Plasticity of photoreceptor-generating retinal progenitors revealed by prolonged retinoic acid exposure

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Cameron David A

2011-08-01

Full Text Available Abstract Background Retinoic acid (RA is important for vertebrate eye morphogenesis and is a regulator of photoreceptor development in the retina. In the zebrafish, RA treatment of postmitotic photoreceptor precursors has been shown to promote the differentiation of rods and red-sensitive cones while inhibiting the differentiation of blue- and UV-sensitive cones. The roles played by RA and its receptors in modifying photoreceptor fate remain to be determined. Results Treatment of zebrafish embryos with RA, beginning at the time of retinal progenitor cell proliferation and prior to photoreceptor terminal mitosis, resulted in a significant alteration of rod and cone mosaic patterns, suggesting an increase in the production of rods at the expense of red cones. Quantitative pattern analyses documented increased density of rod photoreceptors and reduced local spacing between rod cells, suggesting rods were appearing in locations normally occupied by cone photoreceptors. Cone densities were correspondingly reduced and cone photoreceptor mosaics displayed expanded and less regular spacing. These results were consistent with replacement of approximately 25% of positions normally occupied by red-sensitive cones, with additional rods. Analysis of embryos from a RA-signaling reporter line determined that multiple retinal cell types, including mitotic cells and differentiating rods and cones, are capable of directly responding to RA. The RA receptors RXRγ and RARαb are expressed in patterns consistent with mediating the effects of RA on photoreceptors. Selective knockdown of RARαb expression resulted in a reduction in endogenous RA signaling in the retina. Knockdown of RARαb also caused a reduced production of rods that was not restored by simultaneous treatments with RA. Conclusions These data suggest that developing retinal cells have a dynamic sensitivity to RA during retinal neurogenesis. In zebrafish RA may influence the rod vs. cone cell fate

Rod and cone photoreceptor cells produce ROS in response to stress in a live retinal explant system.

LENUS (Irish Health Repository)

Bhatt, Lavinia

2010-01-01

PURPOSE: The production of reactive oxygen species (ROS) can lead to oxidative stress, which is a strong contributory factor to many ocular diseases. In this study, the removal of trophic factors is used as a model system to investigate the effects of stress in the retina. The aims were to determine if both rod and cone photoreceptor cells produce ROS when they are deprived of trophic factor support and to demonstrate if the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (Nox) enzymes are responsible for this ROS production. METHODS: Retinas were explanted from mice aged between postnatal days 8-10 and cultured overnight. The following morning, confocal microscopy combined with various fluorescent probes was used to detect the production of ROS. Each time peanut agglutinin (PNA), a cone photoreceptor marker, was used to facilitate orientation of the retina. Dihydroethidium and dihydrorhodamine 123 (DHR123) were used to determine which cells produce ROS. Subsequently, western blots of retinal serial sections were used to detect the presence of Noxs in the different retinal layers. The Nox inhibitor apocynin was then tested to determine if it altered the production of ROS within these cells. RESULTS: Live retinal explants, viewed at high magnifications using confocal microscopy, displayed an increase in the fluorescent products of dihydroethidium and DHR123 upon serum removal when compared to controls. DHR123 fluorescence, once oxidized, localized to mitochondria and was found in the same focal plane as the PNA staining. This showed that cones and rods produced ROS when stressed. Retinal serial sectioning established that the photoreceptor layer expressed Nox4, dual oxidase (Duox) 1, and Duox2 at varying levels. Finally, the Nox inhibitor apocynin decreased the burst stimulated by the stress of serum removal. CONCLUSIONS: Confocal microscopy and PNA staining allowed differentiation of cell types within the outermost layers of the retina, demonstrating

Light-induced translocation of RGS9-1 and Gβ5L in mouse rod photoreceptors.

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Mei Tian

Full Text Available The transducin GTPase-accelerating protein complex, which determines the photoresponse duration of photoreceptors, is composed of RGS9-1, Gβ5L and R9AP. Here we report that RGS9-1 and Gβ5L change their distribution in rods during light/dark adaptation. Upon prolonged dark adaptation, RGS9-1 and Gβ5L are primarily located in rod inner segments. But very dim-light exposure quickly translocates them to the outer segments. In contrast, their anchor protein R9AP remains in the outer segment at all times. In the dark, Gβ5L's interaction with R9AP decreases significantly and RGS9-1 is phosphorylated at S(475 to a significant degree. Dim light exposure leads to quick de-phosphorylation of RGS9-1. Furthermore, after prolonged dark adaptation, RGS9-1 and transducin Gα are located in different cellular compartments. These results suggest a previously unappreciated mechanism by which prolonged dark adaptation leads to increased light sensitivity in rods by dissociating RGS9-1 from R9AP and redistributing it to rod inner segments.

Fasudil, a Clinically Used ROCK Inhibitor, Stabilizes Rod Photoreceptor Synapses after Retinal Detachment.

