Affiliation: University of California
- Characterization of native retinal fluorophores involved in biosynthesis of A2E and lipofuscin-associated retinopathiesTam V Bui
Sytera, Inc, 505 Coast Boulevard South, La Jolla, CA 92037, USA
J Biol Chem 281:18112-9. 2006..Fluorescence properties of A2PE-H(2) and other related fluorophores characterized in this report have significance for evaluation of human retinal diseases characterized by aberrant fundus autofluorescence...
- Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agentsGabriel H Travis
Department of Ophthalmology, UCLA School of Medicine, Los Angeles, CA 90095, USA
Annu Rev Pharmacol Toxicol 47:469-512. 2007..Recent progress in both areas provides hope that multiple inherited retinal diseases will soon be treated by pharmaceutical intervention...
- Reductions in serum vitamin A arrest accumulation of toxic retinal fluorophores: a potential therapy for treatment of lipofuscin-based retinal diseasesRoxana A Radu
Jules Stein Eye Institute, University of California, Los Angeles, California, USA
Invest Ophthalmol Vis Sci 46:4393-401. 2005..CONCLUSIONS: These findings demonstrate the vitamin A-dependent nature of A2E biosynthesis and validate a novel therapeutic approach with potential to halt the accumulation of lipofuscin fluorophores in the eye...
- Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following Vitamin A supplementationRoxana A Radu
Department of Ophthalmology, University of California at Los Angeles School of Medicine, Los Angeles, California, USA
Invest Ophthalmol Vis Sci 49:3821-9. 2008..Here the authors tested the hypothesis that dietary supplementation with vitamin A may accelerate lipofuscin pigment formation in abca4(-/-) mice...
- Isomerization and oxidation of vitamin a in cone-dominant retinas: a novel pathway for visual-pigment regeneration in daylightNathan L Mata
Jules Stein Eye Institute, UCLA School of Medicine, Los Angeles, CA 90095, USA
Neuron 36:69-80. 2002..Together these activities comprise a novel pathway that regenerates opsin photopigments at a rate 20-fold faster than the known visual cycle. We suggest that this pathway is responsible for sustained daylight vision in vertebrates...
- The role of interphotoreceptor retinoid-binding protein on the translocation of visual retinoids and function of cone photoreceptorsMinghao Jin
Department of Ophthalmology, Neuroscience Center, Louisiana State University School of Medicine, New Orleans, Louisiana 70112, USA
J Neurosci 29:1486-95. 2009....
- Analysis of the retinoid isomerase activities in the retinal pigment epithelium and retinaGabriel H Travis
Jules Stein Eye Institute, UCLA School of Medicine, Los Angeles, CA, USA
Methods Mol Biol 652:329-39. 2010..Isomerase-2 appears to be present in Müller cells but has not yet been identified. Methods are provided in this chapter for assaying isomerase-2 in chicken retina homogenates...
- Rpe65 is a retinyl ester binding protein that presents insoluble substrate to the isomerase in retinal pigment epithelial cellsNathan L Mata
Jules Stein Eye Institute, University of California School of Medicine, Los Angeles, California 90095, USA
J Biol Chem 279:635-43. 2004..These observations suggest that Rpe65 presents retinyl esters as substrate to the isomerase for synthesis of visual chromophore. This proposed function explains the phenotype in mice and humans lacking Rpe65...
- Treatment with isotretinoin inhibits lipofuscin accumulation in a mouse model of recessive Stargardt's macular degenerationRoxana A Radu
Jules Stein Eye Institute and Department of Biological Chemistry, University of California School of Medicine, Los Angeles, CA 90095, USA
Proc Natl Acad Sci U S A 100:4742-7. 2003..Finally, the results suggest that isotretinoin may be an effective treatment for other forms of retinal or macular degeneration associated with lipofuscin accumulation...
- Chicken retinas contain a retinoid isomerase activity that catalyzes the direct conversion of all-trans-retinol to 11-cis-retinolNathan L Mata
Sytera, Inc, La Jolla, California 92037, USA
Biochemistry 44:11715-21. 2005..Kinetic analysis of these two activities suggests that they act in concert to drive the formation of 11-cis-retinoids in chicken retinas. These activities may be part of a new visual cycle for the regeneration of chromophores in cones...
- The Role of Muller Cells in Visual Pigment RegenerationGabriel H Travis; Fiscal Year: 2010..This project is to study the biochemical mechanisms of visual-pigment regeneration. Defects in pigment regeneration cause inherited blinding diseases. The project will help us understand how these genetic diseases arise. ..
- REGENERATION OF CONE PIGMENTS & TREATMENT OF STARGARDT'SGABRIEL TRAVIS; Fiscal Year: 2006..If successful, this strategy should lead to the initiation of clinical trials on patients with active Stargardt's disease. ..
- Biochemical and Genetic Analysis of the Visual CycleGABRIEL TRAVIS; Fiscal Year: 2007....
- The Role of Muller Cells in Visual Pigment RegenerationGABRIEL TRAVIS; Fiscal Year: 2009..This project is to study the biochemical mechanisms of visual-pigment regeneration. Defects in pigment regeneration cause inherited blinding diseases. The project will help us understand how these genetic diseases arise. ..
- Biochemical and Genetic Analysis of the Visual CycleGABRIEL TRAVIS; Fiscal Year: 2009....
- FUNCTIONAL ANALYSIS OF THE PHOTORECEPTOR RIM PROTEINGABRIEL TRAVIS; Fiscal Year: 2001..phosphorylation? (h) what is the phenotype that results from absence of the rim protein in developing and mature outer segments? and (i) are any of the human inherited retinal dystrophies caused by mutations in the rim protein gene? ..
- FUNCTION /INTERACTION OF RDS/PERIPHERIN IN PHOTORECEPTORGABRIEL TRAVIS; Fiscal Year: 2003..Further, valuable tools will have been created to study the biochemical etiologies of human retinal dystrophies caused by mutations in the RDS gene. ..