Animal models in research on retinal degenerations: past progress and future hope
Animal models in research on retinal degenerations: past progress and future hope
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DOI：
10.1016/s0042-6989(01)00212-7
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发表时间：
2002-02-01
期刊：
影响因子：
1.8
通讯作者：
Chader, GJ
中科院分区：
文献类型：
--
作者：
Chader, GJ
视网膜变性 (RD) 是一系列遗传性视网膜变性疾病（营养不良），可导致视力丧失。尽管表型非常不同。 RD 有几个共同特征。它们都是由基因突变引起的，或者至少在病因学上具有遗传成分。它们都导致光感受器功能障碍，许多导致视杆细胞和视锥细胞光感受器死亡。大多数RD的细胞死亡机制似乎是通过细胞凋亡过程。据估计，全球有超过 1500 万人因遗传性 RD 导致视力丧失。其中许多是患有干性年龄相关性黄斑变性（AMD）的患者，他们保留了部分功能性视力。然而，有些人患有其他退行性疾病，例如色素性视网膜炎。莱伯先天性黑蒙或湿性 AMD 可能会导致严重视力丧失或完全失明。 (C) 2002 年，爱思唯尔科学有限公司出版。
The retinal degenerations (RDs) are a family of inherited retinal degenerative diseases (dystrophies) that lead to vision loss. Although phenotypically very different. the RDs have several characteristics in common. They all are caused by gene mutations or at least have a genetic component in the etiology, They all lead to photoreceptor dysfunction, many leading to the death of both rod and cone photoreceptors. The mechanism of cell death in most of the RDs seems to be through the process of apoptosis. It is estimated that more than fifteen million people around the world have vision loss due to an inherited RD. Many of these are patients with the dry form of age-related macular degeneration (AMD) who retain partial functional vision. However, some have other degenerative conditions such as retinitis pigmentosa. Leber congenital amaurosis or wet AMD and can suffer front severe vision loss or total blindness. (C) 2002 Published by Elsevier Science Ltd.