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Townes-Anderson, Ellen; Wang, Jianfeng; Halász, Éva; Sugino, Ilene; Pitler, Amy; Whitehead, Ian; Zarbin, Marco

2017-06-01

Retinal detachment disrupts the rod-bipolar synapse in the outer plexiform layer by retraction of rod axons. We showed that breakage is due to RhoA activation whereas inhibition of Rho kinase (ROCK), using Y27632, reduces synaptic damage. We test whether the ROCK inhibitor fasudil, used for other clinical applications, can prevent synaptic injury after detachment. Detachments were made in pigs by subretinal injection of balanced salt solution (BSS) or fasudil (1, 10 mM). In some animals, fasudil was injected intravitreally after BSS-induced detachment. After 2 to 4 hours, retinae were fixed for immunocytochemistry and confocal microscopy. Axon retraction was quantified by imaging synaptic vesicle label in the outer nuclear layer. Apoptosis was analyzed using propidium iodide staining. For biochemical analysis by Western blotting, retinal explants, detached from retinal pigmented epithelium, were cultured for 2 hours. Subretinal injection of fasudil (10 mM) reduced retraction of rod spherules by 51.3% compared to control detachments ( n = 3 pigs, P = 0.002). Intravitreal injection of 10 mM fasudil, a more clinically feasible route of administration, also reduced retraction (28.7%, n = 5, P ROCK, was decreased with 30 μM fasudil ( n = 8-10 explants, P ROCK signaling with fasudil reduced photoreceptor degeneration and preserved the rod-bipolar synapse after retinal detachment. These results support the possibility, previously tested with Y27632, that ROCK inhibition may attenuate synaptic damage in iatrogenic detachments.

Knocking Down Snrnp200 Initiates Demorphogenesis of Rod Photoreceptors in Zebrafish

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Yuan Liu

2015-01-01

Full Text Available Purpose. The small nuclear ribonucleoprotein 200 kDa (SNRNP200 gene is a fundamental component for precursor message RNA (pre-mRNA splicing and has been implicated in the etiology of autosomal dominant retinitis pigmentosa (adRP. This study aims to determine the consequences of knocking down Snrnp200 in zebrafish. Methods. Expression of the Snrnp200 transcript in zebrafish was determined via whole mount in situ hybridization. Morpholino oligonucleotide (MO aiming to knock down the expression of Snrnp200 was injected into zebrafish embryos, followed by analyses of aberrant splicing and expression of the U4/U6-U5 tri-small nuclear ribonucleoproteins (snRNPs components and retina-specific transcripts. Systemic changes and retinal phenotypes were further characterized by histological study and immunofluorescence staining. Results. Snrnp200 was ubiquitously expressed in zebrafish. Knocking down Snrnp200 in zebrafish triggered aberrant splicing of the cbln1 gene, upregulation of other U4/U6-U5 tri-snRNP components, and downregulation of a panel of retina-specific transcripts. Systemic defects were found correlated with knockdown of Snrnp200 in zebrafish. Only demorphogenesis of rod photoreceptors was detected in the initial stage, mimicking the disease characteristics of RP. Conclusions. We conclude that knocking down Snrnp200 in zebrafish could alter regular splicing and expression of a panel of genes, which may eventually trigger rod defects.

The biochemistry of photoreceptor cells

International Nuclear Information System (INIS)

Voaden, M.J.; Marshall, J.; Oraedu, A.C.I.

1981-01-01

Photoreceptor cells have high rates of metabolism, and enzyme distributions suggest considerable substrate movement. The authors have used tracer techniques to study the effects of light on photoreceptor metabolism. In vitro, glutamine is metabolized alongside glucose by rat photoreceptors, and is, potentially, a major precursor of the neuroactive amino acids glutamate, aspartate and γ-aminobutyrate (GABA). The utilization of both substrates is decreased by light, as is the turnover of glutamate and aspartate. Tritiated glutamic and aspartic acids are taken up by photoreceptor cells. In the primates all rods but only some cones are labelled, whereas in the guinea pig the picture is reversed. The observations support the premise that glutamate and/or aspartate are photoreceptor neurotransmitters but show that cell and species differences may exist. The authors have been unable to find evidence for the involvement of free radical mechanisms in high light-induced photoreceptor damage but the initial results suggest a reduced metabolism of glutamine and GABA in damaged cells. (Auth.)

Transgenic Expression of Constitutively Active RAC1 Disrupts Mouse Rod Morphogenesis

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Song, Hongman; Bush, Ronald A.; Vijayasarathy, Camasamudram; Fariss, Robert N.; Kjellstrom, Sten; Sieving, Paul A.

2014-01-01

Purpose. Dominant-active RAC1 rescues photoreceptor structure in Drosophila rhodopsin-null mutants, indicating an important role in morphogenesis. This report assesses the morphogenetic effect of activated RAC1 during mammalian rod photoreceptor development using transgenic mice that express constitutively active (CA) RAC1. Methods. Transgenic mice were generated by expressing CA RAC1 under control of the Rhodopsin promoter, and morphological features of the photoreceptors were evaluated by histology, immunohistochemistry, and transmission electron microscopy. Function was evaluated by electroretinography. Potential protein partners of CA RAC1 were identified by co-immunoprecipitation of retinal extracts. Results. Constitutively active RAC1 expression in differentiating rods disrupted outer retinal lamination as early as postnatal day (P)6, and many photoreceptor cell nuclei were displaced apically into the presumptive subretinal space. These photoreceptors did not develop normal inner and outer segments and had abnormal placement of synaptic elements. Some photoreceptor nuclei were also mislocalized into the inner nuclear layer. Extensive photoreceptor degeneration was subsequently observed in the adult animal. Constitutively active RAC1 formed a complex with the polarity protein PAR6 and with microtubule motor dynein in mouse retina. The normal localization of the PAR6 complex was disrupted in CA RAC1-expressing rod photoreceptors. Conclusions. Constitutively active RAC1 had a profound negative effect on mouse rod cell viability and development. Rod photoreceptors in the CA RAC1 retina exhibited a defect in polarity and migration. Constitutively active RAC1 disrupted rod morphogenesis and gave a phenotype resembling that found in the Crumbs mutant. PAR6 and dynein are two potential downstream effectors that may be involved in CA RAC1-mediated defective mouse photoreceptor morphogenesis. PMID:24651551

Two types of Tet-On transgenic lines for doxycycline-inducible gene expression in zebrafish rod photoreceptors and a gateway-based tet-on toolkit.

