Research Topics
Genomes and Genes | T Michael RedmondSummaryAffiliation: National Institutes of Health Country: USA Publications
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Publications
Expression and promoter activation of the Rpe65 gene in retinal pigment epithelium cell linesAna Boulanger
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA
Curr Eye Res 24:368-75. 2002..This limits the study of the transcriptional regulation of the mouse Rpe65 gene in vitro to this particular cell line...
Mole quantity of RPE65 and its productivity in the generation of 11-cis-retinal from retinyl esters in the living mouse eyeArkady L Lyubarsky
F M Kirby Center for Molecular Ophthalmology, Department of Ophthalmology, School of Medicine, University of Pennsylvania, 422 Curie Boulevard, Philadelphia, Pennsylvania 19104 6069, USA
Biochemistry 44:9880-8. 2005....
RPE65, visual cycle retinol isomerase, is not inherently 11-cis-specific: support for a carbocation mechanism of retinol isomerizationT Michael Redmond
Laboratory of Retinal Cell and Molecular Biology, NEI, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 285:1919-27. 2010..Specific visual cycle selectivity for 11-cis isomers instead resides downstream, attributable to mass action by CRALBP, retinol dehydrogenase 5, and high affinity of opsin apoproteins for 11-cis-retinal...
Identification, expression, and substrate specificity of a mammalian beta-carotene 15,15'-dioxygenaseT M Redmond
Laboratory of Retinal Cell and Molecular Biology, NEI, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 276:6560-5. 2001..Identification of beta-CD in an accessible model organism will create new opportunities to study vitamin A metabolism...
Effect of Leu/Met variation at residue 450 on isomerase activity and protein expression of RPE65 and its modulation by variation at other residuesT Michael Redmond
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, NIH, Bethesda, MD 20892 0706, USA
Mol Vis 13:1813-21. 2007..We wished to determine how this variant affects RPE65 and if it is modulated by other rodent-specific variations...
Mutation of key residues of RPE65 abolishes its enzymatic role as isomerohydrolase in the visual cycleT Michael Redmond
Laboratory of Retinal Cell and Molecular Biology and Biological Imaging Core, National Eye Institute, National Institutes of Health, Bethesda, MD 20892 0706, USA
Proc Natl Acad Sci U S A 102:13658-63. 2005..These findings establish a catalytic role, in conjunction with lecithin:retinol acyltransferase, for RPE65 in synthesis of 11-cis-retinol, and its identity as the isomerohydrolase...
Rpe65 is necessary for production of 11-cis-vitamin A in the retinal visual cycleT M Redmond
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
Nat Genet 20:344-51. 1998..Disruption of the RPE-based metabolism of all-trans-retinyl esters to 11-cis-retinal thus appears to underlie the Rpe65-/- phenotype, although cone pigment regeneration may be dependent on a separate pathway...
Decreased expression of insulin-like growth factor binding protein-5 during N-(4-hydroxyphenyl)retinamide-induced neuronal differentiation of ARPE-19 human retinal pigment epithelial cells: regulation by CCAAT/enhancer-binding proteinWilliam Samuel
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892 0706, USA
J Cell Physiol 224:827-36. 2010..Thus, our results clearly demonstrate that the IGFBP5 expression is down-regulated during 4HPR-induced neuronal differentiation of human RPE cells through a MAPK signal transduction pathway involving C/EBPbeta...
Key role of conserved histidines in recombinant mouse beta-carotene 15,15'-monooxygenase-1 activityEugenia Poliakov
Laboratory of Retinal Cell and Molecular Biology, NEI, National Institutes of Health, Bethesda, MD 20892 0706, USA
J Biol Chem 280:29217-23. 2005..These data are discussed in the context of the predicted structure for the related eubacterial apocarotenal oxygenase...
Biochemical evidence for the tyrosine involvement in cationic intermediate stabilization in mouse beta-carotene 15, 15'-monooxygenaseEugenia Poliakov
National Eye Institute, NIH, Bethesda, MD 20892 0608, USA
BMC Biochem 10:31. 2009..Enzymatic activity was measured in vitro using His-tag purified proteins and in vivo in a beta-carotene-accumulating E. coli system...
