Department of Medical Genetics, University of Alberta, Edmonton, AB T6G 2H7, Canada.
Department of Ophthalmology and Visual Sciences, University of Alberta, Edmonton, AB T6G 2R7, Canada.
Biomolecules. 2021 Jan 9;11(1):78. doi: 10.3390/biom11010078.
Zebrafish are an instrumental system for the generation of photoreceptor degeneration models, which can be utilized to determine underlying causes of photoreceptor dysfunction and death, and for the analysis of potential therapeutic compounds, as well as the characterization of regenerative responses. We review the wealth of information from existing zebrafish models of photoreceptor disease, specifically as they relate to currently accepted taxonomic classes of human rod and cone disease. We also highlight that rich, detailed information can be derived from studying photoreceptor development, structure, and function, including behavioural assessments and in vivo imaging of zebrafish. Zebrafish models are available for a diversity of photoreceptor diseases, including cone dystrophies, which are challenging to recapitulate in nocturnal mammalian systems. Newly discovered models of photoreceptor disease and drusenoid deposit formation may not only provide important insights into pathogenesis of disease, but also potential therapeutic approaches. Zebrafish have already shown their use in providing pre-clinical data prior to testing genetic therapies in clinical trials, such as antisense oligonucleotide therapy for Usher syndrome.
斑马鱼是用于产生光感受器变性模型的重要系统，可用于确定光感受器功能障碍和死亡的根本原因，分析潜在的治疗化合物，并研究再生反应。我们回顾了现有的斑马鱼光感受器疾病模型的丰富信息，特别是它们与目前公认的人类视杆和视锥疾病分类学类别相关的信息。我们还强调，通过研究光感受器的发育、结构和功能，包括对斑马鱼的行为评估和体内成像，可以获得丰富而详细的信息。斑马鱼模型可用于多种光感受器疾病，包括在夜间哺乳动物系统中难以再现的圆锥细胞营养不良症。新发现的光感受器疾病和玻璃膜疣形成模型不仅可以为疾病的发病机制提供重要的见解，还可能为潜在的治疗方法提供线索。斑马鱼已经在临床试验前的基因治疗中提供了临床前数据，例如用于 Usher 综合征的反义寡核苷酸治疗，这已经证明了其用途。
