Exploration of treatment of hereditary vascular dementia CADASIL using patient-derived iPS cells and its model mice

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Skin fibroblasts were isolated from three CADASIL patients with different mutations in Notch 3 gene. Three lines of iPS cells have been established by using the fibroblasts derived from one patient with CADASIL and endothelial cells and vascular smooth muscle cells were induced from the iPS cells. Their phenotypes were compared with those derived from normal controls. Furthermore, Notch 3 transgenic mice were obtained to investigate the pathogenesis of CADASIL in vivo and to compare the in vitro and in vivo phenotypes.