MicroRNA expression in human retinal pigment epithelial (ARPE-19) cells: increased expression of microRNA-9 by N-(4-hydroxyphenyl)retinamideR Krishnan Kutty
Laboratory of Retinal Cell and Molecular Biology, Bldg 6, Room 112, National Eye Institute, National Institutes of Health, 6 Center Dr, MSC 0608, Bethesda, MD 20892, USA
Mol Vis 16:1475-86. 2010..The aim of the present study was to investigate the expression of miR-9 in ARPE-19 cells in response to 4HPR treatment, and to identify other miRNAs normally expressed in these cells...
Aromatic residues in the substrate cleft of RPE65 protein govern retinol isomerization and modulate its progressionPreethi Chander
Laboratory of Retinal Cell and Molecular Biology, NEI, National Institutes of Health, Bethesda, MD 20892, USA
J Biol Chem 287:30552-9. 2012..These findings provide insight into the mechanism of isomerization central to the visual cycle...
Expression of beta-carotene 15,15' monooxygenase in retina and RPE-choroidRizwan A Bhatti
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, 6 Center Drive, Building 6 Room 339, Bethesda, MD 20892, USA
Invest Ophthalmol Vis Sci 44:44-9. 2003..Given the low and variable expression of beta-CM in the retina and RPE, it can be concluded that beta-CM is not necessary for a conserved retina or RPE-specific function, but may be necessary for a species-specific function...
Differential regulation of microRNA-146a and microRNA-146b-5p in human retinal pigment epithelial cells by interleukin-1β, tumor necrosis factor-α, and interferon-γR Krishnan Kutty
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, MD
Mol Vis 19:737-50. 2013..The aim of the present study is to investigate the expression of miR-146a and miR-146b-5p in human RPE cells and their response to proinflammatory cytokines...
Identification of a KRAB-zinc finger protein binding to the Rpe65 gene promoterZhongjian Lu
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute NIH, 7 Memorial Drive, Bethesda, MD 20892, USA
Curr Eye Res 31:457-66. 2006..We wish to identify transcriptional factors involved in regulation binding to the proximal promoter region of the RPE65 gene that confers RPE-specific expression...
Aromatic lipophilic spin traps effectively inhibit RPE65 isomerohydrolase activityEugenia Poliakov
LRCMB, NEI, National Institutes of Health, Bethesda, Maryland 20892, United States
Biochemistry 50:6739-41. 2011..We found that the aromatic lipophilic spin traps such as N-tert-butyl-α-phenylnitrone (PBN), 2,2-dimethyl-4-phenyl-2H-imidazole-1-oxide (DMPIO), and nitrosobenzene (NB) strongly inhibit RPE65 isomerohydrolase activity in vitro...
The upstream region of the Rpe65 gene confers retinal pigment epithelium-specific expression in vivo and in vitro and contains critical octamer and E-box binding sitesA Boulanger
Laboratory of Retinal Cell and Molecular Biology, NEI, National Institutes of Health, Bethesda, Maryland 20892, USA
J Biol Chem 275:31274-82. 2000....
Mitogen-activated protein kinase pathway mediates N-(4-hydroxyphenyl)retinamide-induced neuronal differentiation in the ARPE-19 human retinal pigment epithelial cell lineWilliam Samuel
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, Bethesda, Maryland, USA
J Neurochem 106:591-602. 2008....
Identification of beta-carotene 15, 15'-monooxygenase as a peroxisome proliferator-activated receptor target geneAna Boulanger
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892 2740, USA
FASEB J 17:1304-6. 2003....
The gene for the retinal pigment epithelium-specific protein RPE65 is localized to human 1p31 and mouse 3C P Hamel
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892
Genomics 20:509-12. 1994..By the use of fluorescence in situ hybridization, this localization was refined to 1p31. The mouse and human loci for this potential candidate gene for hereditary retinal disease do not match those of any known disease in mouse or man...
Molecular cloning and expression of RPE65, a novel retinal pigment epithelium-specific microsomal protein that is post-transcriptionally regulated in vitroC P Hamel
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892
J Biol Chem 268:15751-7. 1993..Such cells, however, contained no immunodetectable RPE65. The possible structure of this RPE-specific protein and hypotheses for the absence of translation in vitro are discussed...
Sequence and structure of the mouse gene for RPE65A Boulanger
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, MD 20892 2740, USA
Mol Vis 7:283-7. 2001..To determine the genomic organization of the mouse gene for the retinal pigment epithelium (RPE) specific protein RPE65...
