Light and inherited retinal degeneration
- 1Medical Scientist Training Program and Beckman Vision Center, UCSF School of Medicine, San Francisco, CA, USA
- 2Beckman Vision Center, UCSF School of Medicine, San Francisco, CA, USA
- Correspondence to: Jacque L Duncan MD, Beckman Vision Center, UCSF School of Medicine, San Francisco, CA 94143–0730, USA; duncanj{at}vision.ucsf.edu
- Accepted 1 May 2006
- Published Online First 17 May 2006
Abstract
Light deprivation has long been considered a potential treatment for patients with inherited retinal degenerative diseases, but no therapeutic benefit has been demonstrated to date. In the few clinical studies that have addressed this issue, the underlying mutations were unknown. Our rapidly expanding knowledge of the genes and mechanisms involved in retinal degeneration have made it possible to reconsider the potential value of light restriction in specific genetic contexts. This review summarises the clinical evidence for a modifying role of light exposure in retinal degeneration and experimental evidence from animal models, focusing on retinitis pigmentosa with regional degeneration, Oguchi disease, and Stargardt macular dystrophy. These cases illustrate distinct pathophysiological roles for light, and suggest that light restriction may benefit carefully defined subsets of patients.
- ADRP, autosomal dominant retinitis pigmentosa
- ERG, electroretinography
- N-ret-PE, N-retinylidene-phosphatidylethanolamine
- RHO, rhodopsin
- RK, rhodopsin kinase
- RmP, rim protein
- RP, retinitis pigmentosa
- RPE, retinal pigment epithelium
- STGD, Stargardt disease
- light
- retinal degeneration
- retinitis pigmentosa
- Stargardt disease
- Oguchi disease
- ADRP, autosomal dominant retinitis pigmentosa
- ERG, electroretinography
- N-ret-PE, N-retinylidene-phosphatidylethanolamine
- RHO, rhodopsin
- RK, rhodopsin kinase
- RmP, rim protein
- RP, retinitis pigmentosa
- RPE, retinal pigment epithelium
- STGD, Stargardt disease
- light
- retinal degeneration
- retinitis pigmentosa
- Stargardt disease
- Oguchi disease
Footnotes
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Competing interests: none.









