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

Figure 1

Structural cores and blood markers of muscle damage (CK and LDH) at 4 months of age. (a–c) Representative histological images of normal fibers (a), fibers with unstructured cores (b), and fibers with contracture cores (c). (d) Percentage of EDL fibers presenting the features classified in (a–c) (white: normal fibers; grey: fibers with unstructured cores; and dark grey: fibers with contracture cores). See also Table S1. (e and f) Serum levels of creatine kinase (CK) and lactate dehydrogenase (LDH). In (e) and (f), data are given as mean ± SEM; , WT versus RYR1^Y522S/WT mice; ^#, untreated RYR1^Y522S/WT versus NAC-treated RYR1^Y522S/WT mice. In (d), n = number of fibers analyzed; in (e-f), n = number of mice. Scale bar: 10 μm.

(a)

(b)

(c)

(d)

(e)

(f)