Genomes and Genes
Gene Symbol: Pde6b
Description: phosphodiesterase 6B, cGMP, rod receptor, beta polypeptide
Alias: Pdeb, rd-1, rd1, rd10, rod cGMP-specific 3',5'-cyclic phosphodiesterase subunit beta, GMP-PDE beta, cGMP-phosphodiesterase beta-subunit, phosphodiesterase, cGMP, rod receptor, beta polypeptide, retinal degeneration 1
Publications135 found, 100 shown here
- Identification of a nonsense mutation in the rod photoreceptor cGMP phosphodiesterase beta-subunit gene of the rd mouseS J Pittler
Department of Ophthalmology, Baylor College of Medicine, Houston, TX 77030
Proc Natl Acad Sci U S A 88:8322-6. 1991..The results strongly argue for the nonsense mutation being responsible for retinal degeneration in the rd mouse...
- Localization of a retroviral element within the rd gene coding for the beta subunit of cGMP phosphodiesteraseC Bowes
Jules Stein Eye Institute, University of California School of Medicine, Los Angeles 90024
Proc Natl Acad Sci U S A 90:2955-9. 1993..Reverse transcription-PCR demonstrates that the integrated Xmv-28 sequences are transcribed in the retina. The provirus is present in every strain of rd mouse tested...
- Mouse P-selectin glycoprotein ligand-1: molecular cloning, chromosomal localization, and expression of a functional P-selectin receptorJ Yang
Center for Hemostasis and Thrombosis Research, Division of Hematology Oncology, New England Medical Center, Boston, MA, USA
Blood 87:4176-86. 1996..PSGL-1 on mouse myeloid cells is critical for high-affinity binding to P-selectin but not E-selectin...
- The clock in the mouse retina: melatonin synthesis and photoreceptor degenerationG Tosini
Department of Biology and NSF Center for Biological Timing, University of Virginia, Charlottesville, VA 22903, USA
Brain Res 789:221-8. 1998..The many genetic and molecular tools available in the mouse can now be applied to analysis of the retinal circadian oscillator...
- Thresholds for masking responses to light in three strains of retinally degenerate miceN Mrosovsky
Department of Zoology, University of Toronto, Ontario, Canada
J Comp Physiol A 184:423-8. 1999..With very dim light pulses, mutants did not change their activity, but wildtypes actually became more active (positive masking). Positive and negative masking appear to depend on different sensory and central processes...
- Stimulation of the insulin/mTOR pathway delays cone death in a mouse model of retinitis pigmentosaClaudio Punzo
Harvard Medical School, Department of Genetics and Howard Hughes Medical Institute, 77 Avenue Louis Pasteur, Boston, Massachusetts 02115, USA
Nat Neurosci 12:44-52. 2009..These data suggest that the non-autonomous cone death in retinitis pigmentosa could, at least in part, be a result of the starvation of cones...
- Retinal organization in the retinal degeneration 10 (rd10) mutant mouse: a morphological and ERG studyClaudia Gargini
Dipartimento di Psichiatria, Neurobiologia, Farmacologia e Biotecnologie, Universita di Pisa, 56100 Pisa, Italy
J Comp Neurol 500:222-38. 2007Retinal degeneration 10 (rd10) mice are a model of autosomal recessive retinitis pigmentosa (RP), identified by Chang et al. in 2002 (Vision Res. 42:517-525)...
- A simple polymerase chain reaction assay for genotyping the retinal degeneration mutation (Pdeb(rd1)) in FVB/N-derived transgenic miceE Gimenez
Departamento de Biologia Molecular y Celular, Centro Nacional de Biotecnologia, Madrid, Spain
Lab Anim 35:153-6. 2001..However, these inbred mice carry a retinal degeneration mutation caused by a proviral insertion into the Pdeb gene, encoding the beta subunit of cGMP phosphodiesterase...
- Morphological and functional abnormalities in the inner retina of the rd/rd mouseEnrica Strettoi
Istituto di Neurofisiologia del Consiglio Nazionale delle Ricerche, 56100 Pisa, Italy
J Neurosci 22:5492-504. 2002..This is even more important when considering that although intervention can be performed before the onset of any symptoms in animals carrying inherited retinopathies, this is obviously not true for human subjects...
- shRNA knockdown of guanylate cyclase 2e or cyclic nucleotide gated channel alpha 1 increases photoreceptor survival in a cGMP phosphodiesterase mouse model of retinitis pigmentosaJoaquin Tosi
Bernard and Shirlee Brown Glaucoma Laboratory, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York, NY, USA
J Cell Mol Med 15:1778-87. 2011..In Pde6b mouse models of retinitis pigmentosa (RP), photoreceptor death is preceded by abnormally high cGMP and Ca(2+) ..
- Proliferation of microglia, but not photoreceptors, in the outer nuclear layer of the rd-1 mouseCaroline J Zeiss
Section of Comparative Medicine, Yale School of Medicine, New Haven, Connecticut 06520, USA
Invest Ophthalmol Vis Sci 45:971-6. 2004..Studies of cell cycle proteins in other models of neuronal death provide consistent evidence that a repertoire of proliferative markers accompanies apoptosis...
- Melanopsin (Opn4) requirement for normal light-induced circadian phase shiftingSatchidananda Panda
Genomics Institute of the Novartis Research Foundation, 10675 John J Hopkins Drive, San Diego, CA 92121, USA
Science 298:2213-6. 2002..However, they display severely attenuated phase resetting in response to brief pulses of monochromatic light, highlighting the critical role of melanopsin in circadian photoentrainment in mammals...
