Fig. 1

Skeletal muscle pathology of Pompe patients. Patients were divided in four groups as indicated based on disease onset and severity. a Representative examples of each group with HE (GMA), PAS (GMA), and acid phosphatase (frozen section) stainings of biopsies from the QF. b Biopsies were scored for vacuole density, % damaged fibers, PAS intensity, and % loss of cross striation for each patient group. Data are means +/− SD of three patients per group. c Scores of (b) were combined in an overall score for muscle damage. Group 1 n = 3, group 2 n = 2, group 3 n = 4, group 4 n = 5. Data are means +/− SD *: p < 0.05; **: p < 0.01. AU: arbitrary units