Generation and characterisation of an animal model for age-related macular degeneration

年龄相关性黄斑变性（AMD）是发达国家老年人不可逆失明的主要原因，也是人们了解最少的视网膜疾病之一。黄斑变性是一种缓慢的、渐进的、无痛的疾病，影响黄斑，黄斑是视网膜的小中心部分，可以让人清楚地看到细节。随着人类预期寿命的不断延长，AMD在75岁以上年龄组的患病率（15-30%）将显著增加，给社会造成巨大的社会和经济问题。尽管这个问题很重要，但AMD的确切病因尚不清楚，也没有永久性有效的治疗或治愈方法。阻碍干预策略或疗法发展的主要障碍之一是缺乏适当的动物模型。目前，可用于眼部疾病的动物模型并不适合人类AMD的情况。本项目旨在建立第一个由视网膜色素上皮细胞（RPE）功能异常引起的视网膜变性动物模型。RPE细胞的主要作用是吞噬和消化眼睛内不断生长的发光受体片段。因此，它们的正常功能对保持良好的视力至关重要。这种动物模型的可用性将使我们能够更多地了解可能发生在眼睛中的变化，从而导致AMD的发展，并设计预防或延缓病情进展的策略。

Age-Related Macular Degeneration (AMD) is the leading cause of irreversible blindness in the aged population in the developed world, and it is one of the least understood retinal diseases. AMD is a slow, progressive and painless condition that affects the macula, the small central part of the retina that allows one to see fine detail clearly. With the ever-increasing human life expectancy, the prevalence of AMD (15-30%) in the age group of over 75 years will significantly increase, causing enormous social and financial problems for the community. In spite of the significance of this problem, the exact cause of AMD is not yet known, and there is no permanent effective treatment or cure for the condition. One of the major obstacles hindering any advances towards the development of intervention strategies or therapies is the lack of an appropriate animal model. Currently, the animal models that are available for ocular diseases do not fit the human AMD situation. This project aims to characterize the first animal model for retinal degeneration caused by abnormal functioning of the retinal pigment epithelial cells (RPE). The main role of RPE cells is the phagocytosis and digestion of the continuously growing and shed light receptor segments in the eye. Their normal functioning therefore is vital to maintaining good vision. The availability of such an animal model will allow us to learn more about the changes that might occur in the eye leading to the development of AMD and to design strategies to prevent or delay progression of the condition.