- Two mouse retinal degenerations caused by missense mutations in the beta-subunit of rod cGMP phosphodiesterase geneB Chang
The Jackson Laboratory, Bar Harbor, ME, USA
Vision Res 47:624-33. 2007..by a missense point mutation in exon 13 of the beta-subunit of the rod cGMP phosphodiesterase (beta-PDE) gene (Pde6b). The gene symbol for this strain was set as Pde6brd10, abbreviated rd10 hereafter...
- MutT homolog-1 attenuates oxidative DNA damage and delays photoreceptor cell death in inherited retinal degenerationYusuke Murakami
Department of Ophthalmology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
Am J Pathol 181:1378-86. 2012..Here, we show that oxidation of nucleic acids is a key component in the initiation of death-signaling pathways in rd10 mice, a model of RP...
- Response properties of ganglion cells in the isolated mouse retinaC Stone
Department of Neurobiology and Physiology, Northwestern University, Evanston, IL 60208
Vis Neurosci 10:31-9. 1993..This homogeneity would simplify the comparison of retinas from normal and mutant strains of the mouse...
- Physiologic diversity and development of intrinsically photosensitive retinal ganglion cellsDaniel C Tu
Department of Ophthalmology and Visual Sciences, Washington University Medical School, St Louis, Missouri 63110, USA
Neuron 48:987-99. 2005..These results demonstrate that ipRGCs are the first light-sensitive cells in the retina and suggest previously unappreciated diversity in this cell population...
- Caspase-1 ablation protects photoreceptors in a model of autosomal dominant retinitis pigmentosaMarijana Samardzija
Laboratory for Retinal Cell Biology, Department of Ophthalmology, Center for Integrative Human Physiology CIHP, Neuroscience Center Zürich ZNZ, University of Zurich, Zurich, Switzerland
Invest Ophthalmol Vis Sci 47:5181-90. 2006..Thus, caspase-1 may be proapoptotic in the retina. To test directly the role of caspase-1 in photoreceptor apoptosis, three mouse models were analyzed for retinal degeneration in the presence or absence of caspase-1...
- Photon capture and signalling by melanopsin retinal ganglion cellsMichael Tri H Do
Solomon H Snyder Department of Neuroscience, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
Nature 457:281-7. 2009..Notably, like rods, these cells are capable of signalling single-photon absorption. A flash causing a few hundred isomerized melanopsin molecules in a retina is sufficient for reaching threshold for the pupillary light reflex...
- Circadian photoreception in the retinally degenerate mouse (rd/rd)R G Foster
Department of Biology, University of Virginia, Charlottesville 22901
J Comp Physiol A 169:39-50. 1991..We have yet to determine whether the opsin immunoreactive perikarya or some other unidentified cell type mediate circadian light detection in the rd/rd retina...
- Structural and functional studies of the early T lymphocyte activation 1 (Eta-1) gene. Definition of a novel T cell-dependent response associated with genetic resistance to bacterial infectionR Patarca
Laboratory of Immunopathology, Dana Farber Cancer Institute, Boston, Massachusetts
J Exp Med 170:145-61. 1989..Eta-1 gene expression appears to be part of a surprisingly rapid T cell-dependent response to bacterial infection that may precede classical forms of T cell-dependent immunity...
- Cyclic guanosine monophosphate: elevation in degenerating photoreceptor cells of the C3H mouse retinaD B Farber
Science 186:449-51. 1974..It is suggested that degeneration of the photoreceptor cells is related to an imbalance in their metabolism or function which is caused by the elevated levels of cyclic guanosine monophosphate...
- Mutation spectrum of the gene encoding the beta subunit of rod phosphodiesterase among patients with autosomal recessive retinitis pigmentosaM E McLaughlin
Howe Laboratory of Ophthalmology, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston 02114, USA
Proc Natl Acad Sci U S A 92:3249-53. 1995..Mutations in the gene encoding the beta subunit of rod phosphodiesterase are the most common identified cause of autosomal recessive retinitis pigmentosa, accounting for approximately 4% of cases in North America...
- Recessive mutations in the gene encoding the beta-subunit of rod phosphodiesterase in patients with retinitis pigmentosaM E McLaughlin
Berman Gund Laboratory, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston 02114
Nat Genet 4:130-4. 1993..PDE beta is the second member of the phototransduction cascade besides rhodopsin that is absent or altered as a cause of retinitis pigmentosa, suggesting that other members of this pathway may be defective in other forms of this disease...
- Regulation of the mammalian pineal by non-rod, non-cone, ocular photoreceptorsR J Lucas
Department of Biology, Sir Alexander Fleming Building, Imperial College of Science, Technology and Medicine, London, SW7 2AZ, UK
Science 284:505-7. 1999..These data indicate that mammals have additional ocular photoreceptors that they use in the regulation of temporal physiology...
- Contribution of classic photoreceptors to entrainmentN Mrosovsky
Department of Zoology, University of Toronto, 25 Harbord Street, Toronto, ON, M5S 3G5, Canada
J Comp Physiol A Neuroethol Sens Neural Behav Physiol 189:69-73. 2003..Thresholds for entrainment appear to be a more sensitive test of deficits in entrainment than phase shifts in response to light pulses...
- Melanopsin is required for non-image-forming photic responses in blind miceSatchidananda Panda
Genomics Institute of the Novartis Research Foundation, 10675 John J Hopkins Drive, San Diego, CA 92121, USA
Science 301:525-7. 2003..This indicates the importance of both nonvisual and classical visual photoreceptor systems for nonvisual photic responses in mammals...
