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
- Brown T, Tsujimura S, Allen A, Wynne J, Bedford R, Vickery G, et al. Melanopsin-based brightness discrimination in mice and humans. Curr Biol. 2012;22:1134-41 pubmed publisher..These data represent the first direct evidence that an aspect of visual discrimination in normally sighted subjects can be supported by inner retinal photoreceptors. ..
- Samardzija M, Wariwoda H, Imsand C, Huber P, Heynen S, Gubler A, et al. Activation of survival pathways in the degenerating retina of rd10 mice. Exp Eye Res. 2012;99:17-26 pubmed publisher..The retinal degeneration 10 (rd10) mouse expresses a mutant form of rod phosphodiesterase leading to autosomal recessive photoreceptor degeneration...
- Tu D, Zhang D, Demas J, Slutsky E, Provencio I, Holy T, et al. Physiologic diversity and development of intrinsically photosensitive retinal ganglion cells. Neuron. 2005;48:987-99 pubmed..These results demonstrate that ipRGCs are the first light-sensitive cells in the retina and suggest previously unappreciated diversity in this cell population. ..
- Hackam A, Strom R, Liu D, Qian J, Wang C, Otteson D, et al. Identification of gene expression changes associated with the progression of retinal degeneration in the rd1 mouse. Invest Ophthalmol Vis Sci. 2004;45:2929-42 pubmed..In the present study, a custom retinal microarray was developed to analyze the rd1 mouse, a well-characterized animal model of human retinal degeneration...
- Takahashi M, Miyoshi H, Verma I, Gage F. Rescue from photoreceptor degeneration in the rd mouse by human immunodeficiency virus vector-mediated gene transfer. J Virol. 1999;73:7812-6 pubmed..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...
- Sahaboglu A, Tanimoto N, Kaur J, Sancho Pelluz J, Huber G, Fahl E, et al. PARP1 gene knock-out increases resistance to retinal degeneration without affecting retinal function. PLoS ONE. 2010;5:e15495 pubmed publisher..polymerase (PARP) group was shown to be involved in photoreceptor degeneration in the human homologous rd1 mouse model for RP...
- Semo M, Peirson S, Lupi D, Lucas R, Jeffery G, Foster R. Melanopsin retinal ganglion cells and the maintenance of circadian and pupillary responses to light in aged rodless/coneless (rd/rd cl) mice. Eur J Neurosci. 2003;17:1793-801 pubmed..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. ..
- Allen A, Cameron M, Brown T, Vugler A, Lucas R. Visual responses in mice lacking critical components of all known retinal phototransduction cascades. PLoS ONE. 2010;5:e15063 pubmed publisher..This anomalous rod opsin-based vision should be considered in experiments relying upon Gnat1 knockout to silence rod phototransduction. ..
- Lin B, Masland R, Strettoi E. Remodeling of cone photoreceptor cells after rod degeneration in rd mice. Exp Eye Res. 2009;88:589-99 pubmed publisher..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. ..
- Kranz K, Paquet Durand F, Weiler R, Janssen Bienhold U, Dedek K. Testing for a gap junction-mediated bystander effect in retinitis pigmentosa: secondary cone death is not altered by deletion of connexin36 from cones. PLoS ONE. 2013;8:e57163 pubmed publisher..We tested this hypothesis in two different mouse models for retinal degeneration (rhodopsin knockout and rd1) by crossbreeding them with connexin36-deficient mice as connexin36 represents the gap junction protein on the cone ..
- Strettoi E, Pignatelli V, Rossi C, Porciatti V, Falsini B. Remodeling of second-order neurons in the retina of rd/rd mutant mice. Vision Res. 2003;43:867-77 pubmed..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. ..
- Vlachantoni D, Bramall A, Murphy M, Taylor R, Shu X, Tulloch B, et al. Evidence of severe mitochondrial oxidative stress and a protective effect of low oxygen in mouse models of inherited photoreceptor degeneration. Hum Mol Genet. 2011;20:322-35 pubmed publisher..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) ..
