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. Author manuscript; available in PMC: 2020 Oct 1.
Published in final edited form as: Cell Metab. 2019 Jul 25;30(4):689–705.e6. doi: 10.1016/j.cmet.2019.07.002

Fig. 6.

Fig. 6.

VAL-0417 reduces LB load in vivo after intramuscular (IM) or intravenous (IV) injection. (A) Biodistribution of VAL-0417 levels determined by sandwich ELISA after IM injection of WT mice with PBS or 0.6 mg VAL-0417. (B) Quantification of polysaccharide in the injected gastrocnemii of WT and Epm2a−/− mice after 3 IM injections of PBS or VAL-0417 administered over the course of one week. (C) Quantification of polysaccharide in the heart of WT and Epm2a−/− mice after 4 IV injections of PBS or VAL-0417 administered over a two-week period. Polysaccharides in (B) and (C) were isolated via the Pflüger method and quantified by glucose measurement assays following hydrolysis. Statistical significance is indicated as determined by ANOVA: ** p ≤ 0.01. (D, E) PAS-stained heart tissue of WT (D) and Epm2a−/− (E) mice after IV treatment regimen. Intensely staining PAS-positive deposits are LBs (purple). Tissues were counterstained with hemotoxylin (blue).