The cell stress machinery and retinal degeneration.
The cell stress machinery and retinal degeneration.
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DOI：
10.1016/j.febslet.2013.05.020
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发表时间：
2013-06-27
期刊：
影响因子：
3.5
通讯作者：
Cheetham ME
中科院分区：
文献类型：
--
作者：
Athanasiou D;Aguilà M;Bevilacqua D;Novoselov SS;Parfitt DA;Cheetham ME
视网膜变性是一组临床和遗传异质性疾病，其特征是由于神经变性导致视力进行性丧失。视网膜是一种高度专业化的组织，具有独特的结构，维持所有不同视网膜细胞类型的稳态对于健康视力至关重要。视网膜可能会受到各种环境损害和压力，包括光诱导的损伤、氧化应激和可能导致蛋白质错误折叠的遗传突变。视网膜细胞内有不同的机制来应对蛋白质稳态紊乱，例如热休克反应、未折叠蛋白反应和自噬。在这篇综述中，我们讨论了视网膜对视网膜变性所涉及的不同类型压力的多重反应，例如色素性视网膜炎、年龄相关性黄斑变性和青光眼。了解维持和重建视网膜蛋白质稳态的机制对于开发抗失明的新治疗方法非常重要。
Retinal degenerations are a group of clinically and genetically heterogeneous disorders characterised by progressive loss of vision due to neurodegeneration. The retina is a highly specialised tissue with a unique architecture and maintaining homeostasis in all the different retinal cell types is crucial for healthy vision. The retina can be exposed to a variety of environmental insults and stress, including light-induced damage, oxidative stress and inherited mutations that can lead to protein misfolding. Within retinal cells there are different mechanisms to cope with disturbances in proteostasis, such as the heat shock response, the unfolded protein response and autophagy. In this review, we discuss the multiple responses of the retina to different types of stress involved in retinal degenerations, such as retinitis pigmentosa, age-related macular degeneration and glaucoma. Understanding the mechanisms that maintain and re-establish proteostasis in the retina is important for developing new therapeutic approaches to fight blindness.
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