Department of Medical Genetics and Developmental Biology, Fourth Military Medical University, Xi'an 710032, China.
J Genet Genomics. 2010 Sep;37(9):573-82. doi: 10.1016/S1673-8527(09)60077-1.
The retina is one of the most essential elements of vision pathway in vertebrate. The dysplasia of retina cause congenital blindness or vision disability in individuals, and the misbalance in adult retinal vascular homeostasis leads to neovascularization-associated diseases in adults, such as diabetic retinopathy or age-related macular degeneration. Many developmental signaling pathways are involved in the process of retinal development and vascular homeostasis. Among them, Notch signaling pathway has long been studied, and Notch signaling-interfered mouse models show both neural retina dysplasia and vascular abnormality. In this review, we discuss the roles of Notch signaling in the maintenance of retinal progenitor cells, specification of retinal neurons and glial cells, and the sustaining of retina vascular homeostasis, especially from the aspects of conditional knockout mouse models. The potential of Notch signal manipulation may provide a powerful cell fate- and neovascularization-controlling tool that could have important applications in treatment of retinal diseases.
视网膜是脊椎动物视觉通路中最重要的组成部分之一。视网膜的发育异常会导致个体先天性失明或视力障碍，而成人视网膜血管内稳态的失衡会导致与新生血管相关的疾病，如糖尿病视网膜病变或年龄相关性黄斑变性。许多发育信号通路参与了视网膜发育和血管内稳态的过程。其中，Notch 信号通路一直是研究的热点，Notch 信号通路干扰的小鼠模型表现出神经视网膜发育不良和血管异常。在这篇综述中，我们讨论了 Notch 信号在维持视网膜祖细胞、视网膜神经元和神经胶质细胞的特化以及维持视网膜血管内稳态中的作用，特别是从条件性基因敲除小鼠模型的角度。Notch 信号的潜在操纵可能提供一种强大的细胞命运和新生血管控制工具，在治疗视网膜疾病方面具有重要的应用价值。
