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Figure 2


Fig. 2. Differential distribution of YFP-nesprin-3 in DYT1 and control human fibroblasts. Primary skin fibroblasts from controls and DYT1 subjects were infected with a lentivirus vector expressing YFP-nesprin-3 and fixed 72 hours later. (A,B) Cells were immunostained for torsinA and GFP, with DAPI staining of nuclei. In control cells, YFP-nesprin is primarily localized in the perinuclear region (A), whereas in DYT1 cells it accumulates in globular structures presumably within the ER/NE (B); this was seen in two different cell lines of each type. (C-E) Immunocytochemical staining of infected control cells showing a typical distribution of torsinA (ER/NE) and vimentin (cytoplasm), with YFP-nesprin-3 primarily localized around the nucleus. (F-H) Staining of infected cells from DYT1 patients showing increased concentration of both torsinA and vimentin in the region of YFP-nesprin-3 globular structures. White arrowheads indicate nuclei. Scale bars: 20 µm in A,B; 10 µm in C-H.





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