Directional protein secretion by the retinal pigment epithelium: roles in retinal health and the development of age-related macular degeneration.

Directional protein secretion by the retinal pigment epithelium: roles in retinal health and the development of age-related macular degeneration.
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DOI:
10.1111/jcmm.12070
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发表时间:
2013-07
影响因子:
5.3
通讯作者:
Paraoan L
Paraoan L
中科院分区:
医学2区
文献类型:
--
作者:
Kay P;Yang YC;Paraoan L

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视网膜色素上皮(RPE)的结构和功能完整性是维持神经视网膜功能的基础。这些特化细胞形成极化单层,其充当视网膜-血液屏障,分离具有高度特化功能的两个不同环境:顶侧的神经视网膜的光感受器和基底侧的布鲁赫膜/高度血管化的脉络膜毛细血管。RPE的极化性质对于这两个区域的健康是必不可少的,不仅在营养物和废物运输中,而且在维持视网膜稳态和功能所需的蛋白质的合成和定向分泌中。尽管RPE细胞内的多种功能障碍与年龄相关性黄斑变性(AMD)的发展有关,AMD是法律的失明的主要原因,但在分子和细胞水平上尚未最终确定明确的致病过程。这篇文章的重点是参与定向分泌的RPE蛋白在视网膜的正常功能和潜在的关联不正确的RPE蛋白分泌与AMD的发展。了解RPE极性的重要性以及基本结构和调节组分的正确分泌成为开发针对AMD的新型治疗策略的关键因素。
The structural and functional integrity of the retinal pigment epithelium (RPE) is fundamental for maintaining the function of the neuroretina. These specialized cells form a polarized monolayer that acts as the retinal–blood barrier, separating two distinct environments with highly specialized functions: photoreceptors of the neuroretina at the apical side and Bruch's membrane/highly vascularized choriocapillaris at the basal side. The polarized nature of the RPE is essential for the health of these two regions, not only in nutrient and waste transport but also in the synthesis and directional secretion of proteins required in maintaining retinal homoeostasis and function. Although multiple malfunctions within the RPE cells have been associated with development of age-related macular degeneration (AMD), the leading cause of legal blindness, clear causative processes have not yet been conclusively characterized at the molecular and cellular level. This article focuses on the involvement of directionally secreted RPE proteins in normal functioning of the retina and on the potential association of incorrect RPE protein secretion with development of AMD. Understanding the importance of RPE polarity and the correct secretion of essential structural and regulatory components emerge as critical factors for the development of novel therapeutic strategies targeting AMD.
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