Ultrastructure of lung elastin and collagen in mouse models of spontaneous emphysema
Ultrastructure of lung elastin and collagen in mouse models of spontaneous emphysema
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DOI：
10.1016/s0945-053x(99)00031-1
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发表时间：
1999-08-01
期刊：
影响因子：
6.9
通讯作者：
Martorana, PA
中科院分区：
文献类型：
--
作者：
O'Donnell, MD;O'Connor, CM;Martorana, PA
C57Bl/6J 品系小鼠的紧皮 (Tsk) 和米色 (bg) 突变体会自发出现气腔扩大，让人想起人类肺气肿。为了确定这种增大是否伴随着基质破坏，就像在人类疾病中一样，我们检查了两种突变体肺部的弹性蛋白和胶原蛋白基质。在受控碱消化（保留胶原蛋白）和甲酸消化（使弹性蛋白可视化）之后，通过扫描电子显微镜分别观察这些基质成分的超微结构。在 Tsk 小鼠的肺部观察到明显的弹性蛋白破坏，表明弹性蛋白溶解过程。在 bg 小鼠的肺部观察到弹性蛋白片层增厚，表明先天性基质重塑可能是该品系中气腔扩大的基础。 (C) 1999 Elsevier Science B.V./国际基质生物学学会。版权所有。
The tight-skin (Tsk) and beige (bg) mutants of the C57Bl/6J strain of mouse spontaneously develop air-space enlargement reminiscent of human emphysema. To determine if this enlargement is accompanied by matrix destruction, as in the human disease, we examined the elastin and collagen matrices of the lungs of both mutants. The ultrastructure of these matrix components was separately visualized by scanning electron microscopy following controlled alkali digestion, which preserves collagen, and formic acid digestion, which enables visualization of elastin. Significant elastin destruction suggestive of an elastolytic process was observed in the lungs of Tsk mice. Thickening of elastin lamellae was observed in the lungs of bg mice, suggesting that congenital matrix remodeling may underlie air-space enlargement in this strain. (C) 1999 Elsevier Science B.V./International Society of Matrix Biology. All rights reserved.