Figure 9.

Adenoviral-mediated expression of GFP-ClC-1-FLAG significantly reverses myotonia in TA muscle of HSALR mice. (A) GFP fluorescence of cross sections of HSALR TA muscle showing distribution of cytosolic GFP (left) and GFP-ClC-1-FLAG (right) 3 wk after injection. Note that the images represent regions of transduction at the site of injection and are not representative of the total muscle body. (B) Myotonia score (see Materials and methods) was significantly reduced in the TA muscle of HSALR mice after adenoviral-mediated expression of GFP-ClC-1-FLAG compared with contralateral TA muscle expressing cytoplasmic GFP. Non-transduced gastrocnemius muscle from HSALR mice served as an untreated positive control. Data are presented as mean ± SEM (6 and 14 experiments for WT and HSALR mice, respectively). A one-way ANOVA with Tukey’s post-hoc test was used for group comparisons. *, P ≤ 0.01.

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