Fig. 1 Fbln4E57K/E57K mice develop arterial elongation, vascular tortuosity, ascending aortic aneurysms, elastic fiber fragmentation, and SMC disarray.

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Date of download: 6/3/2016 Copyright © 2016 American Medical Association. All rights reserved. From: Incidence of Aortic Complications in Patients With.

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Fig. 1 Fbln4E57K/E57K mice develop arterial elongation, vascular tortuosity, ascending aortic aneurysms, elastic fiber fragmentation, and SMC disarray. Fbln4E57K/E57K mice develop arterial elongation, vascular tortuosity, ascending aortic aneurysms, elastic fiber fragmentation, and SMC disarray. (A) Gross morphology of the thoracic aorta and its branches in adult Fbln4E57K/E57K mice and control (Fbln4+/+ and Fbln4+/E57K) littermates injected with yellow latex to visualize the vasculature. Arrows indicate aortic root dilatation and ascending aortic aneurysm seen in approximately half of Fbln4E57K/E57K mice; arrowheads indicate the different angles at which arterial branches come off the aortic arch. Arterial tortuosity and elongation are noted in all Fbln4E57K/E57K mice. (B to D) VVG (Verhoeff–van Gieson) stain (B) and transmission electron micrographs of the ascending aorta of 3-month-old Fbln4E57K/E57K mice and littermate controls at two different magnifications (C versus D). In addition to severe elastic fiber fragmentation, there is increased medial wall thickness and SMC disarray in the aorta of Fbln4E57K/E57K mice. At higher magnification (D), the moth-eaten or spongy appearance of the fragmented elastic fibers is appreciated in the aorta of Fbln4E57K/E57K mice. In addition, there is loss of smooth muscle contact with elastic fibers in Fbln4E57K/E57K vessels. Scale bars, 8 μm (C) and 4 μm (D). Asterisks indicate vessel lumen. Carmen M. Halabi et al. Sci Adv 2017;3:e1602532 Published by AAAS