Article
Patient-derived extracellular matrix demonstrates role of COL3A1 in blood vessel mechanics.
Acta biomaterialia - 1 Aug 2023
Doherty Elizabeth L, Aw Wen Yih, Warren Emily C, Hockenberry Max, Whitworth Chloe P, Krohn Grace, Howell Stefanie, Diekman Brian O, Legant Wesley R, Nia Hadi Tavakoli, Hickey Anthony J, Polacheck William J
Abstract excerpt
Vascular Ehlers-Danlos Syndrome (vEDS) is a rare autosomal dominant disease caused by mutations in the COL3A1 gene, which renders patients susceptible to aneurysm and arterial dissection and rupture. To determine the role of COL3A1 variants in the biochemical and biophysical properties of human arterial ECM, we developed a method for synthesizing ECM directly from vEDS donor fibroblasts. We found that the protein...
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