Support cells worsened disease features in lab-grown human vessels

In lab-grown human blood vessel models, researchers tested how three cell types contributed to vascular disease in Hutchinson-Gilford progeria syndrome, an accelerated-aging disease. They built three-layer vessels using smooth muscle cells, which help vessels contract; endothelial cells, which line vessels; and fibroblasts, which make supporting tissue. They modelled atherosclerosis, the build-up of fatty deposits in arteries.

Fibroblasts carrying the progeria-associated mutation increased several disease features. Correcting the mutation with base editing, a technique for changing individual DNA letters, returned many disease characteristics to healthy levels. Experiments combining cells with and without the mutation linked fibroblasts to disrupted regulation of the material surrounding cells and signals promoting scar-like tissue. Smooth muscle cells contributed to accumulation of collagen, a protein that supports tissue. Vessel-lining cells contributed to inflammation.