Figure 1From: Glycogenosome accumulation in the arrector pili muscle in Pompe diseaseQuantification of cytoplasmic glycogen content in smooth muscle cells of arrector pili muscle. The smooth muscle cells were assigned to five categories: 1 - No visible glycogen content; 2 – Scattered glycogen granules; 3 - Large amount of granular glycogen; 4 – Glycogenosomes; 5 – Extensive autophagy, filling a significant part of the cytoplasm. A: A large fraction of smooth muscle cells from Pompe patients shows abnormal forms of glycogen accumulation, while the cells in controls contain mainly no or granular glycogen, which is considered as a normal property for this cell type. (Chi square test: p < 0,0001); B: The number of cells containing glycogenosomes or presenting extensive autophagy is decreased after ERT in the two patients where a follow up biopsy was available. (Significance could not be determined due to the low subject number).Back to article page