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Leah J Campbell

Full Text Available The ability to control transgene expression within specific tissues is an important tool for studying the molecular and cellular mechanisms of development, physiology, and disease. We developed a Tet-On system for spatial and temporal control of transgene expression in zebrafish rod photoreceptors. We generated two transgenic lines using the Xenopus rhodopsin promoter to drive the reverse tetracycline-controlled transcriptional transactivator (rtTA, one with self-reporting GFP activity and one with an epitope tagged rtTA. The self-reporting line includes a tetracycline response element (TRE-driven GFP and, in the presence of doxycycline, expresses GFP in larval and adult rods. A time-course of doxycycline treatment demonstrates that maximal induction of GFP expression, as determined by the number of GFP-positive rods, is reached within approximately 24 hours of drug treatment. The epitope-tagged transgenic line eliminates the need for the self-reporting GFP activity by expressing a FLAG-tagged rtTA protein. Both lines demonstrate strong induction of TRE-driven transgenes from plasmids microinjected into one-cell embryos. These results show that spatial and temporal control of transgene expression can be achieved in rod photoreceptors. Additionally, system components are constructed in Gateway compatible vectors for the rapid cloning of doxycycline-inducible transgenes and use in other areas of zebrafish research.

Light adaptation and the evolution of vertebrate photoreceptors.

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Morshedian, Ala; Fain, Gordon L

2017-07-15

Lamprey are cyclostomes, a group of vertebrates that diverged from lines leading to jawed vertebrates (including mammals) in the late Cambrian, 500 million years ago. It may therefore be possible to infer properties of photoreceptors in early vertebrate progenitors by comparing lamprey to other vertebrates. We show that lamprey rods and cones respond to light much like rods and cones in amphibians and mammals. They operate over a similar range of light intensities and adapt to backgrounds and bleaches nearly identically. These correspondences are pervasive and detailed; they argue for the presence of rods and cones very early in the evolution of vertebrates with properties much like those of rods and cones in existing vertebrate species. The earliest vertebrates were agnathans - fish-like organisms without jaws, which first appeared near the end of the Cambrian radiation. One group of agnathans became cyclostomes, which include lamprey and hagfish. Other agnathans gave rise to jawed vertebrates or gnathostomes, the group including all other existing vertebrate species. Because cyclostomes diverged from other vertebrates 500 million years ago, it may be possible to infer some of the properties of the retina of early vertebrate progenitors by comparing lamprey to other vertebrates. We have previously shown that rods and cones in lamprey respond to light much like photoreceptors in other vertebrates and have a similar sensitivity. We now show that these affinities are even closer. Both rods and cones adapt to background light and to bleaches in a manner almost identical to other vertebrate photoreceptors. The operating range in darkness is nearly the same in lamprey and in amphibian or mammalian rods and cones; moreover background light shifts response-intensity curves downward and to the right over a similar range of ambient intensities. Rods show increment saturation at about the same intensity as mammalian rods, and cones never saturate. Bleaches decrease

Lateral interactions in the photoreceptor membrane: a NMR study

International Nuclear Information System (INIS)

Mollevanger, L.C.P.J.

1987-01-01

The photoreceptor membrane has an exceptionally high content of polyunsaturated fatty acyl chains combined with a high amount of phosphatidyl ethanolamine. It is situated in a cell organelle, the rod outer segment, with a high biological activity in which controlable trans-membrane currents of different ions play an important role. These characteristics make it a very interesting biological membrane to search for the existence of non-bilayer structures. Therefore in this thesis a detailed study of the polymorphic phase behaviour of the rod outer segment photoreceptor lipids was undertaken, concerning modulation of the polymorphic phase behaviour of photoreceptor membrane lipids by divalent cations and temperature, polymorphism of the individual phospholipid classes phosphatidylethanolamine and phosphatidylserine and effects of cholesterol, bilayer stabilization by (rhod)opsin. Morphologically intact rod outer segment possesses a large magnetic anisotropy. This property is used to obtain 31 P-NMR of oriented photoreceptor membranes which allows spectral analysis and identification of individual phospholipid classes, and allows to study lateral lipid diffusion in intact disk membranes. The power of high resolution solid state 13 C-NMR to study the conformation of the chromophore in rhodopsin is demonstrated. (Auth.)

Characterization of photoreceptor cell types in the little brown bat Myotis lucifugus (Vespertilionidae).

