Fig. 5.
Immunofluorescent localization of SMN in SMA fibroblasts treated with valproic acid (VPA) or hydroxyurea (HU). SMA patient fibroblasts (3813) were treated for 3 days with either 5 mM VPA (C), 65.7 µM HU (D) or vehicle (ddH2O; A). Fibroblasts were then labeled for SMN protein using 4F11. As a control, SMA carrier fibroblasts (3814; B) were also stained for SMN localization with 4F11. VPA and HU increase the amount of SMN in the nucleus and cytosol of patient fibroblasts. Arrowheads show SMN-positive gems in the nuclei. Scale bar, 10 µm.