Article
Modeling and rescue of the vascular phenotype of Williams-Beuren syndrome in patient induced pluripotent stem cells.
Stem cells translational medicine - 1 Jan 2013
Kinnear Caroline, Chang Wing Y, Khattak Shahryar, Hinek Aleksander, Thompson Tadeo, de Carvalho Rodrigues Deivid, Kennedy Karen, Mahmut Naila, Pasceri Peter, Stanford William L, Ellis James, Mital Seema
Abstract excerpt
Elastin haploinsufficiency in Williams-Beuren syndrome (WBS) leads to increased vascular smooth muscle cell (SMC) proliferation and stenoses. Our objective was to generate a human induced pluripotent stem (hiPS) cell model for in vitro assessment of the WBS phenotype and to test the ability of candidate agents to rescue the phenotype. hiPS cells were reprogrammed from skin fibroblasts of a WBS patient with aortic...
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