- Allocca M, Manfredi A, Iodice C, Di Vicino U, Auricchio A. 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 deficiency. Invest Ophthalmol Vis Sci. 2011;52:5713-9 pubmed publisherMutations in the PDE6B gene cause recessive, severe retinitis pigmentosa (RP)...
- Read D, McCall M, Gregg R. Absence of voltage-dependent calcium channels delays photoreceptor degeneration in rd mice. Exp Eye Res. 2002;75:415-20 pubmed..These data indicate that Ca(2+) entry via the L-type voltage-dependent Ca(2+) channel contributes to the mechanisms responsible for photoreceptor cell death in this mouse model of retinitis pigmentosa. ..
- Semo M, Gias C, Ahmado A, Sugano E, Allen A, Lawrence J, et al. Dissecting a role for melanopsin in behavioural light aversion reveals a response independent of conventional photoreception. PLoS ONE. 2010;5:e15009 pubmed publisher..Our findings have significant implications for the understanding of how melanopsin signalling may modulate aversive responses to light in mice and humans. In addition, we also reveal a clear potential for light perception in TKO mice. ..
- Sanyal S, Hawkins R. Genetic interaction in the retinal degeneration of mice. Exp Eye Res. 1981;33:213-22 pubmed
- Hughes S, Pothecary C, Jagannath A, Foster R, Hankins M, Peirson S. Profound defects in pupillary responses to light in TRPM-channel null mice: a role for TRPM channels in non-image-forming photoreception. Eur J Neurosci. 2012;35:34-43 pubmed publisher..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. ..
- Phillips M, Otteson D, Sherry D. Progression of neuronal and synaptic remodeling in the rd10 mouse model of retinitis pigmentosa. J Comp Neurol. 2010;518:2071-89 pubmed publisherThe Pde6b(rd10) (rd10) mouse has a moderate rate of photoreceptor degeneration and serves as a valuable model for human autosomal recessive retinitis pigmentosa (RP)...
- Ekstrom P, Sanyal S, Narfstrom K, Chader G, van Veen T. Accumulation of glial fibrillary acidic protein in Müller radial glia during retinal degeneration. Invest Ophthalmol Vis Sci. 1988;29:1363-71 pubmed..abstract truncated at 250 words) ..
- Thompson S, Foster R, Stone E, Sheffield V, Mrosovsky N. Classical and melanopsin photoreception in irradiance detection: negative masking of locomotor activity by light. Eur J Neurosci. 2008;27:1973-9 pubmed publisher..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. ..
- Zeiss C, Neal J, Johnson E. Caspase-3 in postnatal retinal development and degeneration. Invest Ophthalmol Vis Sci. 2004;45:964-70 pubmed..The temporal nature of apoptotic retardation in the absence of caspase-3 implies the presence of caspase-independent mechanisms of developmental and pathologic cell death. ..
- Mrosovsky N, Hampton R. Spatial responses to light in mice with severe retinal degeneration. Neurosci Lett. 1997;222:204-6 pubmed..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. ..
- Lohr H, Kuntchithapautham K, Sharma A, Rohrer B. Multiple, parallel cellular suicide mechanisms participate in photoreceptor cell death. Exp Eye Res. 2006;83:380-9 pubmed..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. ..
- Panda S, Provencio I, Tu D, Pires S, Rollag M, Castrucci A, et al. Melanopsin is required for non-image-forming photic responses in blind mice. Science. 2003;301:525-7 pubmed..This indicates the importance of both nonvisual and classical visual photoreceptor systems for nonvisual photic responses in mammals. ..
- Keeler C. The Inheritance of a Retinal Abnormality in White Mice. Proc Natl Acad Sci U S A. 1924;10:329-33 pubmed
- Hafezi F, Abegg M, Grimm C, Wenzel A, Munz K, Sturmer J, et al. Retinal degeneration in the rd mouse in the absence of c-fos. Invest Ophthalmol Vis Sci. 1998;39:2239-44 pubmed..These data suggest that in contrast to its role in light-induced photoreceptor degeneration, c-Fos is not essential for apoptosis in the rd mouse. ..
