Fig. 5
From: Long-term neurologic and cardiac correction by intrathecal gene therapy in Pompe disease
Strength is partially improved in the absence of muscular pathology correction. Treatment groups were as described in Fig. 1. a Wire-hang strength measures the ability of the mice to hold their own weight when suspended to a wire screen. Mean latency to fall is multiplied by the weight of the mice (Linear mixed effects, time effect: P < 0.0001; n = 8 to 13 per group). b Four-legs grip strength measurement using a grip meter and a force transducer (one-way ANOVA with Newman-Keuls post hoc test; n = 8 to 13 per group: *P < 0.05, ***P < 0.001) (c) Correlation between the accelerating rotarod latency and the grip strength. d Representative cross sections of extensor digitorum longus muscle (EDL), paraffin embedding, PAS stain. The glycogen storage appears purple. Arrows point to vacuoles in the cytoplasm of the fibers and arrowheads point to central nuclei. e In situ contraction study, maximal twitch tension developed by the EDL after 6 V stimulation (one-way ANOVA with Newman-Keuls post hoc test; n = 8 to 12 per group: *P < 0.05, **P < 0.01). f Glycogen concentration measurement in muscle tissue extracts obtained from samples that were snap-frozen in liquid nitrogen rapidly after sacrifice (one-way ANOVA with Newman-Keuls post hoc test; n = 4 to 5 per group: **P < 0.01, ***P < 0.001) (g) GAA enzymatic assay in the same extracts using 4-methylumbelliferyl-α-D-glucopyranoside as substrate (one-way ANOVA with Newman-Keuls post hoc test; n = 4 to 5 per group: ***P < 0.001)