Research Paper Volume 3, Issue 4 pp 380—390

Generation of induced pluripotent stem cell lines from 3 distinct laminopathies bearing heterogeneous mutations in lamin A/C


Figure 5. Increased senescence and susceptibility to apoptosis in iPSC-derived fibroblasts containing lamin A/C mutations. (A) Phase contrast photographs after β-galactosidase staining of primary and secondary fibroblasts from the 3 diseases. Samples were measured in triplicate. Scale bars indicate 100 μm. (B-C) Quantification of β-galactosidase positive cells. Secondary fibroblasts from H1 ESCs were included (also in E). The mean of 3 independent experiments +/− SD is shown (also in F-G). * indicates p value <0.05. (D-E) Proliferation of the indicated cell types measured at different time points; a representative experiment is shown. (F) Electrical stimulation (E-Stim) significantly elevated the apoptotic rate in primary fibroblasts of the 3 patients compared with control fibroblasts as assessed by Annexin V apoptotic assay. (G) A similar trend of induced apoptosis was observed after electrical stimulation of the secondary fibroblasts from the 3 diseases.