Fig. 2. Abnormal localization of INM proteins and nucleoporins in the NE of fibroblasts from FPLD patients. Fibroblasts from control individuals (top) and from FPLD patients (bottom) were labeled for double immunofluorescence as indicated, then observed with a confocal microscope. The patterns of nuclear defects were similar in the three patients. Note the systematic colocalization of lamin A/C and emerin in nuclei from controls and patients and, in B-type lamin-depleted buds (arrowheads), the presence of emerin and the partial or total absence of LAP2ß and Nup153. Bars, 5 µm.
