Figure 7.

(A) Mitochondrial morphometrical analysis. All mitochondria are counted in cross-sections of EDL muscle by EM. Number of mitochondria per 1 μm² of muscle fiber cross-sectional area is shown (n = 20). The percentage of area occupied by mitochondria in a cross-section of muscle fiber is not different in rmd and littermates (n = 20). The average total mitochondrial area per muscle fiber is larger in rmd compared with littermates (n = 20). *P < 0.005, **P < 0.0005. (B) mtDNA copy number is decreased in rmd compared with littermate controls. Copy number of mtDNA (ND1) was normalized by nuclear DNA (pcam1) (M; month-old, 1 M hindlimb: rmd; n = 4, littermates; n = 4. 2 M hindlimb: rmd; n = 5, littermates; n= 6, 3 M hindlimb: rmd; n = 4, littermates; n = 6. 2 M liver: rmd; n = 5, littermates; n = 5. 2 M forelimb: rmd; n = 6, littermates; n = 6).