Fig. 5.

Lamin A/C protein abundance (A) and nuclear organization in human dermal fibroblasts from control subjects and OI patients with mutations in CRTAP, P3H1, and PPIB genes (B). A: Representative 2-D western blot images are shown. Histograms visualize the total spot Vol of each isoelectric series: lamin A in dark gray and lamin C in light gray. B: Representative confocal immunofluorescence images are shown. Lamin A/C signal is shown in red. Counterstaining of the nuclei was performed using DAPI (blue). An altered lamin distribution was detected in all pathological primary cells. Scale bar 10 μm.