Perkins Brian D
Department of Ophthalmic Research, Cole Eye Institute, Cleveland Clinic, OH 44195, USA.
Department of Ophthalmology, Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, OH 44195, USA.
J Transl Genet Genom. 2022;6(1):95-110. doi: 10.20517/jtgg.2021.47. Epub 2022 Feb 8.
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.
遗传性视网膜变性(IRD)由于视杆和视锥光感受器死亡而导致永久性视力损害或失明。IRD的动物模型有助于了解导致光感受器死亡的病理机制,并开发出可能减缓或预防视力丧失的成功方法。IRD的斑马鱼模型是一个理想的模型系统,用于在富含视锥细胞的视网膜中研究IRD,并测试利用受损神经元自然再生能力的策略。本综述重点介绍了那些表现出成年期视网膜变性并可作为色素性视网膜炎、视锥-视杆营养不良和纤毛病模型的斑马鱼突变体和转基因品系。