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...
- Identification of DES1 as a vitamin A isomerase in Müller glial cells of the retinaJoanna J Kaylor
Jules Stein Eye Institute, University of California, Los Angeles School of Medicine, Los Angeles, California, USA
Nat Chem Biol 9:30-6. 2013..Purified DES1 had very high isomerase-2 activity in the presence of appropriate cofactors, suggesting that DES1 by itself is sufficient for isomerase activity...
- 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..Here, we used an animal model that manifests accelerated lipofuscin accumulation (ABCA4-/- mutant) to evaluate the efficacy of a therapeutic approach based on reduction of serum retinol...
- 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...
- 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...
- 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....
- 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...
- 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...
- 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. ..
- Biochemical and Genetic Analysis of the Visual CycleGABRIEL TRAVIS; Fiscal Year: 2009....