RPE65 is highly uveitogenic in ratsDon-Il Ham
Laboratory of Immunology, National Eye Institute, National Institutes of Health, 10 Center Drive, Building 10, Room 10N112, Bethesda, MD 20892-1857, USA
Invest Ophthalmol Vis Sci 43:2258-63. 2002..CONCLUSIONS: RPE65 is highly uveitogenic in rats, thus suggesting that this molecule could be involved in pathogenic autoimmunity in the human eye...
Inflammatory cytokines regulate microRNA-155 expression in human retinal pigment epithelial cells by activating JAK/STAT pathwayR Krishnan Kutty
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA
Biochem Biophys Res Commun 402:390-5. 2010..All these activities were effectively blocked by JAK inhibitor 1. Our results show that the inflammatory cytokines increase miR-155 expression in human retinal pigment epithelial cells by activating the JAK/STAT signaling pathway...
Synthesis of an immunopathogenic fusion protein derived from a bovine interphotoreceptor retinoid-binding protein cDNA cloneT M Redmond
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, Bethesda, MD 20892
Gene 80:109-18. 1989..Thus, it is evident that biological activity of this region of IRBP, as manifested by immuno-pathogenicity, is retained by the fusion protein...
Origin and evolution of retinoid isomerization machinery in vertebrate visual cycle: hint from jawless vertebratesEugenia Poliakov
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, MD, USA
PLoS ONE 7:e49975. 2012..in the last common ancestor of the jawless and jawed vertebrates...
Cloning of cDNAs encoding human interphotoreceptor retinoid-binding protein (IRBP) and comparison with bovine IRBP sequencesJ S Si
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, Bethesda, MD 20892
Gene 80:99-108. 1989..Thus, the human sequence is virtually full length, is similar to the bovine sequence, and contains a striking fourfold repeat...
RPE65, the major retinal pigment epithelium microsomal membrane protein, associates with phospholipid liposomesE Tsilou
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
Arch Biochem Biophys 346:21-7. 1997..This RPE65-phospholipid liposome association may explain the solubilization characteristics of RPE65 and may be related to the function of RPE65 and to its physical association with the RPE smooth endoplasmic reticulum...
A developmentally regulated microsomal protein specific for the pigment epithelium of the vertebrate retinaC P Hamel
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, NIH, Bethesda, Maryland 20892
J Neurosci Res 34:414-25. 1993....
Interphotoreceptor retinoid-binding protein. Gene characterization, protein repeat structure, and its evolutionD E Borst
Laboratory of Retinal Cell and Molecular Biology, National Eye Institute, Bethesda, Maryland 20892
J Biol Chem 264:1115-23. 1989..The human IRBP gene has a sequence that is similar to one of the introns from the bovine gene. The unexpected gene structure and protein repeat structure in the bovine gene lead us to propose a model for the evolution of the IRBP gene...
Transgenic expression of an immunologically privileged retinal antigen extraocularly enhances self tolerance and abrogates susceptibility to autoimmune uveitisH Xu
NEI NIH, Bethesda, USA
Eur J Immunol 30:272-8. 2000..The same level of expression is, however, insufficient to tolerize wild-type effector T cells in the periphery...
Safety and efficacy of gene transfer for Leber's congenital amaurosisAlbert M Maguire
Scheie Eye Institute, University of Pennsylvania, USA
N Engl J Med 358:2240-8. 2008..Although the follow-up was very short and normal vision was not achieved, this study provides the basis for further gene therapy studies in patients with LCA...
Correlation of regenerable opsin with rod ERG signal in Rpe65-/- mice during development and agingBaerbel Rohrer
Department of Ophthalmology Medical University of South Carolina, 167 Ashley Avenue, Charleston, SC 29425, USA
Invest Ophthalmol Vis Sci 44:310-5. 2003..This project was designed to determine the amount of regenerable opsin in Rpe65-/- mice during development and aging, and to examine the function of this rhodopsin by electroretinography (ERG)...
11-cis-retinal reduces constitutive opsin phosphorylation and improves quantum catch in retinoid-deficient mouse rod photoreceptorsZsolt Ablonczy
Department of Ophthalmology, Medical University of South Carolina, 167 Ashley Avenue, Charleston, SC 29425, USA
J Biol Chem 277:40491-8. 2002..Our results indicate that opsin, which has not been exposed to 11-cis-retinal, does not generate the activity generally associated with the bleached apoprotein...