- Light-induced c-fos in melanopsin retinal ganglion cells of young and aged rodless/coneless (rd/rd cl) miceMa ayan Semo
Department of Integrative and Molecular Neuroscience, Imperial College London, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK
Eur J Neurosci 18:3007-17. 2003..Collectively, our data suggest that melanopsin RGCs form a heterogeneous population of neurons, and that most of the light-induced c-fos expression within these cells is associated with the endogenous photosensitivity of these neurons...
- Studies of visual function and its decay in mice with hereditary retinal degenerationU C Drager
J Comp Neurol 180:85-114. 1978..This is consistent with morphological changes known to occur in the retina as a consequence, of the rd mutation the rods degenerating before the cones...
- Differential effect of the rd mutation on rods and cones in the mouse retinaL D Carter-Dawson
Invest Ophthalmol Vis Sci 17:489-98. 1978..About 5% of the cone nuclei are still present in the far periphery at 18 months of age...
- Dopamine and its agonists reduce a light-sensitive pool of cyclic AMP in mouse photoreceptorsA I Cohen
Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, MO 63110
Vis Neurosci 4:43-52. 1990..The D1 antagonist SCH23390 only reduced cyclic AMP levels of DKA-DI retinas when cyclic AMP levels had been elevated by adding dopamine to the incubation medium...
- Retinal degeneration in the rd mouse is caused by a defect in the beta subunit of rod cGMP-phosphodiesteraseC Bowes
Jules Stein Eye Institute, University of California, School of Medicine, Los Angeles 90024
Nature 347:677-80. 1990..Here we present evidence that the candidate cDNA is the murine homologue of bovine phosphodiesterase beta cDNA. We conclude that the mouse rd locus encodes the rod photoreceptor cGMP-phosphodiesterase beta subunit...
- The persistence of cone photoreceptors within the dorsal retina of aged retinally degenerate mice (rd/rd): implications for circadian organizationJ M Garcia-Fernandez
Departamento de Morfologia y Biologia Celular, Universidad de Oviedo, Spain
Neurosci Lett 187:33-6. 1995..As rd mice show unattenuated circadian responses to light, these remaining photoreceptor cells within the dorsal retina become candidates for the regulation of circadian physiology by light...
- Apoptotic photoreceptor cell death in mouse models of retinitis pigmentosaC Portera-Cailliau
Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, MD 21287 9257
Proc Natl Acad Sci U S A 91:974-8. 1994..These findings raise the possibility that retinal degenerations may be slowed by interfering with the apoptotic mechanism itself...
- Photoreceptor cell rescue in retinal degeneration (rd) mice by in vivo gene therapyJ Bennett
Department of Ophthalmology, F M Kirby Center for Molecular Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia 19104, USA
Nat Med 2:649-54. 1996..The findings demonstrate cell rescue by in vivo gene transfer, thus supporting the feasibility of treating an inherited retinal degeneration by somatic gene therapy...
- Overexpression of Bcl-2 or Bcl-XL transgenes and photoreceptor degenerationR M Joseph
Berman Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston 02114, USA
Invest Ophthalmol Vis Sci 37:2434-46. 1996..To test the hypothesis that overexpression of genes coding for the anti-apoptotic proteins Bcl-2 or Bcl-XL in photoreceptor cells may prevent or delay photoreceptor degenerations...
- Spatial responses to light in mice with severe retinal degenerationN Mrosovsky
Department of Zoology, University of Toronto, Ontario, Canada
Neurosci Lett 222:204-6. 1997..This was true of mutant mice over a year old, when retinal degeneration is very severe, and also of a transgenic strain of mice whose rods are destroyed as they begin to develop in the first few weeks after birth...
- Variability in rate of cone degeneration in the retinal degeneration (rd/rd) mouseM M LaVail
Department of Anatomy, University of California, San Francisco, CA, 94143 0730, USA
Exp Eye Res 65:45-50. 1997....
- Fos expression in the retina of rd/rd mice during the light/dark cycleJ J Huerta
Departamento de Morfologia y Biologia Celular, Universidad de Oviedo, Spain
Neurosci Lett 232:143-6. 1997..Aged rd animals also show Fos expression in GCL and INL in response to light stimuli suggesting that severely degenerate retinas are still able to transduce light stimulus...
- Protection of mouse photoreceptors by survival factors in retinal degenerationsM M LaVail
Department of Anatomy, University of California, San Francisco 94143 0730, USA
Invest Ophthalmol Vis Sci 39:592-602. 1998....
- Retinal degeneration in the rd mouse in the absence of c-fosF Hafezi
Department of Ophthalmology, University Hospital, Zurich, Switzerland
Invest Ophthalmol Vis Sci 39:2239-44. 1998..To determine whether c-Fos is also involved in the apoptotic pathway of inherited retinal degeneration, rd/rd, c-fos -/- double-mutant mice have been generated...
- Rescue from photoreceptor degeneration in the rd mouse by human immunodeficiency virus vector-mediated gene transferM Takahashi
Laboratory of Genetics, The Salk Institute for Biological Studies, La Jolla, California 92037, USA
J Virol 73:7812-6. 1999..Mutations in the rod photoreceptor cGMP phosphodiesterase beta subunit (PDEbeta) gene are found in patients with autosomal recessive RP as well as in the rd mouse...
