Human trilayer engineered blood vessel reveals influence of fibroblasts on disease progression in model of Hutchinson-Gilford progeria.

Fibroblasts significantly worsen vascular pathology in a human trilayer blood vessel model of Hutchinson-Gilford progeria, with mutation correction reversing many disease features.

• Why it matters: Understanding the specific role of fibroblasts in vascular disease progression is crucial, as their influence in conditions like HGPS remains poorly defined, hindering targeted therapies.
• What they did: Researchers engineered trilayer tissue-engineered blood vessels with smooth muscle cells, endothelial cells, and fibroblasts, modeling atherosclerosis in HGPS and testing mutation correction effects.
• The result: Fibroblasts contributed to extracellular matrix dysregulation and fibrosis, while mutation correction improved disease markers, enabling insights into cell-specific roles and potential therapeutic strategies.