- Yi H, Nakamura R, Mohamed O, Dufort D, Hackam A. Characterization of Wnt signaling during photoreceptor degeneration. Invest Ophthalmol Vis Sci. 2007;48:5733-41 pubmed..The authors recently demonstrated altered expression of Wnt pathway genes during photoreceptor death in rd1 mice, suggesting an involvement for Wnt signaling in the disease process...
- Chang B, Hawes N, Pardue M, German A, Hurd R, Davisson M, et al. Two mouse retinal degenerations caused by missense mutations in the beta-subunit of rod cGMP phosphodiesterase gene. Vision Res. 2007;47:624-33 pubmed..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...
- Frasson M, Picaud S, Leveillard T, Simonutti M, Mohand Said S, Dreyfus H, et al. Glial cell line-derived neurotrophic factor induces histologic and functional protection of rod photoreceptors in the rd/rd mouse. Invest Ophthalmol Vis Sci. 1999;40:2724-34 pubmed..GDNF represents a candidate neurotrophic factor for palliating some forms of hereditary human blindness. ..
- Huerta J, Llamosas M, Cernuda Cernuda R, Garcia Fernandez J. Fos expression in the retina of rd/rd mice during the light/dark cycle. Neurosci Lett. 1997;232:143-6 pubmed..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. ..
- McLaughlin M, Sandberg M, Berson E, Dryja T. Recessive mutations in the gene encoding the beta-subunit of rod phosphodiesterase in patients with retinitis pigmentosa. Nat Genet. 1993;4:130-4 pubmed..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...
- Grafstein B, Murray M, Ingoglia N. Protein synthesis and axonal transport in retinal ganglion cells of mice lacking visual receptors. Brain Res. 1972;44:37-48 pubmed
- Davis R, Tosi J, Janisch K, Kasanuki J, Wang N, Kong J, et al. Functional rescue of degenerating photoreceptors in mice homozygous for a hypomorphic cGMP phosphodiesterase 6 b allele (Pde6bH620Q). Invest Ophthalmol Vis Sci. 2008;49:5067-76 pubmed publisher..In mice homozygous for a nonsense Pde6b(rd1) allele, absence of PDE6 activity is associated with retinal disease similar to humans...
- Paquet Durand F, Beck S, Michalakis S, Goldmann T, Huber G, Mühlfriedel R, et al. A key role for cyclic nucleotide gated (CNG) channels in cGMP-related retinitis pigmentosa. Hum Mol Genet. 2011;20:941-7 pubmed publisherThe 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...
- Brown T, Gias C, Hatori M, Keding S, Semo M, Coffey P, et al. Melanopsin contributions to irradiance coding in the thalamo-cortical visual system. PLoS Biol. 2010;8:e1000558 pubmed publisher..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. ..
- Cameron M, Suaning G, Lovell N, Morley J. Electrical stimulation of inner retinal neurons in wild-type and retinally degenerate (rd/rd) mice. PLoS ONE. 2013;8:e68882 pubmed publisher..This confounding oscillation may significantly reduce the efficacy of electrical stimulation of the degenerate retina, and a greater understanding of its origin will potentially enable it to be dampened or eliminated. ..
- Delyfer M, Forster V, Neveux N, Picaud S, Leveillard T, Sahel J. Evidence for glutamate-mediated excitotoxic mechanisms during photoreceptor degeneration in the rd1 mouse retina. Mol Vis. 2005;11:688-96 pubmedKinetic studies of photoreceptor cell death in the retinal degeneration (rd1) mouse model suggest that photoreceptor degeneration could result from cumulative damage...
- Farber D, Lolley R. Cyclic guanosine monophosphate: elevation in degenerating photoreceptor cells of the C3H mouse retina. Science. 1974;186:449-51 pubmed..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. ..
- Menzler J, Zeck G. Network oscillations in rod-degenerated mouse retinas. J Neurosci. 2011;31:2280-91 pubmed publisher..Recent work in a mouse model of retinal degeneration (rd1) found that, although some intraretinal circuitry is preserved and RGCs maintain characteristic physiological ..
