Fig. 2
Satellite cell numbers remain unchanged and are independent of Pompe disease severity. a Identification of satellite cells (white arrows) by immunofluorescent analysis of Pax7 in human skeletal muscle biopsies. Upper left: Pax7; upper right: Hoechst; lower left: Laminin; lower right: merge. b, c Quantification of Pax7-positive cells in all Pompe biopsies examined in this study. Healthy control biopsies are included, as well as biopsies from two DMD patients. b Number of satellite cells (SCs)/mm2. c Percentage of SCs/nuclei. d Representative examples of each Pompe patient group, age-matched controls, and a DMD patient. Pompe patients: group 1 n = 3, group 2 n = 4, group 3 n = 4, group 4 n = 3. DMD patients: n = 2. Controls: infants n = 2, juveniles n = 2 and adult n = 5. For both Fig. 2b and c: Group 1 Pompe patients were statistically significant from other Pompe patient groups (p < 0.0001). Control infants were statistically significant from other controls (p < 0.0001). Other comparisons were not significantly different