iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome

• DOI: 10.1016/j.stemcr.2020.01.005
• Publication Date: 2020-02-06T15:30:04Z
• Authors: Leigh Atchison; Nadia O. Abutaleb; Elizabeth Snyder-...; Yantenew Gete; Alim Ladha; Thomas Ribar; Kan Cao; George A. Truskey
• Keywords: Progeria; Affect

Hutchinson-Gilford progeria syndrome (HGPS) is a rare disorder caused by point mutation in the Lamin A gene that produces protein progerin. Progerin toxicity leads to accelerated aging and death from cardiovascular disease. To elucidate effects of progerin on endothelial cells, we prepared tissue-engineered blood vessels (viTEBVs) using induced pluripotent stem cell-derived smooth muscle cells (viSMCs) (viECs) HGPS patients. viECs aligned with flow but exhibited reduced flow-responsive expression altered NOS3 levels. Relative viTEBVs healthy showed function markers disease associated endothelium. reduction both vasoconstriction vasodilation. Preparing viSMCs only Furthermore, produced VCAM1 E-selectin TEBVs or viSMCs. In summary, viTEBV model has identified role endothelium HGPS.