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. 2021 May 14;108(6):1095–1114. doi: 10.1016/j.ajhg.2021.04.016

Figure 3.

Figure 3

Ultrastructural analysis of the ECM in dermal biopsies

(A–D) Ultrastructural analysis of the elastic fibers in a skin biopsy of affected individual F1:IV-2 (C and D) and a control subject (A and B).

(E–H) Ultrastructural analysis of collagen fibrils in a skin biopsy of affected individual F1:IV-2 (G and H) and a control subject (E and F). Scale bar represents 2 μm (A, C, E, and G) and scale bar represents 500 nm (B, D, F, and H). The elastin core (dotted while line) is surrounded by a spare mantle of microfibrils in the control subject (red dotted line, white arrow). Note that microfibrils infiltrate into the periphery of the elastic fiber in a skin biopsy of affected individual F1:IV-2 (red dotted line, black triangle). Col, collagen; Ef, elastic fiber; Mf, microfibrils.