Jiao J, Tian W, Qiu P, Norton EL, Wang MM, Chen YE, Yang B (2018) Induced pluripotent stem cells with NOTCH1 gene mutation show impaired differentiation into smooth muscle and endothelial cells: Implications for bicuspid aortic valve-related aortopathy. J Thorac Cardiovasc Surg 156:515-522. doi: 10.1016/j.jtcvs.2018.02.087 PMID: 29653750
Objective: To develop an in vitro model with human-induced pluripotent stem cells (iPSCs) to evaluate the role of NOTCH1 in smooth muscle and endothelial cell differentiation.
Summary: NOTCH1 is critical in SMC and EC differentiation of iPSCs through neural crest stem cells and cardiovascular progenitor cells, respectively. NOTCH1gene mutations may potentially contribute to the development of thoracic aortic aneurysms by affecting SMC differentiation in some patients with bicuspid aortic valve-related aortopathy.
Usage: Immunofluorescence staining and flow cytometry was performed.
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