Figure 3.

Bioactivity evaluated by quantification of biochemical testing

Samples were obtained 2 weeks following an infusion of ERT, when GAA activity from ERT was at a trough. (A) Serum GAA activity. (B) Muscle GAA activity. (C) Muscle glycogen content. (D) Urine Glc4. ∗ = p < 0.05; ns = not significant as determined by a paired t test for the comparison indicated by connecting lines. # indicates when ERT was discontinued at week 26. The dotted line box indicates the normal range, although no normal range has been established for the experimental endpoint of serum GAA.