From the Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104.
J Biol Chem. 2014 May 9;289(19):13419-33. doi: 10.1074/jbc.M114.562157. Epub 2014 Apr 1.
Retinal detachment is the physical separation of the retina from the retinal pigment epithelium. It occurs during aging, trauma, or during a variety of retinal disorders such as age-related macular degeneration, diabetic retinopathy, retinopathy of prematurity, or as a complication following cataract surgery. This report investigates the role of fibulin 2, an extracellular component, in retinal detachment. A major mechanism for detachment resolution is enhancement of cellular adhesion between the retina and the retinal pigment epithelium and prevention of its cellular migration. This report shows that fibulin 2 is mainly present in the retinal pigment epithelium, Bruch membrane, choriocapillary, and to a lesser degree in the retina. In vitro studies revealed the presence of two isoforms for fibulin 2. The small isoform is located inside the cell, and the large isoform is present inside and outside the cells. Furthermore, fibulin 2 is post-translationally modified by tyrosine sulfation, and the sulfated isoform is present outside the cell, whereas the unsulfated pool is internally located. Interestingly, sulfated fibulin 2 significantly reduced the rate of cellular growth and migration. Finally, levels of fibulin 2 dramatically increased in the retinal pigment epithelium following retinal detachment, suggesting a direct role for fibulin 2 in the re-attachment of the retina to the retinal pigment epithelium. Understanding the role of fibulin 2 in enhancing retinal attachment is likely to help improve the current therapies or allow the development of new strategies for the treatment of this sight-threatening condition.
视网膜脱离是视网膜与视网膜色素上皮分离的物理现象。它发生于衰老、创伤或各种视网膜疾病中,如年龄相关性黄斑变性、糖尿病性视网膜病变、早产儿视网膜病变,或作为白内障手术后的并发症。本报告研究了细胞外成分纤连蛋白 2 在视网膜脱离中的作用。脱离复位的主要机制是增强视网膜和视网膜色素上皮之间的细胞黏附,并防止其细胞迁移。本报告显示纤连蛋白 2 主要存在于视网膜色素上皮、Bruch 膜、脉络膜中,在视网膜中含量较少。体外研究表明纤连蛋白 2 存在两种同工型。小同工型位于细胞内,大同工型存在于细胞内外。此外,纤连蛋白 2 发生酪氨酸硫酸化的翻译后修饰,硫酸化同工型存在于细胞外,而未硫酸化的同工型则位于细胞内。有趣的是,硫酸化纤连蛋白 2 显著降低了细胞生长和迁移的速度。最后,视网膜脱离后视网膜色素上皮中的纤连蛋白 2 水平显著增加,提示纤连蛋白 2 在视网膜重新附着于视网膜色素上皮中起直接作用。了解纤连蛋白 2 在增强视网膜附着中的作用可能有助于改善目前的治疗方法或允许开发治疗这种威胁视力的疾病的新策略。