Abstract
Purpose: :
We have previously reported a cynomolgus (Macaca fascicularis) pedigree with early-onset macular degeneration. These monkeys show cardinal features of age-related macular degeneration in human such as pigmentary disorders and/or drusen-like spots at two years after birth (Umeda et al., 2005) and ERG abnormality. Here we report a characterization of retinal pigment epithelium (RPE) cells isolated from two affected monkeys.
Methods: :
RPE cells were isolated from normal and affected monkeys with severe and modest disease progression based on fundus photograph, ERG, and fluorescence fundus photograph. The RPE cells were used for immunohistochemistry analysis, autophagy detection, phagocytosis analysis, gene expression analysis using cDNA microarray.
Results: :
RPE cells from affected monkeys were significantly reduced of autophagy recycle and phagocytosis activity. These RPE cells were also unable to form tight junction observed by Zonula Occludens-1 (ZO-1) immunostaining. Gene expression of RPE cells were significantly exhibiting significant expression changed at different disease stages compared with normal RPE cells.
Conclusions: :
In this study, primary RPE cells from affected and control monkeys were analyzed. Significant change of gene expression leading to loss of tight junction and reduces of autophagy and phagocytosis activity in RPE cells suggests that dysfunction become to macular degeneration.
Keywords: age-related macular degeneration • retinal pigment epithelium • drusen