Figure 4.

Fibroblasts from an individual with E203K lamin A mutation exhibit decreased lamin A sumoylation, altered lamin A localization/nuclear morphology, and increased cell death. (A) Extracts prepared from skin fibroblasts of a normal individual and individual heterozygous for the E203K lamin A mutation were subjected to immunoprecipitation using anti–lamin A antibodies followed by Western blot assay using anti–SUMO-2 antibodies or anti–lamin A antibodies (different from those used for immunoprecipitation). (B) Skin fibroblasts of a normal individual and the individual heterozygous for the E203K lamin A mutation were subjected to immunofluorescence analysis using anti–lamin A antibodies. DNA was visualized by staining with HOECHST 33342. Bar, 5 μm. (C) Skin fibroblasts of a normal individual and E203K lamin A individual were analyzed by trypan blue assay to measure cell death. Data are shown as means ± SEM (P < 0.0038 for E203K lamin A mutant cells vs. wild-type cells) and are from three datasets.