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Feller, K D; Lagerholm, S; Clubwala, R; Silver, M T; Haughey, D; Ryan, J M; Loew, E R; Deutschlander, M E; Kenyon, K L

2009-12-01

We report the expression of three visual opsins in the retina of the little brown bat (Myotis lucifugus, Vespertilionidae). Gene sequences for a rod-specific opsin and two cone-specific opsins were cloned from cDNA derived from bat eyes. Comparative sequence analyses indicate that the two cone opsins correspond to an ultraviolet short-wavelength opsin (SWS1) and a long-wavelength opsin (LWS). Immunocytochemistry using antisera to visual opsins revealed that the little brown bat retina contains two types of cone photoreceptors within a rod-dominated background. However, unlike other mammalian photoreceptors, M. lucifugus cones and rods are morphologically indistinguishable by light microscopy. Both photoreceptor types have a thin, elongated outer segment. Using microspectrophotometry we classified the absorption spectrum for the ubiquitous rods. Similar to other mammals, bat rhodopsin has an absorption peak near 500 nm. Although we were unable to confirm a spectral range, cellular and molecular analyses indicate that M. lucifugus expresses two types of cone visual pigments located within the photoreceptor layer. This study provides important insights into the visual capacity of a nocturnal microchiropteran species.

Human amniotic fluid promotes retinal pigmented epithelial cells' trans-differentiation into rod photoreceptors and retinal ganglion cells.

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Ghaderi, Shima; Soheili, Zahra-Soheila; Ahmadieh, Hamid; Davari, Maliheh; Jahromi, Fatemeh Sanie; Samie, Shahram; Rezaie-Kanavi, Mozhgan; Pakravesh, Jalil; Deezagi, Abdolkhalegh

2011-09-01

To evaluate the effect of human amniotic fluid (HAF) on retinal pigmented epithelial cells growth and trans-differentiation into retinal neurons, retinal pigmented epithelium (RPE) cells were isolated from neonatal human cadaver eye globes and cultured in Dulbecco's modified Eagle's medium-F12 supplemented with 10% fetal bovine serum (FBS). Confluent monolayer cultures were trypsinized and passaged using FBS-containing or HAF-containing media. Amniotic fluid samples were received from pregnant women in the first trimester of gestation. Cell proliferation and death enzyme-linked immunosorbent assays were performed to assess the effect of HAF on RPE cell growth. Trans-differentiation into rod photoreceptors and retinal ganglion cells was also studied using immunocytochemistry and real-time polymerase chain reaction techniques. Primary cultures of RPE cells were successfully established under FBS-containing or HAF-containing media leading to rapid cell growth and proliferation. When RPE cells were moved to in vitro culture system, they began to lose their differentiation markers such as pigmentation and RPE65 marker and trans-differentiated neural-like cells followed by spheroid colonies pertaining to stem/progenitor cells were morphologically detected. Immunocytochemistry (ICC) analysis of HAF-treated cultures showed a considerable expression of Rhodopsin gene (30% Rhodopsin-positive cells) indicating trans-differentiation of RPE cells to rod photoreceptors. Real-time polymerase chain reaction revealed an HAF-dose-dependant expression of Thy-1 gene (RGC marker) and significant promoting effect of HAF on RGCs generation. The data presented here suggest that HAF possesses invaluable stimulatory effect on RPE cells growth and trans-differentiation into retinal neurons. It can be regarded as a newly introduced enriched supplement in serum-free kinds of media used in neuro-retinal regeneration studies.

Overexpression of guanylate cyclase activating protein 2 in rod photoreceptors in vivo leads to morphological changes at the synaptic ribbon.

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Natalia López-del Hoyo

Full Text Available Guanylate cyclase activating proteins are EF-hand containing proteins that confer calcium sensitivity to retinal guanylate cyclase at the outer segment discs of photoreceptor cells. By making the rate of cGMP synthesis dependent on the free intracellular calcium levels set by illumination, GCAPs play a fundamental role in the recovery of the light response and light adaptation. The main isoforms GCAP1 and GCAP2 also localize to the synaptic terminal, where their function is not known. Based on the reported interaction of GCAP2 with Ribeye, the major component of synaptic ribbons, it was proposed that GCAP2 could mediate the synaptic ribbon dynamic changes that happen in response to light. We here present a thorough ultrastructural analysis of rod synaptic terminals in loss-of-function (GCAP1/GCAP2 double knockout and gain-of-function (transgenic overexpression mouse models of GCAP2. Rod synaptic ribbons in GCAPs-/- mice did not differ from wildtype ribbons when mice were raised in constant darkness, indicating that GCAPs are not required for ribbon early assembly or maturation. Transgenic overexpression of GCAP2 in rods led to a shortening of synaptic ribbons, and to a higher than normal percentage of club-shaped and spherical ribbon morphologies. Restoration of GCAP2 expression in the GCAPs-/- background (GCAP2 expression in the absence of endogenous GCAP1 had the striking result of shortening ribbon length to a much higher degree than overexpression of GCAP2 in the wildtype background, as well as reducing the thickness of the outer plexiform layer without affecting the number of rod photoreceptor cells. These results indicate that preservation of the GCAP1 to GCAP2 relative levels is relevant for maintaining the integrity of the synaptic terminal. Our demonstration of GCAP2 immunolocalization at synaptic ribbons at the ultrastructural level would support a role of GCAPs at mediating the effect of light on morphological remodeling changes of

Characterization of multiple light damage paradigms reveals regional differences in photoreceptor loss.

