Establishment and characterization of human induced pluripotent stem cell line (WMUi020-A) from a patient with bicuspid aortic valve aortopathy.

A human induced pluripotent stem cell (hiPSC) line (WMUi020-A) was generated from the aortic smooth muscle cells of a 56-year-old donor with bicuspid aortic valve and ascending aortic aneurysm. Episomal vector-mediated Non-integration iPSC reprogramming was used for this iPSC line generation. The established iPSC line highly expressed pluripotency markers with three germ-layer differentiation potential in vitro, as well as a normal karyotype. We further found that this iPSC line has a potential mutation of ROBO4 (c.161 T>C, p.Q54R), which may be useful for the disease modeling of bicuspid aortic valve aortopathy.