Retinoids for treatment of retinal diseases

A decade of molecular biology of retinoic acid receptors

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Safety and Efficacy of Gene Transfer for Leber's Congenital Amaurosis

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Effect of gene therapy on visual function in Leber’s congenital amaurosis

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A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Starqardt macular dystrophy

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Gene therapy restores vision in a canine model of childhood blindness

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A point mutation of the rhodopsin gene in one form of retinitis pigmentosa

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Insights into the Function of Rim Protein in Photoreceptors and Etiology of Stargardt's Disease from the Phenotype in abcr Knockout Mice

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Mutation of the Stargardt Disease Gene ( ABCR ) in Age-Related Macular Degeneration

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G Protein–Coupled Receptor Rhodopsin

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Rhodopsin dimers in native disc membranes

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Dark adaptation and the retinoid cycle of vision

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Human gene therapy for RPE65 isomerase deficiency activates the retinoid cycle of vision but with slow rod kinetics

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G Proteins and Phototransduction

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Organization of the G Protein-coupled Receptors Rhodopsin and Opsin in Native Membranes

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The lipofuscin fluorophore A2E mediates blue light-induced damage to retinal pigmented epithelial cells.

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Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in the Stargardt's disease gene ABCR

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C AROTENOID B IOAVAILABILITY AND B IOCONVERSION

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Phototransduction Motifs and Variations

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Long-Term Restoration of Rod and Cone Vision by Single Dose rAAV-Mediated Gene Transfer to the Retina in a Canine Model of Childhood Blindness

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Isolation and one-step preparation of A2E and <i>iso</i> -A2E, fluorophores from human retinal pigment epithelium

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A Randomized Trial of Vitamin A and Vitamin E Supplementation for Retinitis Pigmentosa

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Biosynthesis of a major lipofuscin fluorophore in mice and humans with <i>ABCR</i> -mediated retinal and macular degeneration

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A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy

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Hepatic stellate cell lipid droplets: A specialized lipid droplet for retinoid storage

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Retinal–specific guanylate cyclase gene mutations in Leber's congenital amaurosis

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Age-related accumulation and spatial distribution of lipofuscin in RPE of normal subjects.

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Diseases Caused by Defects in the Visual Cycle: Retinoids as Potential Therapeutic Agents

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Retinitis pigmentosa caused by a homozygous mutation in the Stargardt disease gene ABCR

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The topography and age relationship of lipofuscin concentration in the retinal pigment epithelium.

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Isomerization and Oxidation of Vitamin A in Cone-Dominant Retinas

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Retinaldehyde represses adipogenesis and diet-induced obesity

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Confronting Complexity: the Interlink of Phototransduction and Retinoid Metabolism in the Vertebrate Retina

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Leber congenital amaurosis: Comprehensive survey of the genetic heterogeneity, refinement of the clinical definition, and genotype-phenotype correlations as a strategy for molecular diagnosis

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Aging and dark adaptation

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A2E, a lipofuscin fluorophore, in human retinal pigmented epithelial cells in culture.

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Further Evidence for an Association of ABCR Alleles with Age-Related Macular Degeneration

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Retinopathy in Mice Induced by Disrupted All-trans-retinal Clearance

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TheRpe65Leu450Met Variation Increases Retinal Resistance Against Light-Induced Degeneration by Slowing Rhodopsin Regeneration

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Turned on by Ca2+! The physiology and pathology of Ca2+-binding proteins in the retina

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The lipofuscin component A2E selectively inhibits phagolysosomal degradation of photoreceptor phospholipid by the retinal pigment epithelium

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Identifying photoreceptors in blind eyes caused by RPE65 mutations: Prerequisite for human gene therapy success

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G Protein-Coupled Receptor Rhodopsin: A Prospectus

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Involvement of All-trans-retinal in Acute Light-induced Retinopathy of Mice

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In vivo measurement of lipofuscin in Stargardt's disease--Fundus flavimaculatus.

