Monoclonal Antibodies Specific for Disease-Associated Point-Mutants: Lamin A/C R453W and R482W
Figure 6
EDMD patient cells contain an increased number of transnuclear tubules.
(A) Immunofluorescence of wild-type (top panel), EDMD (middle panel), and FPLD (bottom panel) primary human fibroblasts. Cells were co-stained with anti Lamin A/C N-18 and anti Lamin A/C 4C11, anti R453W, or anti R482W, respectively. Mid plane sections, xz-projections (above), and yz-projections (right) of the nuclei are shown. Arrowheads indicate transnuclear tubules through which the respective projection planes cut. Bar scale, 10 µm. (B) Number of transnuclear tubules per cell for wild-type, EDMD, and FPLD fibroblasts. n = 44 (wild-type), n = 46 (EDMD), n = 50 (FPLD). ** p<0.02; * p = 0.13. (C) Merged image of a mid plane section, xz-projection (above), and yz-projection (right) of an EDMD nucleus. Bar scale, 10 µm.