Figure 2.

FUS and SmB are mislocalized to the cytoplasm in ALS patient fibroblasts bearing FUS mutations in the NLS. (A and B) IF with antibodies against FUS, SmB, U1-70K, U1A, and U1C was carried out with ALS patient cells carrying mutations in FUS R514G (A) or R521C (B). Control fibroblasts from an unaffected individual (#34) were used as a control (and see Supplementary Figure S2 for five additional control fibroblasts). Scale bar: 50 μm. (C and D) The indicated patient fibroblasts were co-stained with FUS and SmB antibodies. The merged images are shown. Scale bar: 50 μm.