Modeling Thoracic Aortic Dissection Using Patient-Specific iPSCs Reveals VSMC Dysfunction and Extracellular Matrix Dysregulation.

ABSTRACT: Thoracic aortic dissection (TAD) is a life-threatening condition characterized by medial degeneration and vascular smooth muscle cell (VSMC) dysfunction, with no effective medical therapy currently available. The underlying pathological mechanisms of TAD remain incompletely understood. In this study, we used a nonintegrated episomal vector-based reprograming system to generate induced pluripotent stem cells (iPSCs) from TAD patients and healthy controls. Both TAD and normal iPSCs expressed key pluripotency markers and were capable of differentiating into the three germ layers in vitro. These iPSCs were differentiated into vascular smooth muscle cells (VSMCs) through a mesodermal intermediate for disease modeling. VSMCs derived from both TAD and normal iPSCs expressed smooth muscle α

SUBMITTER: Jin P 

PROVIDER: S-EPMC12767432 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature