Impact of dystrophin deficiency on vascular smooth muscle cell.

ABSTRACT: Duchenne Muscular Dystrophy (DMD) is a severe genetic disorder affecting skeletal and cardiac muscles, primarily in males. While much research has focused on these systems, the role of vascular smooth muscle cells (VSMCs) remains underexplored. This study examines how dystrophin deficiency alters VSMCs plasticity using mdx mice and DMD patient-derived iPSC VSMCs. Immunohistochemistry, Western blot, electron microscopy, and transcriptomic analyses revealed significant abnormalities. In mdx mice, abnormal vascular structure and vascular degeneration were observed. DMD VSMCs showed impaired maturation, reduced contractile protein expression, and disrupted mitochondrial dynamics, including excessive fission and reduced mitochondrial area. These cells also exhibited increased apoptosis under ox

SUBMITTER: Xuan W 

PROVIDER: S-EPMC12748682 | biostudies-literature | 2025 Nov

REPOSITORIES: biostudies-literature