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 4. Nuclear abnormalities are restored in iPSC-derived fibroblasts containing mutations in lamin A/C. (A) Immunofluorescence photographs show expression of fibronectin and vimentin, and distribution of actin in stress fibers, in iPSC-derived fibroblasts from the 3 diseases. Abnormalities of the nuclear membrane are shown on the right panels. Scale bars indicate 10 μm (lamin A/C) and 100 μm (rest). (B) Western blot for lamin A/C in iPSC clones from the 3 diseases and the derived secondary fibroblasts. (C) Quantification of cells displaying abnormal nuclear membrane by immunofluorescence microscopy. The mean of 3 independent experiments +/− SD is shown. (D) Electron microscopy photographs show abnormalities of the nuclear membrane in more detail for all types of secondary fibroblasts. Arrows point to more obvious defects. Scale bars indicate 1 μm.