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[WNT信号通路在依赖纤毛的视网膜色素上皮细胞发育过程中的作用]

[The Role of WNT Signaling During Ciliary-Dependent RPE Development].

作者信息

Kretschmer Viola, May-Simera Helen Louise

机构信息

Institut für Molekulare Physiologie, Johannes-Gutenberg-Universität Mainz.

出版信息

Klin Monbl Augenheilkd. 2020 Mar;237(3):248-258. doi: 10.1055/a-1098-8730. Epub 2020 Mar 17.

Abstract

The retinal pigment epithelium (RPE) is a highly polarized single layer of block-shaped epithelial cells that are densely packed with melanin. They lie between the light sensitive external segments of the photoreceptors and the choroid. They play an essential role in the development of photoreceptors and have important functions in nutrient supply and maintenance, in retinal metabolism and in shielding the blood supply of the choroid. The photoreceptors are subject to daily renewal, in which 10% of the external segments are phagocytosed by the retinal pigment epithelium. This requires close interactions between the retinal pigment epithelium and the retina. Thus, disturbance or delay of the maturation of the RPE can trigger pathogenic changes in the retina, leading to degeneration of the photoreceptors. The aging of the RPE can also impair underlying functions, which can lead to progressive loss of photoreceptors and visual acuity. Like many types of ocular cells, the RPE forms a primary cilium during its development, a protuberance of the cell membrane based on microtubuli. This is thought to be associated with some important cellular processes and various important signaling pathways. In particular, the WNT pathway (wingless-related integration site) is essential for the polarization and maturation of the RPE and therefore of decisive importance for the function of the epithelium.

摘要

视网膜色素上皮(RPE)是一层高度极化的块状上皮细胞单层,细胞内密集填充着黑色素。它们位于光感受器的感光外段与脉络膜之间。它们在光感受器的发育中起着至关重要的作用,在营养供应与维持、视网膜代谢以及遮挡脉络膜血液供应方面具有重要功能。光感受器会进行每日更新,其中10%的外段会被视网膜色素上皮吞噬。这需要视网膜色素上皮与视网膜之间紧密相互作用。因此,RPE成熟的紊乱或延迟会引发视网膜的致病性变化,导致光感受器退化。RPE的衰老也会损害其潜在功能,进而导致光感受器和视力的逐渐丧失。与许多类型的眼细胞一样,RPE在其发育过程中会形成一根初级纤毛,这是基于微管的细胞膜突出物。这被认为与一些重要的细胞过程和各种重要的信号通路相关。特别是,WNT通路(无翅相关整合位点)对于RPE的极化和成熟至关重要,因此对上皮细胞的功能具有决定性意义。

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