Figure 5.

AT-GAA Reversed the Level of Galectin 3, a Marker of Lysosomal Damage, in Muscle from KO Mice

Muscle biopsies were collected from age and sex-matched WT, untreated (KO), and AT-GAA-treated KO (KO-ERT) mice. (A) Western blot of muscle lysates from WT, untreated KO (KO), and treated KO (KO-ERT) mice with the indicated antibodies (n = 4 for each group). Only galectin 3 was increased in muscle from untreated KO mice; the level of galectin 3 was reduced on therapy and reached the WT control value. Statistical significance was determined by one-way ANOVA. Graphs represent mean ± SD. ∗∗∗p < 0.001; ∗∗∗∗p < 0.0001. (B) Western blot of lysates from the diaphragm (top) and heart (bottom) of WT and untreated KO (KO) mice with anti-galectin 3 antibody. (C) Western blot of muscle lysates from untreated KO and muscle-specific autophagy-deficient KO mice (DKO) with anti-galectin 3 antibody. Efficient suppression of autophagy in skeletal muscle of DKO mice is indicated by the absence of LC3-II band. The blots are composite images; the samples were run on the same gel. Source data are available online for this figure. GAPDH was used as loading control. (D) Quantification of galectin 3 in serum from the WT and KO mice by ELISA. Student’s t test was used for statistical analysis. Data are mean ± SD. ∗p < 0.05 (n = 6).