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. 2014 May 23;229(9):1283–1291. doi: 10.1002/jcp.24567

Figure 4.

Figure 4

Rescue of muscle function by mAAV8-Jazz treatment. a: Mechanical response of isolated muscles. Effects of changes in voltage on isotonic contractions in EDL and abdominal muscles, from control and mAAV8-Jazz-treated mdx mice. Muscle contractile force for each voltage was determined and considered as a percentage of maximal contraction. Each point represents the mean of 10 EDL muscles (5 animals) and five abdominal muscle strips, with error bars indicating SEM and **P < 0.01 indicating the statistical significance by t-test. b: Procion Orange dye uptake in sections of abdominal and EDL muscles after force test. Left: Representative images demonstrating the increased ability of mAAV8-Jazz-treated mdx mice to exclude dye from stretched fibers. Right: graph shows the mean (±SEM) area of dye-positive fibers expressed as the percentage of the total CSA of muscle sections. c: Single session performance (left) and total running time (right) relative to four weekly treadmill trials with exhaustive exercise protocol. The mdx mice injected with saline and mAAV8-EGFP (controls) or mAAV8-Jazz at day 5 after birth were tested at 3 months of age. For each group, lines indicate the mean duration of running time during each trial (left), and columns indicate the cumulative running time over the four consecutive trials (right). Number of animals was 10 for each experimental group. The mAAV8-Jazz-treated mdx mice showed a significant improvement of exercise performance as compared to mdx and to mAAV8-EGFP-treated mdx mice. Statistical analysis with “unpaired t-test” showed a significant main effect of treatment on mice performance indicated by **P < 0.01 and ***P < 0.001. d: Evan's blue dye (EBD) uptake was used to compare skeletal muscle membrane integrity after exercise. Top: abdominal muscle and diaphragm muscles from untreated and mAAV8-EGFP-treated (controls) or mAAV8-Jazz-treated mdx mice were monitored for EBD uptake by fluorescence microscope. EBD uptake (red) was significantly higher in sections of control mdx mice as compared with mAAV8-Jazz-treated mdx mice. Bottom: Evans blue uptake was also scored as a percentage of Evans blue positive myofibers *P < 0.05 and ***P < 0.001. Scale Bar: 50 μm.