Figure 7.

Combination treatment reduces ribonuclear foci-positive nuclei in the HSA^LR DM1 mouse model. (A) Quantification of the percent of nuclei with ribonuclear foci in quadriceps muscle of HSA^LR mice determined via in situ hybridization of CUG-repeat RNA. A reduction in the percent of nuclei with ribonuclear foci was observed with treatments of 600 mg kg^–1 erythromycin (EM, green) or 10 mg kg^–1 pafuramidine (PaF, blue) or a combination of both (EM+PaF, magenta) per oral administration daily for 14 days. Mean percent of nuclei with ribonuclear foci ± standard deviation values are displayed below each graph. (B) Fluorescent in situ hybridization microscopy in quadriceps muscle of untreated (HSA^LR), erythromycin (HSA^LR + EM), pafuramidine (HSA^LR + PaF), and combination-treated (HSALR + EM + PaF) HSA^LR mice against CUG RNA using a Cy3-(CAG)₈ probe (red). DAPI shown in blue. Larger box inset in lower right hand corner is magnification of smaller boxed region to clearly show foci.