Figure 4.
Accumulation of lysosomal glycogen is abated in skeletal muscle of Pompe mice treated with GS-PPMO. Glycogen levels in the (a) quadriceps, (b) diaphragm, (c) heart, and (d) liver of Pompe and wild-type mice were measured using the amplex red assay described in the Materials and Methods. Data represent mean ± SEM, n = 9–10 mice per group as indicated in the Materials and Methods. Dashed line represents mean glycogen level of wild-type mice. P < 0.05, (*) compared to WT, (^) compared to vehicle, (#) compared to rhGAA, ($) compared to GS-PPMO at 15 mg/kg.