TFEB Overexpression Reduces Storage and Tissue Pathology in a Mouse Model of MSD
(A) Alcian blue staining of GAG content in skeletal muscle and liver from mice injected systemically with either an AAV2/9-CMV-GFP or with an AAV2/9-CMV-TFEB3xflag viral vector.
(B) Quantitative analysis of GAG content in skeletal muscle and liver of MSD mice injected with either an AAV2/9-CMV-GFP or with an AAV2/9-CMV-TFEB3xflag viral vector. GAG content was displayed as mg of GAGs/mg of tissue extract. At least four mice per group were analyzed for each tissue examined (∗p < 0.05). Data are mean ± SEM.
(C) TFEB reduces inflammation in the liver of MSD mice. Macrophages and macrophage-related inflammatory cells were detected in liver sections from mice injected systemically with either an AAV2/9-CMV-GFP or with an AAV2/9-CMV-TFEB3xflag viral vector by immunofluorescence analysis using an antibody against CD68.
(D) Reduction of TUNEL-positive cells (arrows) in 4-month-old MSD mice injected with an AAV2/9-CMV-TFEB3xflag viral vector compared with age-matched MSD noninjected mice. At least four mice per group were analyzed for each tissue examined). Data are mean ± SEM; ∗p < 0.05 (B and D). Scale bars represent 100 μm (A, C, and D).