Modeling of lamin A/C mutation premature cardiac aging using patient-specific induced pluripotent stem cells

Chung-Wah Siu; Yee-Ki Lee; Jenny Chung-Yee Ho; Wing-Hon Lai; Yau-Chi Chan; Kwong-Man Ng; Lai-Yung Wong; Ka-Wing Au; Yee-Man Lau; Jinqiu Zhang; Kenneth Weijian Lay; Alan Colman; Hung-Fat Tse

doi:doi:10.18632/aging.100503

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Research Paper Volume 4, Issue 11 pp 803—822

Modeling of lamin A/C mutation premature cardiac aging using patient-specific induced pluripotent stem cells

Figure 3. Effect of electrical stimulation on LMNA^R225X/WT dermal fibroblasts. (A-D) Immunofluorescence showing typical nuclear morphologies of control and LMNA^R225X/WT dermal fibroblasts before and after electrical stimulation. (E) Effects of electrical stimulation on the percentage of control and LMNA^R225X/WT dermal fibroblasts with nuclear blebs. (F) Effects of electrical stimulation and U0126 (ERK1/2 pathway blocker) on the apoptosis of control and LMNA^R225X/WT dermal fibroblasts. The application of U0126, a highly selective blocker of the MEK1 pathway, nearly completely abolished the apoptotic effects of electrical stimulation on LMNA^R225X/WT dermal fibroblasts. (G-H) Electrical stimulation-activates mitogen-activated protein kinases (MAPK) pathway in iPSC-CM: (G) MEK1 and (H) extracellular signal-regulated protein kinases 1 and 2 (ERK1/2) in LMNA^R225X/WT dermal fibroblasts, but not in control fibroblasts. *p- value <0.05.