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Adv Exp Med Biol. 2014;801:77-83. doi: 10.1007/978-1-4614-3209-8_10.
Renewal and elimination of aged photoreceptor outer segment (POS) tips by cells from the retinal pigment epithelial (RPE) is a daily rhythmic process that is crucial for long-term vision. Anomalies can arise during any of the sequential steps required for completion of this phagocytic function, from POS recognition to complete digestion of POS components. During the past 15 years, many animal models helped us characterize the molecular machinery implicated in RPE phagocytosis as well as understand associated defects leading to various retinal pathologies. Depending on which part of the machinery is flawed, phenotypes can either appear early in life, such as retinitis pigmentosa or Usher syndrome, or develop with aging of the individual, like age-related macular degeneration, affecting first either the peripheral or the central retina. This chapter describes mouse and rat models related to defective phagocytosis, and how they have been a tremendous help for us to comprehend RPE phagocytosis, its rhythm, and its failures.
视网膜色素上皮 (RPE) 细胞对老化的光感受器外节 (POS) 尖端的更新和消除是一个每日节律性过程，对长期视觉至关重要。在完成这个吞噬功能的连续步骤中的任何一个步骤中，都可能会出现异常，从 POS 的识别到 POS 成分的完全消化。在过去的 15 年中，许多动物模型帮助我们描述了涉及 RPE 吞噬作用的分子机制，并了解了导致各种视网膜病变的相关缺陷。根据机器的哪个部分有缺陷，表型可以在生命早期出现，如色素性视网膜炎或 Usher 综合征，或者随着个体的衰老而发展，如年龄相关性黄斑变性，首先影响周边或中央视网膜。本章描述了与吞噬作用缺陷相关的小鼠和大鼠模型，以及它们如何极大地帮助我们理解 RPE 吞噬作用、其节律和失败。
