A review of the mechanisms of cone degeneration in retinitis pigmentosa
Retinitis pigmentosa (RP) is an inherited condition that features degeneration of rod and cone photoreceptors. In all forms of RP, the genetic mutation is expressed exclusively in rods; however, cones die too. The secondary death of cones in RP remains somewhat mysterious. A better understanding of...
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| Veröffentlicht in: | Acta ophthalmologica (Oxford, England) England), 2016-12, Vol.94 (8), p.748-754 |
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| container_title | Acta ophthalmologica (Oxford, England) |
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| creator | Narayan, Daniel S. Wood, John P. M. Chidlow, Glyn Casson, Robert J. |
| description | Retinitis pigmentosa (RP) is an inherited condition that features degeneration of rod and cone photoreceptors. In all forms of RP, the genetic mutation is expressed exclusively in rods; however, cones die too. The secondary death of cones in RP remains somewhat mysterious. A better understanding of the mechanisms that cause cone degeneration in RP could lead to novel treatments that preserve cones. There are a number of prevailing theories that attempt to explain cone degeneration in RP. One concept is that cone survival is dependent on trophic factors produced by rods. Another hypothesis is that cones suffer from a nutrient shortage after rods have been lost. Additionally, oxidative stress and pro‐inflammatory microglial activation have also been suggested to play a role in cone death. The present review evaluates the evidence supporting these theories and provides an update on the mechanisms of cone degeneration in RP. |
| doi_str_mv | 10.1111/aos.13141 |
| format | Article |
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The present review evaluates the evidence supporting these theories and provides an update on the mechanisms of cone degeneration in RP.</description><identifier>ISSN: 1755-375X</identifier><identifier>EISSN: 1755-3768</identifier><identifier>DOI: 10.1111/aos.13141</identifier><identifier>PMID: 27350263</identifier><language>eng</language><publisher>England: Wiley Subscription Services, Inc</publisher><subject>Cones ; Degeneration ; Humans ; Inflammation ; microglia ; Mutation ; Ophthalmology ; Oxidative stress ; Photoreceptors ; RdCVF ; Retinal Cone Photoreceptor Cells - pathology ; Retinal Degeneration - physiopathology ; Retinitis ; Retinitis pigmentosa ; Retinitis Pigmentosa - physiopathology ; Rods ; Trophic factors</subject><ispartof>Acta ophthalmologica (Oxford, England), 2016-12, Vol.94 (8), p.748-754</ispartof><rights>2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd</rights><rights>2016 Acta Ophthalmologica Scandinavica Foundation. 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M.</creatorcontrib><creatorcontrib>Chidlow, Glyn</creatorcontrib><creatorcontrib>Casson, Robert J.</creatorcontrib><title>A review of the mechanisms of cone degeneration in retinitis pigmentosa</title><title>Acta ophthalmologica (Oxford, England)</title><addtitle>Acta Ophthalmol</addtitle><description>Retinitis pigmentosa (RP) is an inherited condition that features degeneration of rod and cone photoreceptors. In all forms of RP, the genetic mutation is expressed exclusively in rods; however, cones die too. The secondary death of cones in RP remains somewhat mysterious. A better understanding of the mechanisms that cause cone degeneration in RP could lead to novel treatments that preserve cones. There are a number of prevailing theories that attempt to explain cone degeneration in RP. One concept is that cone survival is dependent on trophic factors produced by rods. Another hypothesis is that cones suffer from a nutrient shortage after rods have been lost. Additionally, oxidative stress and pro‐inflammatory microglial activation have also been suggested to play a role in cone death. The present review evaluates the evidence supporting these theories and provides an update on the mechanisms of cone degeneration in RP.</description><subject>Cones</subject><subject>Degeneration</subject><subject>Humans</subject><subject>Inflammation</subject><subject>microglia</subject><subject>Mutation</subject><subject>Ophthalmology</subject><subject>Oxidative stress</subject><subject>Photoreceptors</subject><subject>RdCVF</subject><subject>Retinal Cone Photoreceptor Cells - pathology</subject><subject>Retinal Degeneration - physiopathology</subject><subject>Retinitis</subject><subject>Retinitis pigmentosa</subject><subject>Retinitis Pigmentosa - physiopathology</subject><subject>Rods</subject><subject>Trophic factors</subject><issn>1755-375X</issn><issn>1755-3768</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqN0U9LwzAYBvAgitPpwS8gBS96mOZ_uuMQnYLgQQVvIU3fbhltMptW8dsb3dxBUAyBvIRfHggPQkcEn5O0LkyI54QRTrbQHlFCjJiS-fZmFs8DtB_jAmNJpOS7aEAVE5hKtoemk6yFVwdvWaiybg5ZA3ZuvItN_LyxwUNWwgw8tKZzwWfOpwed865zMVu6WQO-C9EcoJ3K1BEO1-cQPV1fPV7ejO7up7eXk7uRFUSQkZJlQVlhTVlCUdAxJaIyAMBNWeVFURFrqMWCq6JKm_NS5lRZlgOUHEuL2RCdrnKXbXjpIXa6cdFCXRsPoY-a5DJnnAs1_gelUmEmOE_05AddhL716SOajDFXCmPK_lQ554pLwWRSZytl2xBjC5Vetq4x7bsmWH_WpVNd-quuZI_XiX3RQLmR3_0kcLECb66G99-T9OT-YRX5AX9znY8</recordid><startdate>201612</startdate><enddate>201612</enddate><creator>Narayan, Daniel S.</creator><creator>Wood, John P. 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| source | Wiley Free Content; MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Cones Degeneration Humans Inflammation microglia Mutation Ophthalmology Oxidative stress Photoreceptors RdCVF Retinal Cone Photoreceptor Cells - pathology Retinal Degeneration - physiopathology Retinitis Retinitis pigmentosa Retinitis Pigmentosa - physiopathology Rods Trophic factors |
| title | A review of the mechanisms of cone degeneration in retinitis pigmentosa |
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