- Joseph R, Li T. Overexpression of Bcl-2 or Bcl-XL transgenes and photoreceptor degeneration. Invest Ophthalmol Vis Sci. 1996;37:2434-46 pubmed..Cell death pathways not regulated by Bcl-2 may be operative in photoreceptor degeneration. ..
- LaVail M, Matthes M, Yasumura D, Steinberg R. Variability in rate of cone degeneration in the retinal degeneration (rd/rd) mouse. Exp Eye Res. 1997;65:45-50 pubmed
- Hart A, McKie L, Morgan J, Gautier P, West K, Jackson I, et al. Genotype-phenotype correlation of mouse pde6b mutations. Invest Ophthalmol Vis Sci. 2005;46:3443-50 pubmed..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...
- Bibb L, Holt J, Tarttelin E, Hodges M, Gregory Evans K, Rutherford A, et al. Temporal and spatial expression patterns of the CRX transcription factor and its downstream targets. Critical differences during human and mouse eye development. Hum Mol Genet. 2001;10:1571-9 pubmed..c., implying that it would be unable to transactivate PDEB, IRBP and arrestin, which were all expressed before 15 weeks...
- Mrosovsky N. Contribution of classic photoreceptors to entrainment. J Comp Physiol A Neuroethol Sens Neural Behav Physiol. 2003;189:69-73 pubmed..Thresholds for entrainment appear to be a more sensitive test of deficits in entrainment than phase shifts in response to light pulses. ..
- Rohrer B, Demos C, Frigg R, Grimm C. Classical complement activation and acquired immune response pathways are not essential for retinal degeneration in the rd1 mouse. Exp Eye Res. 2007;84:82-91 pubmed..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 ..
- Boatright J, Moring A, McElroy C, Phillips M, Do V, Chang B, et al. Tool from ancient pharmacopoeia prevents vision loss. Mol Vis. 2006;12:1706-14 pubmed..Two retinal degeneration models were tested: the rd10 mouse, which has a point mutation in the gene encoding the beta subunit of rod phosphodiesterase, and light induced ..
- Pennesi M, Michaels K, Magee S, Maricle A, Davin S, Garg A, et al. Long-term characterization of retinal degeneration in rd1 and rd10 mice using spectral domain optical coherence tomography. Invest Ophthalmol Vis Sci. 2012;53:4644-56 pubmed publisher..structure of wild-type mice alongside two lines of mice deficient in the ?-subunit of phosphodiesterase (rd1 and rd10 mice) using spectral domain optical coherence tomography (SD-OCT)...
- Marc R, Jones B, Anderson J, Kinard K, Marshak D, Wilson J, et al. Neural reprogramming in retinal degeneration. Invest Ophthalmol Vis Sci. 2007;48:3364-71 pubmed..An instance of human RP provides evidence that rod bipolar cell dendrite switching likely triggers new gene expression patterns and may impair cone pathway function. ..
- Deleon E, Lederman M, Berenstein E, Meir T, Chevion M, Chowers I. Alteration in iron metabolism during retinal degeneration in rd10 mouse. Invest Ophthalmol Vis Sci. 2009;50:1360-5 pubmed publisher..expression of transferrin, transferrin receptor, and ceruloplasmin were evaluated during retinal degeneration in rd10 mice and chemokine receptor 2 (ccr2)-deficient mice...
- Mazzoni F, Novelli E, Strettoi E. Retinal ganglion cells survive and maintain normal dendritic morphology in a mouse model of inherited photoreceptor degeneration. J Neurosci. 2008;28:14282-92 pubmed publisher..2000), which express GFP (green fluorescent protein) in a small number of heterogeneous RGCs types, and rd10 mutants, a model of autosomal recessive RP, which exhibit a typical rod-cone degeneration (Chang et al., 2002)...
