Figure 6.

GYS2-1 Treatment Prevents Fibrosis in the Agl^−/− GSD III Mouse Model

Agl^−/− mice were treated with PBS or GYS2-1 monthly for 12 doses starting at 1 month of age. (A) Liver samples were collected 72 hr following the final dose and analyzed for collagen deposition using Sirius Red staining. Lower panel represents higher magnification of images in upper panel. Representative images of individual animals from PBS- or GYS2-1-treated groups are shown. The scale bars represent 1 mm or 200 μm in the upper or lower panel, respectively. (B) Analysis of Ki67 staining to detect hepatocellular proliferation in Agl^−/− mice. Representative images of nodule and non-nodule areas of individual animals are shown. The scale bars represent 200 μm. (C) Quantitative analysis of immunohistochemistry of Ki67 and Sirius Red staining shown in (A) and (B), respectively, is shown. Detailed methodology of quantitative analysis can be found in Supplemental Information and Figures S4 and S8. (D) Analysis of serum samples was performed to measure circulating liver enzymes. Data are presented as mean ± SD.