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视网膜病变、眼球增大(rge)雏鸡角膜的超微结构变化。

Ultrastructural changes in the retinopathy, globe enlarged (rge) chick cornea.

作者信息

Boote Craig, Hayes Sally, Young Robert D, Kamma-Lorger Christina S, Hocking Paul M, Elsheikh Ahmed, Inglehearn Chris F, Ali Manir, Meek Keith M

机构信息

Structural Biophysics Group, School of Optometry and Vision Sciences, Cardiff University, Maindy Road, Cardiff CF24 4LU, UK.

出版信息

J Struct Biol. 2009 May;166(2):195-204. doi: 10.1016/j.jsb.2009.01.009. Epub 2009 Mar 1.

Abstract

In the cornea, the precise organisation of fibrillar collagen and associated proteoglycans comprising the stromal extracellular matrix plays a major role in governing tissue form and function. Recently, abnormal collagen alignment was noted in the misshapen corneas of mature chickens affected by the retinopathy, globe enlarged (rge) mutation. Here we further characterize corneal ultrastructural changes as the rge eye develops post-hatch. Wide-angle X-ray scattering disclosed alteration to dominant collagen lamellae directions in the rge chick cornea, compared to age-matched controls. These changes accompanied eye globe enlargement and corneal flattening in affected birds, manifesting as a progressive loss of circumferential collagen alignment in the peripheral cornea and limbus in birds older than 1 month. Collagen intermolecular separation was unchanged in rge. However, small-angle X-ray scattering results suggest collagen fibril separation and diameter increase more rapidly towards the corneal periphery in rge at 3 months post-hatch compared to controls, although central collagen fibril diameter was unchanged. By transmission electron microscopy utilising cuprolinic blue stain, the morphology and distribution of stromal proteoglycans were unaltered in rge corneas otherwise demonstrating abnormal collagen fibril organisation. From a numerical simulation of tissue mechanics, progressive remodelling of stromal collagen in rge during globe enlargement post-hatch appears to be related to the corneal morphometric changes presented by the disease.

摘要

在角膜中,构成基质细胞外基质的纤维状胶原蛋白和相关蛋白聚糖的精确组织在决定组织形态和功能方面起着主要作用。最近,在患有视网膜病变、眼球增大(rge)突变的成熟鸡的畸形角膜中发现了胶原蛋白排列异常。在此,我们进一步描述rge眼孵化后发育过程中角膜超微结构的变化。广角X射线散射显示,与年龄匹配的对照相比,rge雏鸡角膜中主要胶原板层方向发生改变。这些变化伴随着患病鸟类眼球增大和角膜扁平,表现为1个月以上鸟类周边角膜和角膜缘周向胶原排列逐渐丧失。rge中胶原分子间间距未改变。然而,小角X射线散射结果表明,与对照相比,孵化后3个月时rge角膜周边胶原纤维间距增加和直径增加更快,尽管中央胶原纤维直径未改变。通过使用铜啉蓝染色的透射电子显微镜观察,rge角膜中基质蛋白聚糖的形态和分布未改变,否则表明胶原纤维组织异常。从组织力学的数值模拟来看,孵化后rge眼球增大过程中基质胶原的渐进性重塑似乎与该疾病呈现的角膜形态测量变化有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b354/2680986/d456fa2ffca7/gr1.jpg

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