- Barone I, Novelli E, Piano I, Gargini C, Strettoi E. Environmental enrichment extends photoreceptor survival and visual function in a mouse model of retinitis pigmentosa. PLoS ONE. 2012;7:e50726 pubmed publisher..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 ..
- Busskamp V, Duebel J, Balya D, Fradot M, Viney T, Siegert S, et al. Genetic reactivation of cone photoreceptors restores visual responses in retinitis pigmentosa. Science. 2010;329:413-7 pubmed publisher..Using human ex vivo retinas, we show that halorhodopsin can reactivate light-insensitive human photoreceptors. Finally, we identified blind patients with persisting, light-insensitive cones for potential halorhodopsin-based therapy. ..
- Comitato A, Sanges D, Rossi A, Humphries M, Marigo V. Activation of Bax in three models of retinitis pigmentosa. Invest Ophthalmol Vis Sci. 2014;55:3555-62 pubmed publisher..Here we investigated activation of Bax in rd1, P23H transgenic, and Rho knockout retinas.
- Komeima K, Usui S, Shen J, Rogers B, Campochiaro P. Blockade of neuronal nitric oxide synthase reduces cone cell death in a model of retinitis pigmentosa. Free Radic Biol Med. 2008;45:905-12 pubmed publisher..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 ..
- Peng G, Chen S. Crx activates opsin transcription by recruiting HAT-containing co-activators and promoting histone acetylation. Hum Mol Genet. 2007;16:2433-52 pubmed
- Samardzija M, Wenzel A, Thiersch M, Frigg R, Reme C, Grimm C. Caspase-1 ablation protects photoreceptors in a model of autosomal dominant retinitis pigmentosa. Invest Ophthalmol Vis Sci. 2006;47:5181-90 pubmed..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...
- Gao H, Hollyfield J. Basic fibroblast growth factor in retinal development: differential levels of bFGF expression and content in normal and retinal degeneration (rd) mutant mice. Dev Biol. 1995;169:168-84 pubmed..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) ..
- Azadi S, Paquet Durand F, Medstrand P, van Veen T, Ekstrom P. Up-regulation and increased phosphorylation of protein kinase C (PKC) delta, mu and theta in the degenerating rd1 mouse retina. Mol Cell Neurosci. 2006;31:759-73 pubmedThe rd1 mouse serves as a model for inherited photoreceptor degeneration: retinitis pigmentosa...
- Pang J, Boye S, Kumar A, Dinculescu A, Deng W, Li J, et al. AAV-mediated gene therapy for retinal degeneration in the rd10 mouse containing a recessive PDEbeta mutation. Invest Ophthalmol Vis Sci. 2008;49:4278-83 pubmed publisherTo 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.
- Lin B, Koizumi A, Tanaka N, Panda S, Masland R. Restoration of visual function in retinal degeneration mice by ectopic expression of melanopsin. Proc Natl Acad Sci U S A. 2008;105:16009-14 pubmed publisher..For therapy in human photodegenerations, channelrhodopsin-2 and melanopsin have different advantages and disadvantages; both proteins (or modifications of them) should be candidates. ..
- Guo C, Otani A, Oishi A, Kojima H, Makiyama Y, Nakagawa S, et al. Knockout of ccr2 alleviates photoreceptor cell death in a model of retinitis pigmentosa. Exp Eye Res. 2012;104:39-47 pubmed publisher..To investigate the role of MCP1/CCR2 in RP pathogenesis, ccr2 mutant RP mice (ccr2(-/-) rd10) were created and analyzed...
- Stone C, Pinto L. Response properties of ganglion cells in the isolated mouse retina. Vis Neurosci. 1993;10:31-9 pubmed..This homogeneity would simplify the comparison of retinas from normal and mutant strains of the mouse. ..
- Thaung C, West K, Clark B, McKie L, Morgan J, Arnold K, et al. Novel ENU-induced eye mutations in the mouse: models for human eye disease. Hum Mol Genet. 2002;11:755-67 pubmed..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...
