Figure 10

Uneven distribution of the phosphorylated RNA pol II in myoblast nuclei from AD-EDMD patient 99-3. Myoblast culture cells from control (a)or patient (b-e") were immunolabelled with mab V/22 recognizing the phosphorylated RNA pol II (a"-c") or with 8WG16 recognizing the unphosphorylated RNA pol II (d"). The patients myoblasts were double labelled with a polyclonal anti-lamin A/C antibody (e) and V/22 (e') and then analyzed by confocal microscopy. The merged picture is shown in (e"). Note that the dramatic concentration of phosphorylated RNA pol II is observed at the poles of the nuclei in patient cells (b",c",e" arrows). Note the normal localization of the phosphorylated RNA pol II in control cells (compare a" with b",c" and e") and of the unphosphorylated RNA pol II (d"). The corresponding phase-contrast images (a-d) and Hoechst fluorescences (a'-d') are shown. Bars: 10 µm.