The role of the pigment epithelium in the etiology of inherited retinal dystrophy in the rat.
The role of the pigment epithelium in the etiology of inherited retinal dystrophy in the rat.
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DOI：
10.1083/jcb.49.3.664
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发表时间：
1971-06
影响因子：
7.8
通讯作者：
Hall, M O
中科院分区：
文献类型：
--
作者：
Bok, D;Hall, M O
采用光学显微镜和放射自显影相结合的方法，对突变的皇家外科医师学会(RCS)大鼠和对照大鼠的视觉细胞外节更新进行了研究。RCS和对照组大鼠在11日龄时注射氨基酸-3 H。放射性棒外段盘由棒内段合成的放射性蛋白质在外段底部组装而成。对照组，11日龄时组装的放射性盘片在注射后8天移位至外节段长度，从外节段末端取出，并被色素上皮吞噬。在RCS大鼠中，注射后前3天的椎间盘组装和移位与对照组相似。然而，随着椎间盘组装之后的一段时间，在外节尖端和色素上皮之间堆积了一层标记的、无组织的板层碎片。碎片的堆积伴随着视觉细胞的死亡。在研究期间，没有任何证据表明色素上皮具有吞噬活性。注射61天后，碎片层是视网膜中唯一具有较强放射性的成分。在RCS大鼠的视网膜中，由于色素上皮不能完成其正常的吞噬功能，导致外节更新机制功能障碍。最终结果是视觉细胞死亡和失明。
Visual cell outer segment renewal was studied in eyes of mutant Royal College of Surgeons (RCS) and Sprague-Dawley (control) rats by a combination of microscopy and radioautography with the light and electron microscopes. RCS and control rats were injected with amino acids-3H at 11 days of age. Radioactive rod outer segment discs were assembled at the outer segment base from radioactive proteins synthesized in the rod inner segments. In controls, all radioactive discs assembled at 11 days of age were displaced the length of the outer segments, removed from outer segment tips, and phagocytized by the pigment epithelium by 8 days after injection. In the RCS rats, disc assembly and displacement resembled controls for the first 3 days after injection. However, as disc assembly continued for some time thereafter, a layer of labeled, disorganized, lamellar debris accumulated between the outer segment tips and the pigment epithelium. The buildup of debris was accompanied by visual cell death. At no time during the study was there evidence for phagocytic activity by the pigment epithelium. 61 days after injection, the layer of debris was the only heavily radioactive component in the retina. In the retina of RCS rats, the outer segment renewal mechanism malfunctions because the pigment epithelium does not fulfill its normal phagocytic role. The end result is visual cell death and blindness.