Matthew W Ellis, Muhammad Riaz, Yan Huang, Christopher W Anderson, Marie Hoareau, Xin Li, Hangqi Luo, Seoyeon Lee, Jinkyu Park, Jiesi Luo, Luke D Batty, Qunhua Huang, Colleen A Lopez, Dieter P Reinhardt, George Tellides, Yibing Qyang
BACKGROUND: A series of incurable cardiovascular disorders arise due to improper formation of elastin during development. Supravalvular aortic stenosis (SVAS), resulting from a haploinsufficiency of ELN , is caused by improper stress sensing by medial vascular smooth muscle cells, leading to progressive luminal occlusion and heart failure. SVAS remains incurable, as current therapies do not address the root issue of defective elastin. METHODS: We use SVAS here as a model of vascular proliferative disease using both human induced pluripotent stem cell-derived vascular smooth muscle cells and developmental Eln ± mouse models to establish de novo elastin assembly as a new therapeutic intervention...
May 16, 2024: Arteriosclerosis, Thrombosis, and Vascular Biology