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Thomas, Jennifer L; Nelson, Craig M; Luo, Xixia; Hyde, David R; Thummel, Ryan

2012-04-01

Zebrafish provide an attractive model to study the retinal response to photoreceptor apoptosis due to its remarkable ability to spontaneously regenerate retinal neurons following damage. There are currently two widely-used light-induced retinal degeneration models to damage photoreceptors in the adult zebrafish. One model uses constant bright light, whereas the other uses a short exposure to extremely intense ultraviolet light. Although both models are currently used, it is unclear whether they differ in regard to the extent of photoreceptor damage or the subsequent regeneration response. Here we report a thorough analysis of the photoreceptor damage and subsequent proliferation response elicited by each individual treatment, as well as by the concomitant use of both treatments. We show a differential loss of rod and cone photoreceptors with each treatment. Additionally, we show that the extent of proliferation observed in the retina directly correlates with the severity of photoreceptor loss. We also demonstrate that both the ventral and posterior regions of the retina are partially protected from light damage. Finally, we show that combining a short ultraviolet exposure followed by a constant bright light treatment largely eliminates the neuroprotected regions, resulting in widespread loss of rod and cone photoreceptors and a robust regenerative response throughout the retina. Copyright © 2012 Elsevier Ltd. All rights reserved.

Pushing the limits of photoreception in twilight conditions: The rod-like cone retina of the deep-sea pearlsides

KAUST Repository

Busserolles, Fanny de; Cortesi, Fabio; Helvik, Jon Vidar; Davies, Wayne I. L.; Templin, Rachel M.; Sullivan, Robert K. P.; Michell, Craig T.; Mountford, Jessica K.; Collin, Shaun P.; Irigoien, Xabier; Kaartvedt, Stein; Marshall, Justin

2017-01-01

retina does not have rod photoreceptors only; instead, it is composed almost exclusively of transmuted cone photoreceptors. These transmuted cells combine the morphological characteristics of a rod photoreceptor with a cone opsin and a cone

Rip3 knockdown rescues photoreceptor cell death in blind pde6c zebrafish.

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Viringipurampeer, I A; Shan, X; Gregory-Evans, K; Zhang, J P; Mohammadi, Z; Gregory-Evans, C Y

2014-05-01

Achromatopsia is a progressive autosomal recessive retinal disease characterized by early loss of cone photoreceptors and later rod photoreceptor loss. In most cases, mutations have been identified in CNGA3, CNGB3, GNAT2, PDE6C or PDE6H genes. Owing to this genetic heterogeneity, mutation-independent therapeutic schemes aimed at preventing cone cell death are very attractive treatment strategies. In pde6c(w59) mutant zebrafish, cone photoreceptors expressed high levels of receptor-interacting protein kinase 1 (RIP1) and receptor-interacting protein kinase 3 (RIP3) kinases, key regulators of necroptotic cell death. In contrast, rod photoreceptor cells were alternatively immunopositive for caspase-3 indicating activation of caspase-dependent apoptosis in these cells. Morpholino gene knockdown of rip3 in pde6c(w59) embryos rescued the dying cone photoreceptors by inhibiting the formation of reactive oxygen species and by inhibiting second-order neuron remodelling in the inner retina. In rip3 morphant larvae, visual function was restored in the cones by upregulation of the rod phosphodiesterase genes (pde6a and pde6b), compensating for the lack of cone pde6c suggesting that cones are able to adapt to their local environment. Furthermore, we demonstrated through pharmacological inhibition of RIP1 and RIP3 activity that cone cell death was also delayed. Collectively, these results demonstrate that the underlying mechanism of cone cell death in the pde6c(w59) mutant retina is through necroptosis, whereas rod photoreceptor bystander death occurs through a caspase-dependent mechanism. This suggests that targeting the RIP kinase signalling pathway could be an effective therapeutic intervention in retinal degeneration patients. As bystander cell death is an important feature of many retinal diseases, combinatorial approaches targeting different cell death pathways may evolve as an important general principle in treatment.

Induction of the unfolded protein response by constitutive G-protein signaling in rod photoreceptor cells.

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Wang, Tian; Chen, Jeannie

2014-10-17

Phototransduction is a G-protein signal transduction cascade that converts photon absorption to a change in current at the plasma membrane. Certain genetic mutations affecting the proteins in the phototransduction cascade cause blinding disorders in humans. Some of these mutations serve as a genetic source of "equivalent light" that activates the cascade, whereas other mutations lead to amplification of the light response. How constitutive phototransduction causes photoreceptor cell death is poorly understood. We showed that persistent G-protein signaling, which occurs in rod arrestin and rhodopsin kinase knock-out mice, caused a rapid and specific induction of the PERK pathway of the unfolded protein response. These changes were not observed in the cGMP-gated channel knock-out rods, an equivalent light condition that mimics light-stimulated channel closure. Thus transducin signaling, but not channel closure, triggers rapid cell death in light damage caused by constitutive phototransduction. Additionally, we show that in the albino light damage model cell death was not associated with increase in global protein ubiquitination or unfolded protein response induction. Taken together, these observations provide novel mechanistic insights into the cell death pathway caused by constitutive phototransduction and identify the unfolded protein response as a potential target for therapeutic intervention. © 2014 by The American Society for Biochemistry and Molecular Biology, Inc.

Cone dystrophy with "supernormal" rod ERG: psychophysical testing shows comparable rod and cone temporal sensitivity losses with no gain in rod function.