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Classification of Fundus Autofluorescence Patterns in Early Age-Related Macular Disease

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Macular degeneration: recent advances and therapeutic opportunities

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Molecular Genetics of Human Retinal Disease

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The G protein‐coupled receptor rhodopsin in the native membrane

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Treatment with isotretinoin inhibits lipofuscin accumulation in a mouse model of recessive Stargardt's macular degeneration

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Reductions in Serum Vitamin A Arrest Accumulation of Toxic Retinal Fluorophores: A Potential Therapy for Treatment of Lipofuscin-Based Retinal Diseases

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Night blindness in Sorsby's fundus dystrophy reversed by vitamin A

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The role of arrestin and retinoids in the regeneration pathway of rhodopsin.

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Histopathology and immunocytochemistry of the neurosensory retina in fundus flavimaculatus.

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Three-dimensional architecture of murine rod outer segments determined by cryoelectron tomography

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Ophthalmic fundus imaging: today and beyond

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Identification and Characterization of All-trans-retinol Dehydrogenase from Photoreceptor Outer Segments, the Visual Cycle Enzyme That Reduces All-trans-retinal to All-trans-retinol

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Vitamin A metabolism in the retinal pigment epithelium: genes, mutations, and diseases

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Pharmacological Chaperone-mediated in Vivo Folding and Stabilization of the P23H-opsin Mutant Associated with Autosomal Dominant Retinitis Pigmentosa

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Noninvasive two-photon imaging reveals retinyl ester storage structures in the eye

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Accelerated Accumulation of Lipofuscin Pigments in the RPE of a Mouse Model for<i>ABCA4</i>-Mediated Retinal Dystrophies following Vitamin A Supplementation

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Lipofuscin accumulation, abnormal electrophysiology, and photoreceptor degeneration in mutant <i>ELOVL4</i> transgenic mice: A model for macular degeneration

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Fundus autofluorescence in patients with age-related macular degeneration and high risk of visual loss

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Lecithin:Retinol Acyltransferase in Retinal Pigment Epithelial Microsomes

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Effect of vitamin A supplementation on rhodopsin mutants threonine-17 → methionine and proline-347 → serine in transgenic mice and in cell cultures

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Rod Outer Segment Retinol Dehydrogenase: Substrate Specificity and Role in Phototransduction

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Light exposure stimulates formation of A2E oxiranes in a mouse model of Stargardt's macular degeneration

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Photooxidation of A2-PE, a photoreceptor outer segment fluorophore, and protection by lutein and zeaxanthin

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Phototransduction: crystal clear

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Retinoid production and catabolism: Role of diet in regulating retinol esterification and retinoic acid oxidation

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The Age Lipid A2E and Mitochondrial Dysfunction Synergistically Impair Phagocytosis by Retinal Pigment Epithelial Cells

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Human cone photoreceptor dependence on RPE65 isomerase

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Recovery of Visual Functions in a Mouse Model of Leber Congenital Amaurosis

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Related enzymes solve evolutionarily recurrent problems in the metabolism of carotenoids

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Intra-retinal visual cycle required for rapid and complete cone dark adaptation

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Downregulation of Cone-Specific Gene Expression and Degeneration of Cone Photoreceptors in the<i>Rpe65</i>^−/−Mouse at Early Ages

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Isolation and characterization of a retinal pigment epithelial cell fluorophore: An <i>all-trans</i> -retinal dimer conjugate

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GCAP1(Y99C) Mutant Is Constitutively Active in Autosomal Dominant Cone Dystrophy

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Opsin/all-trans-Retinal Complex Activates Transducin by Different Mechanisms Than Photolyzed Rhodopsin

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Pharmacological and rAAV Gene Therapy Rescue of Visual Functions in a Blind Mouse Model of Leber Congenital Amaurosis

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Cone Opsin Mislocalization in<i>Rpe65</i>^−/−Mice: A Defect That Can Be Corrected by 11-<i>cis</i>Retinal

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Aging and scotopic sensitivity

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Isorhodopsin rather than rhodopsin mediates rod function in RPE65 knock-out mice

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Positively charged retinoids are potent and selective inhibitors of the trans-cis isomerization in the retinoid (visual) cycle

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