Associated Papers
More insights from Abca4-/- mouse models of recessive Stargardt disease.
Zhao J et al.
The Journal of Biological Chemistry, 2026
Mechanism of Lutein to meso-Zeaxanthin Isomerization by RPE65 Catalysis.
Ballinger A et al.
Biorxiv : the Preprint Server for Biology, 2025
Subtle retinal degeneration in pigmented Abca4-/-Rdh8-/- mice.
Glänzer S et al.
Experimental Eye Research, 2025
Developmental riboflavin deficiency results in structural and functional changes in the neural retina and RPE.
Zhao X et al.
Redox Biology, 2025
Inactivation of cellular retinol-binding protein 1 protects against bis-retinoid accumulation and light-induced retinal degeneration in mice.
Widjaja-Adhi MAK et al.
The Journal of Biological Chemistry, 2025
A Y178C rhodopsin mutation causes aggregation and comparatively severe retinal degeneration.
Vasudevan S et al.
Cell Death Discovery, 2025
Dual CRALBP isoforms unveiled: iPSC-derived retinal modeling and AAV2/5-RLBP1 gene transfer raise considerations for effective therapy.
Damodar K et al.
Molecular Therapy : the Journal of the American Society of Gene Therapy, 2024
Distinct mouse models of Stargardt disease display differences in pharmacological targeting of ceramides and inflammatory responses.
Engfer ZJ et al.
Proceedings of the National Academy of Sciences of the United States of America, 2023
Chromophore supply modulates cone function and survival in retinitis pigmentosa mouse models.
Xue Y et al.
Proceedings of the National Academy of Sciences of the United States of America, 2023
Products of the visual cycle are detected in mice lacking retinol binding protein 4, the only known vitamin A carrier in plasma.
Montenegro D et al.
The Journal of Biological Chemistry, 2022
Riboflavin deficiency leads to irreversible cellular changes in the RPE and disrupts retinal function through alterations in cellular metabolic homeostasis.
Sinha T et al.
Redox Biology, 2022
Mouse Models of Inherited Retinal Degeneration with Photoreceptor Cell Loss.
Collin GB et al.
Cells, 2020
KIT ligand protects against both light-induced and genetic photoreceptor degeneration.
Li H et al.
Elife, 2020
Photic generation of 11-cis-retinal in bovine retinal pigment epithelium.
Zhang J et al.
The Journal of Biological Chemistry, 2019
Aberrant RNA splicing is the major pathogenic effect in a knock-in mouse model of the dominantly inherited c.1430A>G human RPE65 mutation.
Li Y et al.
Human Mutation, 2019
A Novel Mouse Model of MYO7A USH1B Reveals Auditory and Visual System Haploinsufficiencies.
Calabro KR et al.
Frontiers in Neuroscience, 2019
Bisretinoids mediate light sensitivity resulting in photoreceptor cell degeneration in mice lacking the receptor tyrosine kinase Mer.
Zhao J et al.
The Journal of Biological Chemistry, 2018
Flavin homeostasis in the mouse retina during aging and degeneration.
Sinha T et al.
The Journal of Nutritional Biochemistry, 2018
Clinically Relevant Outcome Measures for the I307N Rhodopsin Mouse: A Model of Inducible Autosomal Dominant Retinitis Pigmentosa.
Massengill MT et al.
Investigative Ophthalmology & Visual Science, 2018
Ciliary neurotrophic factor (CNTF) protects retinal cone and rod photoreceptors by suppressing excessive formation of the visual pigments.
Li S et al.
The Journal of Biological Chemistry, 2018
A non-retinoid antagonist of retinol-binding protein 4 rescues phenotype in a model of Stargardt disease without inhibiting the visual cycle.
Racz B et al.
The Journal of Biological Chemistry, 2018
Insights into the pathogenesis of dominant retinitis pigmentosa associated with a D477G mutation in RPE65.
Choi EH et al.
Human Molecular Genetics, 2018
Increased susceptibility to fundus camera-delivered light-induced retinal degeneration in mice deficient in oxidative stress response proteins.
Ding Y et al.
