Tien Luu Viet, Yamamoto Manabu, Tagami Mizuki, Misawa Norihiko, Honda Shigeru
Department of Ophthalmology and Visual Sciences, Graduate School of Medicine, Osaka Metropolitan University, 1-4-3 Asahimachi, Abeno-ku, Osaka, 545-8585, Japan.
Graefes Arch Clin Exp Ophthalmol. 2025 Feb;263(2):415-424. doi: 10.1007/s00417-024-06667-8. Epub 2024 Oct 28.
To investigate the expression patterns of Fibrillin-1 in idiopathic epiretinal membranes (iERM) and identify Fibrillin-1-secreting cells.
iERM samples were collected via standard 27-gauge vitrectomy and subsequently subjected to flat-mount immunohistochemistry with double staining for the following markers: Fibrillin-1, glial acidic fibrillary protein (GFAP), cellular retinaldehyde-binding protein (CRALBP), retinoid isomerohydrolase RPE65 (RPE65), and α-smooth muscle actin (α-SMA).
Fibrillin-1 was detected throughout the iERM. The colocalization of Fibrillin-1 with α-SMA, CRALBP, and RPE65 suggested that myofibroblasts and retinal pigment epithelial (RPE) cells secreted Fibrillin-1. The lack of colocalization between GFAP and Fibrillin-1 indicated that GFAP-positive glial cells did not secrete Fibrillin-1. The colocalization of CRALBP and RPE65 with α-SMA indicated the transformation of RPE cells into myofibroblasts. This suggested that RPE cells transformed into myofibroblasts and secreted Fibrillin-1. The lack of colocalization between GFAP and α-SMA implied that GFAP-positive glial cells did not express α-SMA.
Fibrillin-1 is widely distributed in iERMs, and myofibroblasts were the primary sources of Fibrillin-1 secretion. Additionally, during their transformation into myofibroblasts, RPE cells secreted Fibrillin-1. GFAP-positive glial cells did not express α-SMA nor secrete Fibrillin-1.
What is known Idiopathic epiretinal membranes are a common cause of visual acuity and quality impairment. The protein and cell components of idiopathic epiretinal membrane exhibit diversity. What is new Fibrillin-1 is present throughout the idiopathic epiretinal membrane. Myofibroblasts are the most important source of Fibrillin-1 secretion. Retinal pigment epithelial cells also secrete Fibrillin-1 when transforming into myofibroblast. Glial cells do not transform to myofibroblast and do not secrete Fibrillin-1.
研究原纤维蛋白-1在特发性视网膜前膜（iERM）中的表达模式，并鉴定分泌原纤维蛋白-1的细胞。
通过标准的27G玻璃体切除术收集iERM样本，随后进行平铺免疫组织化学，对以下标志物进行双重染色：原纤维蛋白-1、胶质纤维酸性蛋白（GFAP）、细胞视黄醛结合蛋白（CRALBP）、视黄醇异构水解酶RPE65（RPE65）和α-平滑肌肌动蛋白（α-SMA）。
在整个iERM中均检测到原纤维蛋白-1。原纤维蛋白-1与α-SMA、CRALBP和RPE65的共定位表明，肌成纤维细胞和视网膜色素上皮（RPE）细胞分泌原纤维蛋白-1。GFAP与原纤维蛋白-1之间缺乏共定位表明，GFAP阳性胶质细胞不分泌原纤维蛋白-1。CRALBP和RPE65与α-SMA的共定位表明RPE细胞向肌成纤维细胞的转化。这表明RPE细胞转化为肌成纤维细胞并分泌原纤维蛋白-1。GFAP与α-SMA之间缺乏共定位意味着GFAP阳性胶质细胞不表达α-SMA。
原纤维蛋白-1广泛分布于iERM中，肌成纤维细胞是原纤维蛋白-1分泌的主要来源。此外，在RPE细胞向肌成纤维细胞转化过程中，其分泌原纤维蛋白-1。GFAP阳性胶质细胞不表达α-SMA，也不分泌原纤维蛋白-1。
已知内容 特发性视网膜前膜是视力和视觉质量受损的常见原因。特发性视网膜前膜的蛋白质和细胞成分具有多样性。新发现 原纤维蛋白-1存在于整个特发性视网膜前膜中。肌成纤维细胞是原纤维蛋白-1分泌的最重要来源。视网膜色素上皮细胞在转化为肌成纤维细胞时也分泌原纤维蛋白-1。胶质细胞不会转化为肌成纤维细胞，也不分泌原纤维蛋白-1。