- Glial cell line-derived neurotrophic factor induces histologic and functional protection of rod photoreceptors in the rd/rd mouseM Frasson
Laboratoire de Physiopathologie Retinienne, Clinique Ophthalmologique, Universite Louis Pasteur, Centre Hospitalier Regional Universitaire, Strasbourg, France
Invest Ophthalmol Vis Sci 40:2724-34. 1999..To evaluate the neuroprotective potential of glial cell line-derived neurotrophic factor (GDNF) in the retinal degeneration (rd/rd) mouse model of human retinitis pigmentosa...
- Growth factors in combination, but not individually, rescue rd mouse photoreceptors in organ cultureJ M Ogilvie
Fay and Carl Simons Center for Biology of Hearing and Deafness, Central Institute for the Deaf, St Louis, Missouri, 63110, USA
Exp Neurol 161:676-85. 2000..Increased understanding of receptor interactions and signaling pathways may lead to a potential therapeutic role for combinatorial trophic factors in treatment of photoreceptor dystrophies...
- Modifications of retinal neurons in a mouse model of retinitis pigmentosaE Strettoi
Istituto di Neurofisiologia del Consiglio Nazionale delle Ricerche, Area della Ricerca Consiglio Nazionale delle Ricerche, Via San Cataldo 1, 56100 Pisa, Italy
Proc Natl Acad Sci U S A 97:11020-5. 2000..While describing modifications of the rd retina, we also provide quantitative information about neurons of the wild-type mouse retina, useful for future studies on genetically altered animals...
- Temporal and spatial expression patterns of the CRX transcription factor and its downstream targets. Critical differences during human and mouse eye developmentL C Bibb
Section of Cell and Molecular Biology, Imperial College School of Medicine, Sir Alexander Fleming Building, Exhibition Road, London SW7 2AZ, UK
Hum Mol Genet 10:1571-9. 2001..c., implying that it would be unable to transactivate PDEB, IRBP and arrestin, which were all expressed before 15 weeks...
- Neurotrophic signaling in normal and degenerating rodent retinasK J Wahlin
Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, MD 21287 9277, USA
Exp Eye Res 73:693-701. 2001..These data add to the mounting evidence suggesting that neurotrophic factors act indirectly through Müller cells to promote photoreceptor survival...
- Retinal degeneration mutants in the mouseB Chang
The Jackson Laboratory, 600 Main Street, Bar Harbor, ME, USA
Vision Res 42:517-25. 2002..other retinal cell types have been found: retinal degeneration (formerly rd, identical with rodless retina, r, now Pde6b(rd1)); Purkinje cell degeneration (pcd); nervous (nr); retinal degeneration slow (rds, now Prph(Rd2)); retinal ..
- Novel ENU-induced eye mutations in the mouse: models for human eye diseaseCaroline Thaung
Comparative and Developmental Genetics Section, MRC Human Genetics Unit, Edinburgh EH4 2XU, UK
Hum Mol Genet 11:755-67. 2002..are mutations in genes previously identified to play a role in eye pathophysiology, namely Pax6, Mitf, Egfr and Pde6b. Many of the others are located in genomic regions lacking candidate genes and these define new loci...
- Remodeling of second-order neurons in the retina of rd/rd mutant miceEnrica Strettoi
Laboratorio di Neurofisiologia, Istituto di Neuroscienze del CNR, Area della Ricerca, Via G Moruzzi 1, 56100, Pisa, Italy
Vision Res 43:867-77. 2003..B-wave abnormalities indicate specific postreceptoral dysfunction. Morphological and ERG changes in rd/rd retinas are consistent with substantial inner retinal remodeling associated to photoreceptor degeneration...
- Caspase-independent photoreceptor apoptosis in mouse models of retinal degenerationFrancesca Doonan
Tumour Biology Laboratory, Biochemistry Department, Bioscience Research Institute, University College Cork, Cork, Ireland
J Neurosci 23:5723-31. 2003....
- Retinal remodeling triggered by photoreceptor degenerationsBryan W Jones
John A Moran Eye Center, University of Utah School of Medicine, Salt Lake City, Utah 84132
J Comp Neurol 464:1-16. 2003..Although remodeling may constrain therapeutic intervals for molecular, cellular, or bionic rescue, it suggests that the neural retina may be more plastic than previously believed...
- Calpain is activated in degenerating photoreceptors in the rd1 mouseFrancois Paquet-Durand
Ophthalmology Department, University of Lund, Lund, Sweden
J Neurochem 96:802-14. 2006..cyclic AMP response element-binding protein (CREB)-1, calpastatin and of various calpain genes were analysed in the rd1 mouse compared with its wild-type control...
- Up-regulation and increased phosphorylation of protein kinase C (PKC) delta, mu and theta in the degenerating rd1 mouse retinaSeifollah Azadi
Department of Ophthalmology, Lund University, BMC B13, SE 221 84 Lund, Sweden
Mol Cell Neurosci 31:759-73. 2006The rd1 mouse serves as a model for inherited photoreceptor degeneration: retinitis pigmentosa...
- Multiple, parallel cellular suicide mechanisms participate in photoreceptor cell deathHeather R Lohr
Department of Neurosciences, Division of Research, Medical University of South Carolina, 173 Ashley Avenue, BSB403, Charleston, SC 29425, USA
Exp Eye Res 83:380-9. 2006..Thus, unless the common upstream initiator for a given photoreceptor dystrophy is found, multiple rescue paradigms need to be used to target all active pathways...