Experimental Eye Research, 2017
Tyrosinase-Cre-Mediated Deletion of the Autophagy Gene Atg7 Leads to Accumulation of the RPE65 Variant M450 in the Retinal Pigment Epithelium of C57BL/6 Mice.
Sukseree S et al.
Plos One, 2016
Di-retinoid-pyridinium-ethanolamine (A2E) Accumulation and the Maintenance of the Visual Cycle Are Independent of Atg7-mediated Autophagy in the Retinal Pigmented Epithelium.
Perusek L et al.
The Journal of Biological Chemistry, 2015
The severity of retinal pathology in homozygous Crb1rd8/rd8 mice is dependent on additional genetic factors.
Luhmann UF et al.
Human Molecular Genetics, 2015
Induction of the unfolded protein response by constitutive G-protein signaling in rod photoreceptor cells.
Wang T et al.
The Journal of Biological Chemistry, 2014
Light damage in Abca4 and Rpe65rd12 mice.
Wu L et al.
Investigative Ophthalmology & Visual Science, 2014
Photoreceptors in whirler mice show defective transducin translocation and are susceptible to short-term light/dark changes-induced degeneration.
Tian M et al.
Experimental Eye Research, 2014
Fatty acid transport protein 4 (FATP4) prevents light-induced degeneration of cone and rod photoreceptors by inhibiting RPE65 isomerase.
Li S et al.
The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 2013
Reduction of all-trans-retinal in vertebrate rod photoreceptors requires the combined action of RDH8 and RDH12.
Chen C et al.
The Journal of Biological Chemistry, 2012
Leukemia inhibitory factor coordinates the down-regulation of the visual cycle in the retina and retinal-pigmented epithelium.
Chucair-Elliott AJ et al.
The Journal of Biological Chemistry, 2012
The bisretinoids of retinal pigment epithelium.
Sparrow JR et al.
Progress in Retinal and Eye Research, 2012
174delG mutation in mouse MFRP causes photoreceptor degeneration and RPE atrophy.
Fogerty J et al.
Investigative Ophthalmology & Visual Science, 2011
Moderate light-induced degeneration of rod photoreceptors with delayed transducin translocation in shaker1 mice.
Peng YW et al.
Investigative Ophthalmology & Visual Science, 2011
Interpretations of fundus autofluorescence from studies of the bisretinoids of the retina.
Sparrow JR et al.
Investigative Ophthalmology & Visual Science, 2010
Inner retina remodeling in a mouse model of stargardt-like macular dystrophy (STGD3).
Kuny S et al.
Investigative Ophthalmology & Visual Science, 2010
Photoreceptor cell degeneration in Abcr (-/-) mice.
Wu L et al.
Advances in Experimental Medicine and Biology, 2010
Rdh12 activity and effects on retinoid processing in the murine retina.
Chrispell JD et al.
The Journal of Biological Chemistry, 2009
Novel lipofuscin bisretinoids prominent in human retina and in a model of recessive Stargardt disease.
Wu Y et al.
The Journal of Biological Chemistry, 2009
The severity of retinal degeneration in Rp1h gene-targeted mice is dependent on genetic background.
Liu Q et al.
Investigative Ophthalmology & Visual Science, 2009
A comprehensive clinical and biochemical functional study of a novel RPE65 hypomorphic mutation.
Lorenz B et al.
Investigative Ophthalmology & Visual Science, 2008
The genomic response of the retinal pigment epithelium to light damage and retinal detachment.
Rattner A et al.
The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 2008
Impact of retinal disease-associated RPE65 mutations on retinoid isomerization.
Bereta G et al.
Biochemistry, 2008
R91W mutation in Rpe65 leads to milder early-onset retinal dystrophy due to the generation of low levels of 11-cis-retinal.
Samardzija M et al.
Human Molecular Genetics, 2008
SOD2 knockdown mouse model of early AMD.
Justilien V et al.
Investigative Ophthalmology & Visual Science, 2007
Targeted disruption of the murine retinal dehydrogenase gene Rdh12 does not limit visual cycle function.
Kurth I et al.
Molecular and Cellular Biology, 2007