Glycogen deposition in WT and Gaa^−/− mice that received a transplant of LV-SF-GFP and LV-SF-GAA. (A) PAS staining (dark purple stain) shows glycogen deposition in heart, QF, spleen, liver, and brain (cortex) of 8-month-old mice that received a transplant of LV-SF-GFP. Clearance of glycogen is observed in liver and spleen of mice that received a transplant of LV-SF-GAA. Reduction of glycogen storage is observed in heart and skeletal muscle. Brain does not respond. Heart and QF, original magnification ×1000; liver, spleen, and brain original magnification ×125. (B) Quantification of the PAS staining by ImageJ shows a reduction of glycogen storage in heart, diaphragm, liver, and spleen but only marginal reduction in QF at 8 months after transplantation. PAS stained area as the percentage of total area is presented for LV-SF-GFP and LV-SF-GAA groups (n = 4-6); *P < .05 and **P < .01 indicate a significant difference between mice that received a transplant of LV-SF-GAA and LV-SF-GFP. (C) Enzymatic quantification of the glycogen deposition in tissues at 8 months after transplantation. The figure shows a reduction of glycogen in all tissues tested except brain (n = 4-11); *P < .05 and **P < .01 indicate a significant difference between mice that received a transplant of LV-SF-GAA and LV-SF-GFP.