Fig. 1. (A) Lamin A and the localization of mutations. The N-terminus is on the left. Domains of lamin A are represented by: white, globular head and tail regions and linker regions in the rod-domain; stripes, 
-helical rod-domain regions 1a, 1b and 2; black, the most highly conserved regions of the rod-domain. Underlined mutations are found in dilated cardiomyopathy, mutations in italics in FPLD and others in AD-EDMD. Mutations shown in red are proteins that form aberrant intranuclear foci when expressed in C2C12 cells (Fig. 1B; Table 1). (B) Cellular localization of FLAG-tagged wild-type and mutant forms of lamin A. The panels show laser scanning confocal immunofluorescence microscopy images of C2C12 cells transfected with wild-type prelamin A (WT) or prelamin A with missense mutations as indicated. Arrows show cells with nuclear foci, insets show nucleoplasmic staining in cells expressing mutant protein but lacking foci. Antibodies used were mouse monoclonal antibodies against FLAG recognized by FITC-conjugated secondary antibodies and rabbit polyclonal antibodies against lamin B1 recognized by AlexaTM-conjugated secondary antibodies. The pictures show an overlay of the FITC (green) and AlexaTM (red) channels where areas of colocalization appear yellow. Bar, 10 µm.
