Altered corneal stromal matrix organization is associated with mucopolysaccharidosis I, III and VI
Altered corneal stromal matrix organization is associated with mucopolysaccharidosis I, III and VI
复制标题
DOI：
10.1006/exer.1998.0622
复制
发表时间：
1999-05-01
影响因子：
3.4
通讯作者：
Birk, DE
中科院分区：
文献类型：
--
作者：
Alroy, J;Haskins, M;Birk, DE
角膜混浊是I型和VI型粘多糖沉积症（MPS）的显著特征，但不是MPS IIIA或IIIB。MPS I和VI中角膜混浊的原因是推测性的。角膜的透明度取决于基质内胶原纤维的均匀直径和规则间距和排列。在包括角膜水肿、瘢痕和黄斑角膜营养不良在内的各种病症中胶原原纤维间距的改变在临床上表现为角膜混浊。本研究的目的是比较正常角膜与MPS角膜的基质细胞外基质的结构组织。检查MPS I患者混浊角膜中胶原纤维的大小和排列。观察到的变化是平均原纤维直径增加，在MPS角膜中分布更广。与正常对照角膜相比，MPS I角膜也具有改变的原纤维间距和更不规则的填充。MPS IIIA和IIIB患者的透明角膜也显示平均原纤维直径和原纤维间距增加。然而，变化较少，表明比MPS I中观察到的规律性更强。此外，将来自某些MPS的猫模型的角膜与人角膜进行比较。对MPS I和VI猫以及正常对照猫进行了检查。相对于正常透明角膜，在MPS I和VI猫中观察到与人MPS角膜中观察到的结构变化相当的结构变化。研究结果表明，MPS I和VI中混浊的角膜部分是角膜基质结构改变的结果，包括胶原纤维的异常间距、大小和排列。(C)北京：科学出版社.
The presence of cloudy corneas is a prominent feature of mucopolysaccharidosis (MPS) types I and VI, but not MPS IIIA or IIIB. The cause of corneal cloudiness in MPS I and VI is speculative. Transparency of the cornea is dependent on the uniform diameter and the regular spacing and arrangement of the collagen fibrils within the stroma. Alterations in the spacing of collagen fibrils in a variety of conditions including corneal edema, scars, and macular corneal dystrophy is clinically manifested as corneal opacity. The purpose of this study was to compare the structural organization of the stromal extracellular matrix of normal corneas with that of MPS corneas. The size and arrangement of collagen fibrils in cloudy corneas from patients with MPS I were examined. The alterations observed were an increased mean fibril diameter with a broader distribution in the MPS corneas. The MPS I corneas also had altered fibril spacing and more irregular packing compared with normal control corneas. The clear corneas of patients with MPS IIIA and IIIB also showed increases in mean fibril diameter and fibril spacing. However, there was less variation indicating more regularity than seen in MPS I. In addition, corneas from cat models of certain MPS were compared to the human corneas. Cats with MPS I and VI, as well as normal control cats, were examined. Structural alterations comparable to those seen in human MPS corneas were seen in MPS I and VI cats relative to normal clear corneas. The findings suggest that cloudy corneas in MPS I and VI are in part a consequence of structural alterations in the corneal stroma, including abnormal spacing, size, and arrangement of collagen fibrils. (C) 1999 Academic Press.