- Classical complement activation and acquired immune response pathways are not essential for retinal degeneration in the rd1 mouseBarbel Rohrer
Department of Ophthalmology, Medical University of South Carolina, Charleston, SC 29425, USA
Exp Eye Res 84:82-91. 2007..In addition to this differential regulation of individual genes, we found that in the rd1 retina a significantly higher number of genes involved in immune-related responses were expressed at any given time ..
- Tool from ancient pharmacopoeia prevents vision lossJeffrey H Boatright
Department of Ophthalmology, Emory University School of Medicine, Atlanta, GA 30322, USA
Mol Vis 12:1706-14. 2006..The purpose of this study was to test whether treatment of mice undergoing retinal degeneration with tauroursodeoxycholic acid (TUDCA), a primary constituent of bear bile, alters the course of degeneration...
- Crx activates opsin transcription by recruiting HAT-containing co-activators and promoting histone acetylationGuang Hua Peng
Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, MO 63110, USA
Hum Mol Genet 16:2433-52. 2007....
- Classical and melanopsin photoreception in irradiance detection: negative masking of locomotor activity by lightStewart Thompson
Howard Hughes Medical Institute, Department of Ophthalmology and Visual Sciences, University of Iowa, Iowa City, Iowa 52242, USA
Eur J Neurosci 27:1973-9. 2008..Together the evidence demonstrates a pronounced and sustained classical photoreceptor input to irradiance detection for negative masking, and suggests one role of classical photoreceptor input is to constrain dynamic range...
- Inducible ablation of melanopsin-expressing retinal ganglion cells reveals their central role in non-image forming visual responsesMegumi Hatori
The Salk Institute for Biological Studies, La Jolla, California, United States of America
PLoS ONE 3:e2451. 2008....
- AAV-mediated gene therapy for retinal degeneration in the rd10 mouse containing a recessive PDEbeta mutationJi Jing Pang
Departments of Ophthalmology, University of Florida, Gainesville, Florida 32610, USA
Invest Ophthalmol Vis Sci 49:4278-83. 2008To test AAV-mediated gene therapy in the rd10 mouse, a natural model of recessive RP caused by mutation of the beta-subunit of rod photoreceptor cGMP phosphodiesterase.
- Blockade of neuronal nitric oxide synthase reduces cone cell death in a model of retinitis pigmentosaKeiichi Komeima
Departments of Ophthalmology and Neuroscience, Maumenee 719, The Johns Hopkins University School of Medicine, 600 N Wolfe Street, Baltimore, MD 21287 9277, USA
Free Radic Biol Med 45:905-12. 2008..In this study, we have shown that peroxynitrite-induced nitrosative damage also occurs. In the rd1 mouse model of RP, there was increased staining for S-nitrosocysteine and nitrotyrosine protein adducts that are ..
- Functional and structural modifications during retinal degeneration in the rd10 mouseR Barhoum
Departamento de Fisiologia, Universidad de Alcala, Alcala de Henares 28871, Spain
Neuroscience 155:698-713. 2008..We have studied degeneration in the rd10 mice both by immunocytochemistry and TUNEL-labeling of retinal cells, and through electrophysiological recordings...
- Functional rescue of degenerating photoreceptors in mice homozygous for a hypomorphic cGMP phosphodiesterase 6 b allele (Pde6bH620Q)Richard J Davis
Department of Pathology and Cell Biology, Brown Glaucoma Laboratory, College of Physicians and Surgeons, Columbia University, New York 10032, USA
Invest Ophthalmol Vis Sci 49:5067-76. 2008..In mice homozygous for a nonsense Pde6b(rd1) allele, absence of PDE6 activity is associated with retinal disease similar to humans...
- Restoration of visual function in retinal degeneration mice by ectopic expression of melanopsinBin Lin
Massachusetts General Hospital, Harvard Medical School, Thier 429, 50 Blossom Street, Boston, MA 02114, USA
Proc Natl Acad Sci U S A 105:16009-14. 2008..For therapy in human photodegenerations, channelrhodopsin-2 and melanopsin have different advantages and disadvantages; both proteins (or modifications of them) should be candidates...
- Alteration in iron metabolism during retinal degeneration in rd10 mouseEfrat Deleon
Department of Ophthalmology, Hadassah Hebrew University Medical Center, Jerusalem, Israel
Invest Ophthalmol Vis Sci 50:1360-5. 2009..Altered iron metabolism was implicated in retinal and macular degeneration. This study was designed to further elucidate iron homeostasis during the course of retinal degeneration in mice...
- Remodeling of cone photoreceptor cells after rod degeneration in rd miceBin Lin
Harvard Medical School, Massachusetts General Hospital, Boston, MA 02114, USA
Exp Eye Res 88:589-99. 2009..Cone photoreceptors in retinas of human RP donors appear from their morphology to undergo a similar progression. The therapeutic window for rescue of cone photoreceptors may be longer than would have been thought...
- PKG activity causes photoreceptor cell death in two retinitis pigmentosa modelsFrancois Paquet-Durand
University of Tubingen, Centre for Ophthalmology, Institute for Ophthalmic Research, Division of Experimental Ophthalmology, Röntgenweg 11, Tubingen, Germany
J Neurochem 108:796-810. 2009..Here, we show that activation of cGMP-dependent protein kinase (PKG) hallmarks photoreceptor degeneration in rd1 and rd2 human homologous mouse models...