Impairment of the transient pupillary light reflex in Rpe65(-/-) mice and humans with leber congenital amaurosisTomas S Aleman
Department of Ophthalmology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
Invest Ophthalmol Vis Sci 45:1259-71. 2004..To determine the impairment of the transient pupillary light reflex (TPLR) due to severe retinal dysfunction and degeneration in a murine model of Leber congenital amaurosis (LCA) and in patients with the disease...
Retinyl esters are the substrate for isomerohydrolaseGennadiy Moiseyev
Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina 29425, USA
Biochemistry 42:2229-38. 2003..g., at 36 weeks, the levels are 20x that of WT). Our data indicate that the retinyl esters are the substrate of the isomerization reaction...
Functional and structural recovery of the retina after gene therapy in the RPE65 null mutation dogKristina Narfstrom
Vision Science Group, Department of Veterinary Medicine and Surgery, College of Veterinary Medicine, University of Missouri Columbia, Columbia, Missouri 65211, USA
Invest Ophthalmol Vis Sci 44:1663-72. 2003..To assess the efficacy of AAV-mediated gene therapy to restore vision in a large number of RPE65(-/-) dogs and to determine whether systemic and local side effects are caused by the treatment...
Assessment of rAAV-mediated gene therapy in the Rpe65-/- mouseP Elizabeth Rakoczy
Centre for Ophthalmology and Visual Science, University of Western Australia, Perth, Western Australia, Australia
Adv Exp Med Biol 533:431-8. 2003
In utero gene therapy rescues vision in a murine model of congenital blindnessNadine S Dejneka
F.M. Kirby Center and Scheie Eye Institute, Department of Ophthalmology, University of Pennsylvania, 51 N. 39th Street, Philadelphia, PA 19104-2689, USA
Mol Ther 9:182-8. 2004..The results demonstrate AAV-mediated correction of the deficit and suggest that in utero retinal gene delivery may be a useful approach for treating a variety of blinding congenital retinal diseases...
Acute radiolabeling of retinoids in eye tissues of normal and rpe65-deficient miceNasser M Qtaishat
Lions of Illinois Eye Research Institute, Department of Ophthalmology and Visual Sciences, College of Medicine, University of Illinois at Chicago, Chicago, Illinois 60612, USA
Invest Ophthalmol Vis Sci 44:1435-46. 2003..The present study was undertaken to determine whether the Rpe65-deficient mouse exhibits an abnormal flux of retinoid between the systemic circulation and the eye tissues...
Cone opsin mislocalization in Rpe65-/- mice: a defect that can be corrected by 11-cis retinalBaerbel Rohrer
Department of Ophthalmology, Medical University of South Carolina, Charleston, 29425, USA
Invest Ophthalmol Vis Sci 46:3876-82. 2005..This study was designed to investigate the feasibility of restoring functional cones with exogenous 11-cis retinal...
RPE65 is an iron(II)-dependent isomerohydrolase in the retinoid visual cycleGennadiy Moiseyev
Department of Cell Biology, Department of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, USA
J Biol Chem 281:2835-40. 2006..Inductively coupled plasma mass spectrometry measurements showed that bovine RPE65 binds iron ion with a stoichiometry of 0.8 +/- 0.1. These results indicate that RPE65 is an iron-dependent isomerohydrolase in the visual cycle...
9-cis Retinal increased in retina of RPE65 knockout mice with decrease in coat pigmentationJie Fan
Department of Ophthalmology, Medical University of South Carolina, Charleston, SC, USA
Photochem Photobiol 82:1461-7. 2006..Therefore, photoreceptor damage correlates with the amount of the apoprotein present, supporting findings that the activity from unregenerated opsin can lead to photoreceptor degeneration...
Spontaneous activity of opsin apoprotein is a cause of Leber congenital amaurosisMichael L Woodruff
Department of Physiological Science, University of California Los Angeles, Los Angeles, California 90095, USA
Nat Genet 35:158-64. 2003..A similar mechanism may also be responsible for degeneration induced by vitamin A deprivation...
Assessing the efficacy of gene therapy in Rpe65-/- mice using photoentrainment of circadian rhythmChris W Stoddart
Centre for Ophthalmology and Visual Science, The University of Western Australia, Perth, Western Australia
Adv Exp Med Biol 572:239-45. 2006
Functional and structural evaluation after AAV.RPE65 gene transfer in the canine model of Leber's congenital amaurosisKristina Narfstrom
University of Missouri Columbia, MO, USA
Adv Exp Med Biol 533:423-30. 2003
