Yue B Y, Duvall J, Goldberg M F, Puck A, Tso M O, Sugar J
Ophthalmology. 1986 Apr;93(4):534-41. doi: 10.1016/s0161-6420(86)33704-7.
We performed morphologic and tissue culture studies on scleral tissues obtained from a nanophthalmic patient. Seen by light and electron microscopy, collagens in these unusually thick scleral tissues were arranged in irregularly interlacing bundles. The size of the collagen fibers was more variable than that observed in the controls. These abnormal fibers appeared twisted and, in some areas, were more closely packed. In tissue culture, scleral cells derived from the nanophthalmic patient synthesized proteins and collagen at a rate similar to that of normal control cells. The level of glycosaminoglycan produced, however, was markedly reduced. The modified glycosaminoglycan metabolism in scleral cells may be related to the abnormal packing of collagen bundles, which may in turn contribute to the thickening of sclera and the formation of nanophthalmos.
我们对一名小眼球症患者的巩膜组织进行了形态学和组织培养研究。通过光学显微镜和电子显微镜观察，这些异常增厚的巩膜组织中的胶原蛋白呈不规则交织束状排列。胶原纤维的大小比对照组中观察到的更具变异性。这些异常纤维呈扭曲状，在某些区域排列更为紧密。在组织培养中，来自小眼球症患者的巩膜细胞合成蛋白质和胶原蛋白的速度与正常对照细胞相似。然而，所产生的糖胺聚糖水平明显降低。巩膜细胞中糖胺聚糖代谢的改变可能与胶原束的异常堆积有关，而这反过来可能导致巩膜增厚和小眼球症的形成。