- Differential loss and preservation of glutamate receptor function in bipolar cells in the rd10 mouse model of retinitis pigmentosaTheresa Puthussery
Casey Eye Institute, Department of Ophthalmology, Oregon Health and Science University, Portland, OR, USA
Eur J Neurosci 29:1533-42. 2009..and electron microscopy to correlate physiological with anatomical changes in bipolar cells of the rd10 mouse - a model of autosomal recessive retinitis pigmentosa...
- Visual function in mice with photoreceptor degeneration and transgenic expression of channelrhodopsin 2 in ganglion cellsSenthil Thyagarajan
Department of Neuroanatomy, Max Planck Institute for Brain Research, D 60528 Frankfurt Main, Germany
J Neurosci 30:8745-58. 2010..a transgenic mouse line expressing channelrhodopsin2 (ChR2) under the control of the Thy1 promoter with the Pde6b(rd1) mouse, a model for retinal degeneration (rd1/rd1)...
- Inhibition of ceramide biosynthesis preserves photoreceptor structure and function in a mouse model of retinitis pigmentosaEnrica Strettoi
Neuroscience Institute, Consiglio Nazionale delle Ricerche, Pisa 56100, Italy
Proc Natl Acad Sci U S A 107:18706-11. 2010..Using the retinal degeneration 10 (rd10) mouse model of RP, we investigated the role of ceramide, a proapoptotic sphingolipid, in retinal degeneration...
- Evidence of severe mitochondrial oxidative stress and a protective effect of low oxygen in mouse models of inherited photoreceptor degenerationDafni Vlachantoni
MRC Human Genetics Unit, Institute of Genetics and Molecular Medicine, Edinburgh, UK
Hum Mol Genet 20:322-35. 2011..We investigated this question using four IPD mouse models (Pde6b(rd1/rd1), Pde6b(atrd1/atrd1), Rho(-/-) and Prph2(rds/rds)) and compared the abundance of reduced glutathione (GSH) ..
- A key role for cyclic nucleotide gated (CNG) channels in cGMP-related retinitis pigmentosaFrancois Paquet-Durand
Division of Experimental Ophthalmology, Institute for Ophthalmic Research, University of Tubingen, Röntgenweg 11, 72076 Tubingen, Germany
Hum Mol Genet 20:941-7. 2011The rd1 natural mutant is one of the first and probably the most commonly studied mouse model for retinitis pigmentosa (RP), a severe and frequently blinding human retinal degeneration...
- Melanopsin contributions to irradiance coding in the thalamo-cortical visual systemTimothy M Brown
Faculty of Life Sciences, University of Manchester, Manchester, United Kingdom
PLoS Biol 8:e1000558. 2010..These findings identify mRGCs as a potential origin for aspects of visual perception and indicate that they may support vision in people suffering retinal degeneration...
- AAV-mediated gene replacement, either alone or in combination with physical and pharmacological agents, results in partial and transient protection from photoreceptor degeneration associated with betaPDE deficiencyMariacarmela Allocca
Telethon Institute of Genetics and Medicine TIGEM, Naples, Italy
Invest Ophthalmol Vis Sci 52:5713-9. 2011Mutations in the PDE6B gene cause recessive, severe retinitis pigmentosa (RP)...
- An intrinsic neural oscillator in the degenerating mouse retinaJoanna Borowska
Department of Anatomy, Neuroscience Institute, Dalhousie University, Halifax, Nova Scotia B3H 1X5, Canada
J Neurosci 31:5000-12. 2011..to examine the physiological properties of morphologically identified retinal neurons in a mouse model of RD (rd1)...
- Profound defects in pupillary responses to light in TRPM-channel null mice: a role for TRPM channels in non-image-forming photoreceptionSteven Hughes
The Nuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neurosciences, University of Oxford, Level 5 6 West Wing, John Radcliffe Hospital, Headley Way, Oxford OX3 9DU, UK
Eur J Neurosci 35:34-43. 2012..Expression of TRPM3 is detected in Muller cells and the ciliary body but is absent from pRGCs, and thus our data support an indirect role for TRPM3 in pupillary light responses...
- Long-term characterization of retinal degeneration in rd1 and rd10 mice using spectral domain optical coherence tomographyMark E Pennesi
Casey Eye Institute, Oregon Health and Science University, Portland, OR 97239, USA
Invest Ophthalmol Vis Sci 53:4644-56. 2012..the in vivo changes over time in the retinal structure of wild-type mice alongside two lines of mice deficient in the β-subunit of phosphodiesterase (rd1 and rd10 mice) using spectral domain optical coherence tomography (SD-OCT).
- Melanopsin-based brightness discrimination in mice and humansTimothy M Brown
Faculty of Life Sciences, University of Manchester, Manchester M13 9PT, UK
Curr Biol 22:1134-41. 2012..These data represent the first direct evidence that an aspect of visual discrimination in normally sighted subjects can be supported by inner retinal photoreceptors...
- Environmental enrichment extends photoreceptor survival and visual function in a mouse model of retinitis pigmentosaIlaria Barone
Neuroscience Institute, Italian National Research Council CNR, Pisa, Italy
PLoS ONE 7:e50726. 2012..Here we report the results of prolonged exposure of rd10 mice, a mutant strain undergoing progressive photoreceptor degeneration mimicking human RP, to such an enriched ..
- Repair of the degenerate retina by photoreceptor transplantationAmanda C Barber
Department of Genetics, University College London Institute of Ophthalmology, London EC1V 9EL, United Kingdom
Proc Natl Acad Sci U S A 110:354-9. 2013..can increase (Prph2(+/Δ307)), decrease (Crb1(rd8/rd8), Gnat1(-/-), Rho(-/-)), or remain constant (PDE6β(rd1/rd1), Prph2(rd2/rd2)) with disease progression, depending upon the gene defect, with no correlation with severity...
