title: The cell stress machinery and retinal degeneration.
abstract: 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.
authors.affiliation: UCL Institute of Ophthalmology, 11-43 Bath Street, London EC1V 9EL, UK.
authors.forename: Dimitra
authors.initials: D
authors.lastname: Athanasiou
authors.forename: Monica
authors.initials: M
authors.lastname: Aguilà
authors.forename: Dalila
authors.initials: D
authors.lastname: Bevilacqua
authors.forename: Sergey S
authors.initials: SS
authors.lastname: Novoselov
authors.forename: David A
authors.initials: DA
authors.lastname: Parfitt
authors.forename: Michael E
authors.initials: ME
authors.lastname: Cheetham
mesh_terms: D000818:Animals; D006801:Humans; D009154:Mutation; D057165:Proteostasis Deficiencies / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D012160:Retina / Q000378:metabolism / Q000473:pathology; D012162:Retinal Degeneration / Q000378:metabolism* / Q000473:pathology / Q000628:therapy; D055213:Retinal Pigment Epithelium / Q000378:metabolism* / Q000473:pathology; D012171:Retinal Vessels / Q000473:pathology; D012243:Rhodopsin / Q000235:genetics / Q000378:metabolism; D013312:Stress, Physiological
chemical_list: D012243:Rhodopsin