- Barhoum R, Martinez Navarrete G, Corrochano S, Germain F, Fernandez Sanchez L, de la Rosa E, et al. Functional and structural modifications during retinal degeneration in the rd10 mouse. Neuroscience. 2008;155:698-713 pubmed publisher..We have studied degeneration in the rd10 mice both by immunocytochemistry and TUNEL-labeling of retinal cells, and through electrophysiological recordings...
- Foster R, Provencio I, Hudson D, Fiske S, De Grip W, Menaker M. Circadian photoreception in the retinally degenerate mouse (rd/rd). J Comp Physiol A. 1991;169:39-50 pubmed..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. ..
- Molnár T, Barabas P, Birnbaumer L, Punzo C, Kefalov V, Krizaj D. Store-operated channels regulate intracellular calcium in mammalian rods. J Physiol. 2012;590:3465-81 pubmed publisher..Rod loss in Pde6b(rdl) (rd1), Chx10/Kip1(-/-rdl) and Elovl4(TG2) dystrophic models was associated with ?70% reduction in Trpc1 mRNA ..
- Strettoi E, Gargini C, Novelli E, Sala G, Piano I, Gasco P, et al. Inhibition of ceramide biosynthesis preserves photoreceptor structure and function in a mouse model of retinitis pigmentosa. Proc Natl Acad Sci U S A. 2010;107:18706-11 pubmed publisher..Using the retinal degeneration 10 (rd10) mouse model of RP, we investigated the role of ceramide, a proapoptotic sphingolipid, in retinal degeneration...
- Thyagarajan S, van Wyk M, Lehmann K, Lowel S, Feng G, Wässle H. Visual function in mice with photoreceptor degeneration and transgenic expression of channelrhodopsin 2 in ganglion cells. J Neurosci. 2010;30:8745-58 pubmed publisher..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)...
- Strettoi E, Porciatti V, Falsini B, Pignatelli V, Rossi C. Morphological and functional abnormalities in the inner retina of the rd/rd mouse. J Neurosci. 2002;22:5492-504 pubmed..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. ..
- LaVail M, Yasumura D, Matthes M, Lau Villacorta C, Unoki K, Sung C, et al. Protection of mouse photoreceptors by survival factors in retinal degenerations. Invest Ophthalmol Vis Sci. 1998;39:592-602 pubmed
- Bowes C, Danciger M, Kozak C, Farber D. Isolation of a candidate cDNA for the gene causing retinal degeneration in the rd mouse. Proc Natl Acad Sci U S A. 1989;86:9722-6 pubmed..To our knowledge, this is the earliest molecular defect reported in the rd retina that is observed prior to any phenotypic signs of photoreceptor degeneration. ..
- Garcia Fernandez J, Jimenez A, Foster R. The persistence of cone photoreceptors within the dorsal retina of aged retinally degenerate mice (rd/rd): implications for circadian organization. Neurosci Lett. 1995;187:33-6 pubmed..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. ..
- McLaughlin M, Ehrhart T, Berson E, Dryja T. Mutation spectrum of the gene encoding the beta subunit of rod phosphodiesterase among patients with autosomal recessive retinitis pigmentosa. Proc Natl Acad Sci U S A. 1995;92:3249-53 pubmed..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. ..
- Komeima K, Rogers B, Lu L, Campochiaro P. Antioxidants reduce cone cell death in a model of retinitis pigmentosa. Proc Natl Acad Sci U S A. 2006;103:11300-5 pubmed..In this study, we used the rd1 mouse model of RP to test the hypothesis that cones die from oxidative damage...
- Tucker B, Klassen H, Yang L, Chen D, Young M. Elevated MMP Expression in the MRL Mouse Retina Creates a Permissive Environment for Retinal Regeneration. Invest Ophthalmol Vis Sci. 2008;49:1686-95 pubmed publisher..Experiments were performed using 3- to 4-week-old MRL/MpJ, retinal degenerative (rd1), and C57BL/6 (wild-type) mice...
- Bowes C, Li T, Danciger M, Baxter L, Applebury M, Farber D. Retinal degeneration in the rd mouse is caused by a defect in the beta subunit of rod cGMP-phosphodiesterase. Nature. 1990;347:677-80 pubmed..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. ..