- Functional integrity and modification of retinal dopaminergic neurons in the rd1 mutant mouse: roles of melanopsin and GABACameron L Atkinson
Eye Research Inst, Oakland Univ, 423 Dodge Hall, Rochester, MI 48309, USA
J Neurophysiol 109:1589-99. 2013..spontaneous and light-evoked spike activity of genetically labeled dopamine neurons from the retinas of retinal degeneration 1 (rd1) mice...
- Retinal ganglion cells are resistant to photoreceptor loss in retinal degenerationBin Lin
Department of Anatomy, The University of Hong Kong, Li Ka Shing Faculty of Medicine, Pokfulam, Hong Kong
PLoS ONE 8:e68084. 2013..The present study investigated the morphological changes in several identified RGCs in the retinal degeneration rd1 mouse model of retinitis pigmentosa (RP), using a combination of viral transfection, microinjection of neurobiotin ..
- Network oscillations drive correlated spiking of ON and OFF ganglion cells in the rd1 mouse model of retinal degenerationDavid J Margolis
Department of Cell Biology and Neuroscience, Rutgers, The State University of New Jersey, New Brunswick, New Jersey, United States of America
PLoS ONE 9:e86253. 2014....
- Suppression of microglial activation is neuroprotective in a mouse model of human retinitis pigmentosaBo Peng
Department of Anatomy
J Neurosci 34:8139-50. 2014..In this study, we explored the role of microglia as a contributor to photoreceptor degeneration in the rd10 mouse model of RP. First, we demonstrated that microglia activation was an early alteration in RP retinas...
- The postnatal development of the retina in the normal and rodless CBA mouse: a light and electron microscopic studyD W Caley
Am J Anat 133:179-212. 1972
- Melanopsin retinal ganglion cells and the maintenance of circadian and pupillary responses to light in aged rodless/coneless (rd/rd cl) miceMa ayan Semo
Department of Integrative and Molecular Neuroscience, Division of Neuroscience and Psychological Medicine, Faculty of Medicine, Imperial College London, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK
Eur J Neurosci 17:1793-801. 2003..These findings, together with recent studies on melanopsin knockout mice, are consistent with the hypothesis that melanopsin-expressing ganglion cells are photosensitive and mediate a range of irradiance-detection tasks...
- Evidence for glutamate-mediated excitotoxic mechanisms during photoreceptor degeneration in the rd1 mouse retinaMarie Noelle Delyfer
Laboratoire de Physiopathologie Cellulaire et Moleculaire de la Retine, Institut National de la Santé et de la Recherche Médicale Inserm U592, Universite Pierre et Marie Curie, Paris, France
Mol Vis 11:688-96. 2005Kinetic studies of photoreceptor cell death in the retinal degeneration (rd1) mouse model suggest that photoreceptor degeneration could result from cumulative damage...
- Differential role of Jak-STAT signaling in retinal degenerationsMarijana Samardzija
Laboratory for Retinal Cell Biology, Department Ophthalmology, University Hospital, Frauenklinikstrasse 24, 8091 Zurich, Switzerland
FASEB J 20:2411-3. 2006..This suggests a model in which photoreceptor injury is signaled to cells of the inner retina, which in turn initiate a response either to support viability or accelerate death of injured cells...
- Photic regulation of Fos-like immunoreactivity in the suprachiasmatic nucleus of the mouseC S Colwell
Department of Biology, University of Virginia, Charlottesville 22901
J Comp Neurol 324:135-42. 1992..These results have implications regarding the possible functional roles of Fos in the circadian system and add to our understanding of light regulation of circadian physiology in the mouse...
- Effects of retinal degeneration and brain size upon spatial reversal learning in miceJ L Fuller
Percept Mot Skills 36:947-50. 1973
- Adenylate cyclases in vertebrate retina: enzymatic characteristics in normal and dystrophic mouse retinaJ A Ferrendelli
J Neurochem 38:753-8. 1982..Finally, the present results indicate that regulation of this enzyme in dystrophic retina may be qualitatively and quantitatively different from that in normal retina...
- Basic fibroblast growth factor in retinal development: differential levels of bFGF expression and content in normal and retinal degeneration (rd) mutant miceH Gao
Cullen Eye Institute, Baylor College of Medicine, Houston, Texas 77030, USA
Dev Biol 169:168-84. 1995..These results provide the first evidence that a naturally occurring neuronal degeneration is accompanied by elevated expression of bFGF in degenerating neurons prior to cell death.(ABSTRACT TRUNCATED AT 400 WORDS)..
- Photoreceptor-specific protein expression of mouse retina in organ culture and retardation of rd degeneration in vitro by a combination of basic fibroblast and nerve growth factorsA R Caffe
Department of Anatomy, Erasmus University Rotterdam, The Netherlands
Curr Eye Res 12:719-26. 1993..Neither basic fibroblast growth factor nor nerve growth factor alone has affected the explants phenotype. However, the combination of these proteins has significantly retarded rd cell loss in vitro...
- Mapping the Flv locus controlling resistance to flaviviruses on mouse chromosome 5M Y Sangster
Department of Microbiology, University of Western Australia, Queen Elizabeth II Medical Centre, Nedlands
J Virol 68:448-52. 1994..The chromosomal localization of Flv is the first step in the production of a detailed linkage map of the Flv region, which may open approaches to positional cloning of the resistance gene...
