Abstract

<p>Paraffin-embedded aortic tissue specimen of LDS patients (n = 3) were double stained with anti-Gremlin-1 (brown staining) and anti-CD34 (red staining; C) or anti-smooth muscle actin (red staining; B, D–F). Endothelial cells throughout the vessel wall showed expression for Gremlin-1 including ECs of the intima (B) and of vessels within the media (C) and the adventitia (D and in more detail in E). Gremlin-1 positive staining was also observed on smooth muscle cells of the media (B, C) as well as on vessel surrounding smooth muscle cells in the adventitial layer (D, E). In aortic tissue specimen of healthy controls (n = 3), a similar staining pattern without gross differences of staining intensity was observed as shown for a small vessel within the adventitia (F). In A, an isotype control instead of primary antibody was used revealing the specificity of the staining (EC = endothelial cell, SMC = smooth muscle cell; magnification A–D, F: 400×; E: 1000×; scale bar represents 20 µm in A–D and F and 8 µm in E).</p

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