CHOLESTEROL DEPOSITION IN HUMAN CORNEA

胆固醇不仅在动脉粥样硬化中病理性蓄积， 血管也在角膜中。 该项目的重点是病理 胆固醇在角膜组织中的沉积， 了解胆固醇从所有组织中的沉积和去除。 我们 先证者中央角膜的特征性脂质蓄积 三种常染色体显性遗传的角膜脂质 证词 主要是未酯化的细胞外沉积物 观察到结晶和非结晶形式的胆固醇 在角膜组织切片中使用胆固醇的组织化学染色 用菲律宾蛋白和电子显微镜 角膜组织切片还 含有脂质沉积物，被油红0染色，表明 存在胆固醇酯蓄积。 因此，患者角膜 累积超过10倍的总胆固醇和5倍的 与正常角膜相比， 患者和正常角膜 含有相当于其总胆固醇的约50%， 酯化形式。 角膜中的脂质积聚 这些患者在组织化学和化学上都非常相似， 发生在人类动脉粥样硬化病变中的情况。 在这两种疾病中， 未酯化和酯化的胆固醇在细胞外积累， 空间和细胞。 因此，阐明遗传因素的责任 对于这种角膜营养不良的胆固醇积累应该有帮助 在确定哪些局部血管壁因素可能导致 在动脉粥样硬化斑块形成过程中胆固醇沉积。

Cholesterol accumulates pathologically not only in atherosclerotic vessels but also in the cornea. This project focuses on pathological deposition of cholesterol in corneal tissue as a means to more generally understand deposition and removal of cholesterol from all tissues. We characterized lipid accumulation in central corneas removed from probands of three kindreds with autosomal dominantly inherited corneal lipid deposition. Predominantly extracellular deposits of unesterified cholesterol in both crystalline and non-crystalline forms were observed in corneal tissue sections using histochemical staining of cholesterol with filipin and electron microscopy. Corneal tissue sections also contained lipid deposits which stained with oil red 0 suggesting the presence of cholesteryl ester accumulation. Thus, patient corneas accumulated greater than 10-fold total cholesterol and 5-fold phospholipid as compared to normal corneas. Patient and normal corneas contained equals approximately 50% of their total cholesterol in esterified form. The lipid accumulation that occurs in the corneas of these patients is remarkably similar both histochemically and chemically to that which occurs in human atherosclerotic lesions. In both diseases, unesterified and esterified cholesterol accumulate in the extracellular space and in cells. Thus, elucidation of the genetic factors responsible for cholesterol accumulation in this corneal dystrophy should be helpful in determining what local vessel wall factors may contribute to cholesterol deposition during development of atherosclerotic plaques.