Tuberville A W, Wood T O, McLaughlin B J
Curr Eye Res. 1986 Dec;5(12):939-47. doi: 10.3109/02713688608995175.
The normal human corneal endothelial monolayer maintains stromal water equilibrium and thus, transparency, by means of a pump-leak mechanism. Water leaks into the stroma through non-tight lateral cell junctional complexes and is drawn out by an energy dependent cell membrane ion pump. We investigated the histochemical localization of cytochrome oxidase activity (CO), an important energy-deriving mitochondrial enzyme in dysfunctional corneas with Fuchs' endothelial dystrophy (ED), which is a regionally distributed disease. Keratoconus corneas were used as controls for functional control endothelium. In the central area of the corneal button, decreased CO activity was demonstrated which correlated clinically with central corneal edema. This reflects decreased metabolic activity and/or decreased numbers of mitochondria in the attenuated dysfunctional cells. In the mid-periphery, CO activity was increased in the cellular rosettes surrounding guttata, which may be related to increased synthesis of abnormal Descemet's membrane and guttata. Peripherally, the large polygonal cells resembled functional endothelium in their morphology and CO activity. We have, therefore, demonstrated regional differences in energy metabolism in endothelium from Fuchs' ED patients which may be related to decreased numbers of mitochondria in the dysfunctional cells, and/or to synthesis of abnormal Descemet's membrane material.
正常人类角膜内皮单层通过泵-漏机制维持基质水平衡，从而保持透明度。水通过非紧密的侧向细胞连接复合体漏入基质，并由依赖能量的细胞膜离子泵排出。我们研究了细胞色素氧化酶活性（CO）的组织化学定位，CO是一种重要的产生能量的线粒体酶，在患有富克斯内皮营养不良（ED）的功能失调角膜中进行研究，ED是一种区域分布性疾病。圆锥角膜用作功能正常内皮的对照。在角膜片的中央区域，显示CO活性降低，这在临床上与中央角膜水肿相关。这反映了功能减弱的细胞中代谢活性降低和/或线粒体数量减少。在中周边区域，围绕角膜小滴的细胞玫瑰花结中CO活性增加，这可能与异常Descemet膜和角膜小滴的合成增加有关。在周边区域，大的多边形细胞在形态和CO活性上类似于功能正常的内皮。因此，我们证明了富克斯ED患者内皮能量代谢的区域差异，这可能与功能失调细胞中线粒体数量减少和/或异常Descemet膜物质的合成有关。
