Antioxidant Treatment Reduces Formation of Structural Cores and Improves Muscle Function in RYR1^Y522S/WT Mice

Figure 5

Effects of long-term NAC treatment at 10 months of age. (a) Quantitative analysis of EDL fibers presenting the features classified in Figures 1(a), 1(b), and 1(c) shown as percentage of fibers analyzed (white: normal fibers; grey: fibers with unstructured cores; and dark grey: fibers with contracture cores). See also Table S4. (b and c) Serum levels of creatine kinase (CK) and lactate dehydrogenase (LDH). (d) Time course of grip strength from 4 (beginning of long-term NAC treatment) to 10 months (end of long-term NAC treatment) of age expressed as force on body weight (N/g). (e) Change in grip strength from 4 to 10 months of age (shown as a percentage). (f and g) Force-frequency (1–140 Hz) relationship curves of specific force (f) and specific force during a single 2 s, 120 Hz stimulation train (g) recorded for the same EDL muscles. In s (b–g), data are given as mean ± SEM; , WT versus RYR1^Y522S/WT mice; ^#, untreated RYR1^Y522S/WT versus NAC-treated RYR1^Y522S/WT mice. In (a), n = number of EDL fibers analyzed; in (b–e), n = number of mice; in (f and g), n = number of EDL muscles.

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