Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following Vitamin A supplementation - Wikidata - wikimedia - TriplyDB

Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following Vitamin A supplementation

Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following Vitamin A supplementation

http://www.wikidata.org/entity/Q33777110

about

Functional roles of bestrophins in ocular epithelia The lipid translocase, ABCA4: seeing is believing.Stargardt disease: clinical features, molecular genetics, animal models and therapeutic options Differential expression and function of ABCG1 and ABCG4 during development and aging.Cytochrome P450 2C epoxygenases mediate photochemical stress-induced death of photoreceptors.Complement modulation in the retinal pigment epithelium rescues photoreceptor degeneration in a mouse model of Stargardt disease.Phospholipid meets all-trans-retinal: the making of RPE bisretinoids Retinoids for treatment of retinal diseases The retinal pigment epithelium in health and disease Photoreceptor cell degeneration in Abcr (-/-) mice Defective lipid transport and biosynthesis in recessive and dominant Stargardt macular degeneration Identification of genetic variation and haplotype structure of the canine ABCA4 gene for retinal disease association studies Deuterium enrichment of vitamin A at the C20 position slows the formation of detrimental vitamin A dimers in wild-type rodents Quantitative fundus autofluorescence in mice: correlation with HPLC quantitation of RPE lipofuscin and measurement of retina outer nuclear layer thickness A CTRP5 gene S163R mutation knock-in mouse model for late-onset retinal degeneration Complement system dysregulation and inflammation in the retinal pigment epithelium of a mouse model for Stargardt macular degeneration.Common synonymous variants in ABCA4 are protective for chloroquine induced maculopathy (toxic maculopathy).Fundus Autofluorescence and RPE Lipofuscin in Age-Related Macular Degeneration Membrane receptors and transporters involved in the function and transport of vitamin A and its derivatives.Diacylglycerol O-acyltransferase type-1 synthesizes retinyl esters in the retina and retinal pigment epithelium Gene Therapy of ABCA4-Associated Diseases.Stargardt macular dystrophy: common ABCA4 mutations in South Africa--establishment of a rapid genetic test and relating risk to patients.The bisretinoids of retinal pigment epithelium Clinical phenotypes and prognostic full-field electroretinographic findings in Stargardt disease.Rescue of the Stargardt phenotype in Abca4 knockout mice through inhibition of vitamin A dimerization.Dietary profile of patients with Stargardt's disease and Retinitis Pigmentosa: is there a role for a nutritional approach?Predicting Progression of ABCA4-Associated Retinal Degenerations Based on Longitudinal Measurements of the Leading Disease Front.Retinal phenotypes in patients homozygous for the G1961E mutation in the ABCA4 gene.Effectiveness and safety of nutritional supplements in the treatment of hereditary retinal dystrophies: a systematic review.Correlations between Photodegradation of Bisretinoid Constituents of Retina and Dicarbonyl Adduct Deposition Retinoic Acid Receptor Beta in Pathophysiology of Age-Related Macular Degeneration.Vitamin A-aldehyde adducts: AMD risk and targeted therapeutics.Botanical compounds: effects on major eye diseases Photodegradation of retinal bisretinoids in mouse models and implications for macular degeneration All-Trans Retinoic Acid Modulates DNA Damage Response and the Expression of the VEGF-A and MKI67 Genes in ARPE-19 Cells Subjected to Oxidative Stress Fundus autofluorescence in the Abca4(-/-) mouse model of Stargardt disease--correlation with accumulation of A2E, retinal function, and histology.The role of interphotoreceptor retinoid-binding protein on the translocation of visual retinoids and function of cone photoreceptors G1961E mutant allele in the Stargardt disease gene ABCA4 causes bull's eye maculopathy Evaluation of potential therapies for a mouse model of human age-related macular degeneration caused by delayed all-trans-retinal clearance Progress toward effective treatments for human photoreceptor degenerations.

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Accelerated accumulation of lipofuscin pigments in the RPE of a mouse model for ABCA4-mediated retinal dystrophies following Vitamin A supplementation

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Gabriel H Travis

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Jane Hu

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Quan Yuan

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P2860

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

The photoreceptor rim protein is an ABC transporter encoded by the gene for recessive Stargardt's disease (ABCR)

Retinal stimulates ATP hydrolysis by purified and reconstituted ABCR, the photoreceptor-specific ATP-binding cassette transporter responsible for Stargardt disease

Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice

Stargardt's ABCR is localized to the disc membrane of retinal rod outer segments

Rpe65 is the retinoid isomerase in bovine retinal pigment epithelium

Delayed dark-adaptation and lipofuscin accumulation in abcr+/- mice: implications for involvement of ABCR in age-related macular degeneration

Retinol-binding protein-deficient mice: biochemical basis for impaired vision

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

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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3821-3829

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10.1167/IOVS.07-1470

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18515570

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