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Stockman, Andrew; Henning, G Bruce; Michaelides, Michel; Moore, Anthony T; Webster, Andrew R; Cammack, Jocelyn; Ripamonti, Caterina

2014-02-10

We report a psychophysical investigation of 5 observers with the retinal disorder "cone dystrophy with supernormal rod ERG," caused by mutations in the gene KCNV2 that encodes a voltage-gated potassium channel found in rod and cone photoreceptors. We compared losses for rod- and for cone-mediated vision to further investigate the disorder and to assess whether the supernormal ERG is associated with any visual benefit. L-cone, S-cone, and rod temporal acuity (critical flicker fusion frequency) were measured as a function of target irradiance; L-cone temporal contrast sensitivity was measured as a function of temporal frequency. Temporal acuity measures revealed that losses for vision mediated by rods, S-cones, and L-cones are roughly equivalent. Further, the gain in rod function implied by the supernormal ERG provides no apparent benefit to near-threshold rod-mediated visual performance. The L-cone temporal contrast sensitivity function in affected observers was similar in shape to the mean normal function but only after the mean function was compressed by halving the logarithmic sensitivities. The name of this disorder is potentially misleading because the comparable losses found across rod and cone vision suggest that the disorder is a generalized cone-rod dystrophy. Temporal acuity and temporal contrast sensitivity measures are broadly consistent with the defect in the voltage-gated potassium channel producing a nonlinear distortion of the photoreceptor response but after otherwise normal transduction processes.

Derivation of Traceable and Transplantable Photoreceptors from Mouse Embryonic Stem Cells

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Sarah Decembrini

2014-06-01

Full Text Available Retinal degenerative diseases resulting in the loss of photoreceptors are one of the major causes of blindness. Photoreceptor replacement therapy is a promising treatment because the transplantation of retina-derived photoreceptors can be applied now to different murine retinopathies to restore visual function. To have an unlimited source of photoreceptors, we derived a transgenic embryonic stem cell (ESC line in which the Crx-GFP transgene is expressed in photoreceptors and assessed the capacity of a 3D culture protocol to produce integration-competent photoreceptors. This culture system allows the production of a large number of photoreceptors recapitulating the in vivo development. After transplantation, integrated cells showed the typical morphology of mature rods bearing external segments and ribbon synapses. We conclude that a 3D protocol coupled with ESCs provides a safe and renewable source of photoreceptors displaying a development and transplantation competence comparable to photoreceptors from age-matched retinas.

Inverted nuclear architecture and its development during differentiation of mouse rod photoreceptor cells: a new model to study nuclear architecture.

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Solovei, I; Joffe, B

2010-09-01

Interphase nuclei have a conserved architecture: heterochromatin occupies the nuclear periphery, whereas euchromatin resides in the nuclear interior. It has recently been found that rod photoreceptor cells of nocturnal mammals have an inverted architecture, which transforms these nuclei in microlenses and supposedly facilitates a reduction in photon loss in the retina. This unique deviation from the nearly universal pattern throws a new light on the nuclear organization. In the article we discuss the implications of the studies of the inverted nuclei for understanding the role of the spatial organization of the nucleus in nuclear functions.

Pushing the limits of photoreception in twilight conditions: The rod-like cone retina of the deep-sea pearlsides

KAUST Repository

Busserolles, Fanny de

2017-11-09

Most vertebrates have a duplex retina comprising two photoreceptor types, rods for dim-light (scotopic) vision and cones for bright-light (photopic) and color vision. However, deep-sea fishes are only active in dim-light conditions; hence, most species have lost their cones in favor of a simplex retina composed exclusively of rods. Although the pearlsides, Maurolicus spp., have such a pure rod retina, their behavior is at odds with this simplex visual system. Contrary to other deep-sea fishes, pearlsides are mostly active during dusk and dawn close to the surface, where light levels are intermediate (twilight or mesopic) and require the use of both rod and cone photoreceptors. This study elucidates this paradox by demonstrating that the pearlside retina does not have rod photoreceptors only; instead, it is composed almost exclusively of transmuted cone photoreceptors. These transmuted cells combine the morphological characteristics of a rod photoreceptor with a cone opsin and a cone phototransduction cascade to form a unique photoreceptor type, a rod-like cone, specifically tuned to the light conditions of the pearlsides\\' habitat (blue-shifted light at mesopic intensities). Combining properties of both rods and cones into a single cell type, instead of using two photoreceptor types that do not function at their full potential under mesopic conditions, is likely to be the most efficient and economical solution to optimize visual performance. These results challenge the standing paradigm of the function and evolution of the vertebrate duplex retina and emphasize the need for a more comprehensive evaluation of visual systems in general.

Usher syndrome type 1-associated cadherins shape the photoreceptor outer segment.

