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  4. Differentiation defect in neural crest-derived smooth muscle cells in patients with aortopathy associated with bicuspid aortic valves.

Differentiation defect in neural crest-derived smooth muscle cells in patients with aortopathy associated with bicuspid aortic valves.

Jiao J, Xiong W, Wang L, Yang J, Qiu P, Hirai H, Shao L, Milewicz D, Chen Y, Yang B (2016) Differentiation defect in neural crest-derived smooth muscle cells in patients with aortopathy associated with bicuspid aortic valves. EBioMedicine 10:282-290. doi: 10.1016/j.ebiom.2016.06.045

Summary: Individuals with bicuspid aortic valves (BAV) are at a higher risk of developing thoracic aortic aneurysms (TAA) than patients with trileaflet aortic valves (TAV). Aneurysms associated with BAV most commonly involve the ascending aorta. Smooth muscle cells (SMCs) in the ascending and descending aorta arise from neural crest (NC) and paraxial mesoderm (PM), respectively. Scientists hypothesized defective differentiation of the neural crest stem cells (NCSCs)-derived SMCs but not paraxial mesoderm cells (PMCs)- derived SMCs contributes to the aortopathy associated with BAV. Induced pluripotent stem cells (iPSCs) from BAV/TAA patients were differentiated into NCSC-derived SMCs and showed decreased expression of a marker of SMC differentiation (MYH11) and impaired contraction. The scientists demonstrated that decreased differentiation and contraction of patient’s NCSC-derived SMCs may contribute to the aortopathy associated with BAV.

Usage: Anti-NGFr (ME20.4, p75, Cat. #AB-N07) was used for the immunofluorescence staining and flow cytometry of NCSCs.

Related Products: NGFr (ME20.4, p75) Mouse Monoclonal (Cat. #AB-N07)

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