期刊
FEBS LETTERS
卷 587, 期 13, 页码 2008-2017出版社
WILEY
DOI: 10.1016/j.febslet.2013.05.020
关键词
Molecular chaperones; Cell stress; Neurodegeneration; Retinal degeneration photoreceptor; Rhodopsin ERAD; Autophagy; Retinitis pigmentosa; Glaucoma; Age-related macular degeneration
资金
- Wellcome Trust
- RP Fighting Blindness
- BIG Lottery Fund (BLF)
- Fight for Sight
- Medical Research Council [G0700412] Funding Source: researchfish
- MRC [G0700412] Funding Source: UKRI
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. (C) 2013 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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