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Schietroma, Cataldo; Parain, Karine; Estivalet, Amrit; Aghaie, Asadollah; Boutet de Monvel, Jacques; Picaud, Serge; Sahel, José-Alain; Perron, Muriel; El-Amraoui, Aziz; Petit, Christine

2017-06-05

Usher syndrome type 1 (USH1) causes combined hearing and sight defects, but how mutations in USH1 genes lead to retinal dystrophy in patients remains elusive. The USH1 protein complex is associated with calyceal processes, which are microvilli of unknown function surrounding the base of the photoreceptor outer segment. We show that in Xenopus tropicalis , these processes are connected to the outer-segment membrane by links composed of protocadherin-15 (USH1F protein). Protocadherin-15 deficiency, obtained by a knockdown approach, leads to impaired photoreceptor function and abnormally shaped photoreceptor outer segments. Rod basal outer disks displayed excessive outgrowth, and cone outer segments were curved, with lamellae of heterogeneous sizes, defects also observed upon knockdown of Cdh23 , encoding cadherin-23 (USH1D protein). The calyceal processes were virtually absent in cones and displayed markedly reduced F-actin content in rods, suggesting that protocadherin-15-containing links are essential for their development and/or maintenance. We propose that calyceal processes, together with their associated links, control the sizing of rod disks and cone lamellae throughout their daily renewal. © 2017 Schietroma et al.

Age-related deterioration of rod vision in mice.

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Kolesnikov, Alexander V; Fan, Jie; Crouch, Rosalie K; Kefalov, Vladimir J

2010-08-18

Even in healthy individuals, aging leads to deterioration in visual acuity, contrast sensitivity, visual field, and dark adaptation. Little is known about the neural mechanisms that drive the age-related changes of the retina and, more specifically, photoreceptors. According to one hypothesis, the age-related deterioration in rod function is due to the limited availability of 11-cis-retinal for rod pigment formation. To determine how aging affects rod photoreceptors and to test the retinoid-deficiency hypothesis, we compared the morphological and functional properties of rods of adult and aged B6D2F1/J mice. We found that the number of rods and the length of their outer segments were significantly reduced in 2.5-year-old mice compared with 4-month-old animals. Aging also resulted in a twofold reduction in the total level of opsin in the retina. Behavioral tests revealed that scotopic visual acuity and contrast sensitivity were decreased by twofold in aged mice, and rod ERG recordings demonstrated reduced amplitudes of both a- and b-waves. Sensitivity of aged rods determined from single-cell recordings was also decreased by 1.5-fold, corresponding to not more than 1% free opsin in these photoreceptors, and kinetic parameters of dim flash response were not altered. Notably, the rate of rod dark adaptation was unaffected by age. Thus, our results argue against age-related deficiency of 11-cis-retinal in the B6D2F1/J mouse rod visual cycle. Surprisingly, the level of cellular dark noise was increased in aged rods, providing an alternative mechanism for their desensitization.

Effect of hydroxylamine on photon-like events during dark adaptation in toad rod photoreceptors.

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Leibrock, C S; Lamb, T D

1997-01-01

1. The suction pipette technique was used to investigate the recovery of toad rod photoreceptors following small bleaches of 0.2-3% of the rhodopsin. 2. The reduction in sensitivity and the increase in noise elicited by bleaches were measured, and from these measurements the underlying rate of occurrence of photon-like events was calculated as a function of time after the bleach. 3. Exposure to hydroxylamine solution was used to hasten the decomposition of the metarhodopsin photoproducts. The outer segment was exposed to 110 mM hydroxylamine in a low-Ca2+ Ringer solution for a period of 10-50 s beginning 10-17 min after the bleaching exposure. 4. By the time of the hydroxylamine exposure, the flash sensitivity and response kinetics had returned almost to normal, and were not significantly altered by the exposure. 5. Following hydroxylamine exposure, the rate of spontaneous photon-like events in the rods declined rapidly to near pre-bleach levels. 6. We conclude that hydroxylamine reduces the rate of occurrence of photon-like events induced by a bleach, and we postulate that this reduction results from the removal of metarhodopsin (most likely metarhodopsin II) from the outer segment. 7. Our results are consistent with a model in which photon-like events result from reversal of the reactions (phosphorylation and capping by arrestin) that lead to inactivation of the activated form of rhodopsin, Rh*. PMID:9174997

Identification of endogenous fluorophores in the photoreceptors using autofluorescence spectroscopy

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Zhao, Lingling; Qu, Junle; Niu, Hanben

2007-11-01

In this paper, we present our investigation on the identification of endogenous fluorophores in photoreceptors using autofluorescence spectroscopy, which is performed with an inverted laser scanning confocal microscope equipped with an Argon ion laser and a GreNe laser. In our experiments, individual cones and rods are clearly resolved even in freshly prepared retina samples, without slicing or labeling. The experiment results show that autofluorescence spectrum of the photoreceptors has three peaks approximately at 525nm, 585nm and 665nm. Furthermore, the brightest autofluorescence originates from the photoreceptor outer segments. We can, therefore, come to a conclusion that the peaks at 525nm, 585nm are corresponding to FAD and A2-PE, respectively, which are distributed in the photoreceptor outer segments.

