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Fig. 4 | Acta Neuropathologica Communications

Fig. 4

From: Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease

Fig. 4

Unchanged satellite cell proliferation in Pompe disease. a Example of a proliferating (arrow) and a quiescent (arrowhead) satellite cell in a Pompe muscle biopsy. Cryosections were co-stained for Pax7 (red) to mark satellite cells and Ki67 (green) using immunofluorescence to assess active proliferation. Nuclei were stained with Hoechst (blue). White arrow indicates an activated satellite cell. The yellow arrowhead indicates a quiescent satellite cell. b Quantification of Ki67-positive satellite cells in Pompe patients (red symbols; different symbols per patient group), controls (green symbols), and a DMD patient (blue symbol). Pompe patients: group 1 n = 3, group 2 n = 3, group 3 n = 4, group 4 n = 3. DMD patient: n = 1. Controls: infants n =1, juveniles n = 2 and adults n = 5. There was no statistical difference between groups. The power to detect differences in Ki67-positive satellite cells was 0.8 (sample size 3, alpha 5 %, 2-sided equality, SD 5 %, delta 24). Delta of 24 is based on a previous report describing an increase of proliferating/activated satellite cells from ~0.5 % in healthy muscle to about 12 % after light resistance training [48]

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