Fig. 2.

Organization of nuclear pores and membranes in HGPS cells. The distribution and organization of nuclear pore complexes in a passage 6 HGPS cell (HGADFN003; a–c) was very similar to control AG09602B cells (not shown), as determined by double-label indirect immunofluorescence with LA and mAb 414 Abs. The typical punctate nuclear pore staining pattern along the nuclear envelope is evident. In the misshapen nuclei typifying passage 24 (d and e), the nuclear pores were frequently aggregated into large masses as detected with NUP153 Ab. Comparison of enlarged views of small regions (c and f) showing that the pores were not uniformly distributed in the nuclei of passage 26 HGPS cells (f); bracket in f shows an area that was essentially free of nucleoporins as detected by mAb414. In HGADFN003 cells stained with emerin Ab, emerin remained associated with the nuclear envelope throughout all passages (g and h). Emerin distribution appeared normal in control AG09602 cells at all passages (i). [Scale bars = 5 μm(b, e, and g–i) and 1 μm(c and f).]