- Paquet Durand F, Hauck S, van Veen T, Ueffing M, Ekstrom P. PKG activity causes photoreceptor cell death in two retinitis pigmentosa models. J Neurochem. 2009;108:796-810 pubmed publisher..Here, we show that activation of cGMP-dependent protein kinase (PKG) hallmarks photoreceptor degeneration in rd1 and rd2 human homologous mouse models...
- Acosta M, Fletcher E, Azizoglu S, Foster L, Farber D, Kalloniatis M. Early markers of retinal degeneration in rd/rd mice. Mol Vis. 2005;11:717-28 pubmed..Therefore, altered metabolic activity as indicated by LDH measurements in the retina appeared to be the earliest sensitive sign of future photoreceptor dysfunction in the rd/rd mice. ..
- Provencio I, Foster R. Circadian rhythms in mice can be regulated by photoreceptors with cone-like characteristics. Brain Res. 1995;694:183-90 pubmed..If this is the case, circadian sensitivity can be maintained by very few degenerate cones. Alternatively, an unknown class of ocular photoreceptor may fulfill this function. ..
- Lucas R, Freedman M, Munoz M, Garcia Fernandez J, Foster R. Regulation of the mammalian pineal by non-rod, non-cone, ocular photoreceptors. Science. 1999;284:505-7 pubmed..These data indicate that mammals have additional ocular photoreceptors that they use in the regulation of temporal physiology. ..
- Portera Cailliau C, Sung C, Nathans J, Adler R. Apoptotic photoreceptor cell death in mouse models of retinitis pigmentosa. Proc Natl Acad Sci U S A. 1994;91:974-8 pubmed..These findings raise the possibility that retinal degenerations may be slowed by interfering with the apoptotic mechanism itself. ..
- Lupi D, Semo M, Foster R. Impact of age and retinal degeneration on the light input to circadian brain structures. Neurobiol Aging. 2012;33:383-92 pubmed publisher..Furthermore, we show light-induced Fos within the vLGN and IGL is predominantly subserved by rods and cones, and once again aging reduces the amplitude of this response. ..
- Bi A, Cui J, Ma Y, Olshevskaya E, Pu M, Dizhoor A, et al. Ectopic expression of a microbial-type rhodopsin restores visual responses in mice with photoreceptor degeneration. Neuron. 2006;50:23-33 pubmed..Thus, expression of microbial-type channelrhodopsins, such as ChR2, in surviving inner retinal neurons is a potential strategy for the restoration of vision after rod and cone degeneration. ..
- Chang G, Hao Y, Wong F. Apoptosis: final common pathway of photoreceptor death in rd, rds, and rhodopsin mutant mice. Neuron. 1993;11:595-605 pubmed..Since DNA fragmentation by internucleosomal cleavage is a cardinal feature of apoptosis, our data suggest that all three of these genetic mutations lead to apoptosis. ..
- Allen A, Brown T, Lucas R. A distinct contribution of short-wavelength-sensitive cones to light-evoked activity in the mouse pretectal olivary nucleus. J Neurosci. 2011;31:16833-43 pubmed publisher..These data reveal a new role for S-cones unrelated to color vision and suggest renewed consideration of cone contributions to NIF vision at shorter wavelengths. ..
- Atkinson C, Feng J, Zhang D. Functional integrity and modification of retinal dopaminergic neurons in the rd1 mutant mouse: roles of melanopsin and GABA. J Neurophysiol. 2013;109:1589-99 pubmed publisher..spontaneous and light-evoked spike activity of genetically labeled dopamine neurons from the retinas of retinal degeneration 1 (rd1) mice...
- Park H, Tan C, Faulkner A, Jabbar S, Schmid G, Abey J, et al. Retinal degeneration increases susceptibility to myopia in mice. Mol Vis. 2013;19:2068-79 pubmed..Dopamine, a potential stop signal for refractive eye growth, was assessed as a potential underlying mechanism...