- Adenovirus-mediated gene transfer of ciliary neurotrophic factor can prevent photoreceptor degeneration in the retinal degeneration (rd) mouseM Cayouette
Laboratoire de Transfert de Gènes, Centre de Recherche Universite Laval Robert Giffard, Beauport, Quebec, Canada
Hum Gene Ther 8:423-30. 1997....
- Rod and cone degeneration in the rd mouse is p53 independentJ Wu
Vision Science Research Program, Cellular and Molecular Division, Toronto Western Research Institute, University Health Network, Toronto, Canada
Mol Vis 7:101-6. 2001..To determine whether p53 is required for the death of rod and cone photoreceptors in rd mice, a model of human retinitis pigmentosa, and/or for the natural degeneration of inner nuclear layer (INL) cells in the developing retina...
- Increased c-fos-like immunoreactivity in the superior colliculus and lateral geniculate nucleus of the rd mouseBin Lu
Department of Ophthalmology and Visual Science, Moran Eye Center, University of Utah Health Science Center, 75 N Medical Drive, Salt Lake City, UT 84132, USA
Brain Res 1025:220-5. 2004....
- 3,4-dihydroxyphenylalanine reverses the motor deficits in Pitx3-deficient aphakia mice: behavioral characterization of a novel genetic model of Parkinson's diseaseDong Youn Hwang
Molecular Neurobiology Laboratory, McLean Hospital and Harvard Medical School, Belmont, Massachusetts 02478, USA
J Neurosci 25:2132-7. 2005..Thus, ak mice represent a novel genetic model exhibiting useful characteristics to test the efficacy of symptomatic therapies for PD and to study the functional changes in the striatum after dopamine depletion and L-DOPA treatment...
- Genotype-phenotype correlation of mouse pde6b mutationsAlan W Hart
MRC Human Genetics Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, Scotland, UK
Invest Ophthalmol Vis Sci 46:3443-50. 2005..the underlying molecular defects causing retinal degeneration in seven N-ethyl-N-nitrosourea (ENU) induced mutant alleles of the Pde6b gene and to analyze the timescale of retinal degeneration in these new models of retinitis pigmentosa.
- Efficiency of lentiviral transduction during development in normal and rd miceJijing Pang
Eye Research Institute, Oakland University, Rochester, MI, USA
Mol Vis 12:756-67. 2006..To compare the transduction efficiency of a lentiviral vector in the retina of normal mice and retinal degenerative (rd) mice following subretinal injection at various postnatal ages...
- MICROARRAY ANALYSIS OF RETINAL DEGENERATION IN MICECaroline Zeiss; Fiscal Year: 2007..Colin Barnstable, Professor of Neurobiology and Ophthalmology, who is an internationally recognized molecular biologist using mouse models of retinal development and degeneration. ..
- INNER RETINAL NEURONS IN NORMAL AND DEGENERATING Enrica Strettoi; Fiscal Year: 2009..This is very important to design mutation-independent therapies. ..
- PERMISSIVE RETINAL GENE EXPRESSION VIA HYPOMETHYLATIONJEFFREY BOATRIGHT; Fiscal Year: 2001..abstract_text> ..
- A transgenic model of ocular inflammationRussell Van Gelder; Fiscal Year: 2007..unreadable] [unreadable]..
- ROD NEURODEGENERATIVE AND PROTECTIVE GENESBaerbel Rohrer; Fiscal Year: 2005..We expect to isolate common downstream target genes that are activated both during degeneration and neuroprotection. ..
- Intrinsic Anatomy of the Circadian Rhythm SystemLAWRENCE MORIN; Fiscal Year: 2005..The results will provide the anatomical framework necessary for interpretation of future physiological and molecular experiments. ..
- Brain, Behavior and Biological RhythmsLAWRENCE MORIN; Fiscal Year: 2006..The overall goal of the project is to further elucidate the contribution of the visual system to the regulation of circadian rhythm response to light with particular emphasis on phase control. ..
- Genetics of Age-Related CataractsBo Chang; Fiscal Year: 2007..abstract_text> ..
- Intrinsic photosensitivity of retinal ganglion cellsMICHAEL TRI DO; Fiscal Year: 2007..Understanding the photosensitive ganglion cells will help in understanding basic, light-dependent physiology and its disturbance in illness. [unreadable] [unreadable]..
- Gene expression and retinal vascularizationJohn Ash; Fiscal Year: 2004..By identifying new candidate proteins, this project would feed into our long-range goals of identifying proteins that are essential regulators of retinal vascular development. ..
- Transgenic Rod Development and FunctionBaerbel Rohrer; Fiscal Year: 2005..However, more importantly, it will provide avenues for studying gene defects at the single cell level not only in the retina, but in other systems as well. ..
- Photoreceptor Rescue By Inhibition of Dopamine SignalingJUDITH OGILVIE; Fiscal Year: 2006The rod photoreceptors of the rd1 mouse degenerate in vivo or in organ culture by one month of age as a result of a defect in the beta-subunit of the cGMP-phosphodiesterase gene...
- Retinal Neurons Afferent to the Circadian SystemGARY EDWARD PICKARD; Fiscal Year: 2010..Understanding the retinal neurons afferent to the SCN will aid in our ability to understand and treat these disturbances of phase. ..
- Retinal Neurons Afferent to the Circadian SystemGARY PICKARD; Fiscal Year: 2005..Understanding the retinal neurons and circuits afferent to the SCN and IGL will aid in our ability to understand and treat these disturbances of phase. ..