Zebrafish models of inherited retinal dystrophies

B. Perkins
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引用次数: 5

Abstract

Inherited retinal degenerations (IRDs) cause permanent vision impairment or vision loss due to the death of rod and cone photoreceptors. Animal models of IRDs have been instrumental in providing knowledge of the pathological mechanisms that cause photoreceptor death and in developing successful approaches that could slow or prevent vision loss. Zebrafish models of IRDs represent an ideal model system to study IRDs in a cone-rich retina and to test strategies that exploit the natural ability to regenerate damaged neurons. This review highlights those zebrafish mutants and transgenic lines that exhibit adult-onset retinal degeneration and serve as models of retinitis pigmentosa, cone-rod dystrophy, and ciliopathies.
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遗传性视网膜营养不良斑马鱼模型
遗传性视网膜退化(IRD)会导致永久性视力损伤或因视杆和视锥感光器死亡而导致的视力丧失。IRD的动物模型在提供导致光感受器死亡的病理机制知识以及开发减缓或预防视力丧失的成功方法方面发挥了重要作用。斑马鱼IRD模型代表了一个理想的模型系统,用于研究富含视锥的视网膜中的IRD,并测试利用自然能力再生受损神经元的策略。这篇综述重点介绍了那些表现出成年视网膜变性的斑马鱼突变体和转基因系,它们是视网膜色素变性、视锥杆营养不良和纤毛病的模型。
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CiteScore
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