complex activity in mitochondria isolated from 3 pooled wt and 3 pooled αMHC403/+ hearts. (B) Mean ± SD of MtDNA copy number normalized to 18S rDNA. (C) Mean ± SD of gene expression of αMHC403/+ MtDNA copy number regulators TFAM, PPARγ and PGC1 relative to wt. (D) Mean ± SD of FGF21. (E and F) Representative TEM images demonstrating disordered distribution of mitochondria in αMHC403/+ (F) versus wt (E) heart sections. M = mitochondria; S = sarcomere. Scale = 1 μm. (G and H) Confocal imaging demonstrating disordered mitochondrial distribution and disorganization of F-actin in representative αMHC403/+ (H) versus wt (G) myocytes. Mitochondria shown in red (MitoTracker), F-actin shown in green (phalloidin), nuclei shown in blue (DAPI). Scale = 10 μm. The Mann-Whitney test was used for all comparisons. MtDNA = mitochondrial DNA; TEM = transmission electron microscopy; wt = wild type.

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