Fig. 1

Characterization of lysosomal and muscle wasting pathology during disease progression in Gaa^−/− mice. a. Glycogen accumulation. Glycogen levels were measured biochemically in TA muscles at the indicated ages. b. Lysosomal pathology. Immunofluorescent analysis of TA sections using a Lamp1 antibody (in green). Representative images are shown. The basal lamina was stained using a Laminin antibody (in red). Nuclei were stained with Hoechst (in blue). Black and white images of Lamp1 staining are included for better visualization. c. Quantification of the number of Lamp1-positive spots per fiber from B. Data are from two TA muscles derived from two different animals per genotype per timepoint, and are expressed as mean ± SD. ***p < 0.001. d. Wet weight of TA muscles. Each dot represents TA wet weight from one muscle of one animal. Means ± SD are indicated as lines (n = 4–12 animals per genotype per timepoint). *p < 0.05 and ***p < 0.001. e. HE staining of TA sections. Representative images are shown. f. Quantification of fiber size from E. Data from individual mice are plotted (n = 2–4 animals per genotype per timepoint). Means ± SD are indicated. *p < 0.05 and **p < 0.01