2016•CirculationRequires access
Abstract 15474: Differential Proteoglycan Expression Contributes to the Pathogenesis of Heritable Aortic Aneurysm Formation
Morvarid Emami, Jasha W. Trompf, E. Robertson, Stefanie S. Portelli, Yaxin Lu, Sanaz Maleki, Craig Steven McLachlan, Murat Kekic, Shisan Bao, Brett David Hambly, Richmond Jeremy
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Abstract
Excessive proteoglycan (PG) accumulation is a distinct feature of heritable thoracic aortic aneurysms (TAA), damaging the elastic lamellar units. PGs have both mechanical and signaling roles. Versican, perlecan and decorin can be upregulated by TGFβ signaling in various disease states. Increased TGFβ signaling is implicated in the pathogenesis of Bicuspid Aortic Valve (BAV) and Marfan Syndrome (MFS) associated thoracic aortic aneurysms (TAA). Perlecan organizes the endothelial BM and elastic lamellae and inhibits VSMC proliferation. Versican forms a loose matrix and its degradation products can alter VSMC proliferation. Biglycan and decorin have overlapping roles in fibrillogenesis, collagen assembly and TGFβ sequestration. We hypothesized that in both BAV-TAA and MFS-TAA PG expression will be altered and may exacerbate TAA pathogenesis. Computational immunohistochemistry was used to quantify PG expression in aortic tissue from human BAV-TAA (n=8), human MFS-TAA (n=8) and controls (n=5). Perlecan expression was increased (p