Figure 5

Pcolce protein is sequestered within nuclear aggregates of the mouse OPMD model system. Immunofluorescence staining of TA muscle sections derived from A17.1 OPMD model mice. (A) PABPN1 aggregates were recognised by the high fluorescence intensity of nuclear PABPN1 and their co-localisation with ubiquitin (arrows). Scale bars are 20 μm. (B) Co-localisation of Pcolce with PABPN1 was conducted on TA muscle sections from 12, 18 and 26 week old A17.1 mice. PABPN1 is visualised with 3 F5-VHH and mouse anti-myc antibodies (green), Pcolce or ubiquitin are visualised with rabbit antibodies and are marked in red. Co-localisation between Pabpn1 and Pcolce is shown in yellow in the merged image. The nucleus is counterstained with DAPI (blue). Nuclear aggregates are indicated with arrows. Scale bars equal 10 μm. Immunostaining was performed on sections from 3 mice. Shown are representative images.