Usher syndrome type 1–associated cadherins shape the photoreceptor outer segment

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Parain, Karine; Aghaie, Asadollah; Picaud, Serge

2017-01-01

Usher syndrome type 1 (USH1) causes combined hearing and sight defects, but how mutations in USH1 genes lead to retinal dystrophy in patients remains elusive. The USH1 protein complex is associated with calyceal processes, which are microvilli of unknown function surrounding the base of the photoreceptor outer segment. We show that in Xenopus tropicalis, these processes are connected to the outer-segment membrane by links composed of protocadherin-15 (USH1F protein). Protocadherin-15 deficiency, obtained by a knockdown approach, leads to impaired photoreceptor function and abnormally shaped photoreceptor outer segments. Rod basal outer disks displayed excessive outgrowth, and cone outer segments were curved, with lamellae of heterogeneous sizes, defects also observed upon knockdown of Cdh23, encoding cadherin-23 (USH1D protein). The calyceal processes were virtually absent in cones and displayed markedly reduced F-actin content in rods, suggesting that protocadherin-15–containing links are essential for their development and/or maintenance. We propose that calyceal processes, together with their associated links, control the sizing of rod disks and cone lamellae throughout their daily renewal. PMID:28495838

Rax Homeoprotein Regulates Photoreceptor Cell Maturation and Survival in Association with Crx in the Postnatal Mouse Retina.

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Irie, Shoichi; Sanuki, Rikako; Muranishi, Yuki; Kato, Kimiko; Chaya, Taro; Furukawa, Takahisa

2015-08-01

The Rax homeobox gene plays essential roles in multiple processes of vertebrate retina development. Many vertebrate species possess Rax and Rax2 genes, and different functions have been suggested. In contrast, mice contain a single Rax gene, and its functional roles in late retinal development are still unclear. To clarify mouse Rax function in postnatal photoreceptor development and maintenance, we generated conditional knockout mice in which Rax in maturing or mature photoreceptor cells was inactivated by tamoxifen treatment (Rax iCKO mice). When Rax was inactivated in postnatal Rax iCKO mice, developing photoreceptor cells showed a significant decrease in the level of the expression of rod and cone photoreceptor genes and mature adult photoreceptors exhibited a specific decrease in cone cell numbers. In luciferase assays, we found that Rax and Crx cooperatively transactivate Rhodopsin and cone opsin promoters and that an optimum Rax expression level to transactivate photoreceptor gene expression exists. Furthermore, Rax and Crx colocalized in maturing photoreceptor cells, and their coimmunoprecipitation was observed in cultured cells. Taken together, these results suggest that Rax plays essential roles in the maturation of both cones and rods and in the survival of cones by regulating photoreceptor gene expression with Crx in the postnatal mouse retina. Copyright © 2015, American Society for Microbiology. All Rights Reserved.

Cone visual pigments are present in gecko rod cells.

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Kojima, D; Okano, T; Fukada, Y; Shichida, Y; Yoshizawa, T; Ebrey, T G

1992-08-01

The Tokay gecko (Gekko gekko), a nocturnal lizard, has two kinds of visual pigments, P467 and P521. In spite of the pure-rod morphology of the photoreceptor cells, the biochemical properties of P521 and P467 resemble those of iodopsin (the chicken red-sensitive cone visual pigment) and rhodopsin, respectively. We have found that the amino acid sequence of P521 deduced from the cDNA was very similar to that of iodopsin. In addition, P467 has the highest homology with the chicken green-sensitive cone visual pigment, although it also has a relatively high homology with rhodopsins. These results give additional strength to the transmutation theory of Walls [Walls, G. L. (1934) Am. J. Ophthalmol. 17, 892-915], who proposed that the rod-shaped photoreceptor cells of lizards have been derived from ancestral cone-like photoreceptors. Apparently amino acid sequences of visual pigments are less changeable than the morphology of the photoreceptor cells in the course of evolution.

Shifts in Selective Pressures on Snake Phototransduction Genes Associated with Photoreceptor Transmutation and Dim-Light Ancestry.

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Schott, Ryan K; Van Nynatten, Alexander; Card, Daren C; Castoe, Todd A; S W Chang, Belinda

2018-06-01

The visual systems of snakes are heavily modified relative to other squamates, a condition often thought to reflect their fossorial origins. Further modifications are seen in caenophidian snakes, where evolutionary transitions between rod and cone photoreceptors, termed photoreceptor transmutations, have occurred in many lineages. Little previous work, however, has focused on the molecular evolutionary underpinnings of these morphological changes. To address this, we sequenced seven snake eye transcriptomes and utilized new whole-genome and targeted capture sequencing data. We used these data to analyze gene loss and shifts in selection pressures in phototransduction genes that may be associated with snake evolutionary origins and photoreceptor transmutation. We identified the surprising loss of rhodopsin kinase (GRK1), despite a low degree of gene loss overall and a lack of relaxed selection early during snake evolution. These results provide some of the first evolutionary genomic corroboration for a dim-light ancestor that lacks strong fossorial adaptations. Our results also indicate that snakes with photoreceptor transmutation experienced significantly different selection pressures from other reptiles. Significant positive selection was found primarily in cone-specific genes, but not rod-specific genes, contrary to our expectations. These results reveal potential molecular adaptations associated with photoreceptor transmutation and also highlight unappreciated functional differences between rod- and cone-specific phototransduction proteins. This intriguing example of snake visual system evolution illustrates how the underlying molecular components of a complex system can be reshaped in response to changing selection pressures.

Retinal cone photoreceptors of the deer mouse Peromyscus maniculatus: development, topography, opsin expression and spectral tuning.

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Patrick Arbogast

Full Text Available A quantitative analysis of photoreceptor properties was performed in the retina of the nocturnal deer mouse, Peromyscus maniculatus, using pigmented (wildtype and albino animals. The aim was to establish whether the deer mouse is a more suitable model species than the house mouse for photoreceptor studies, and whether oculocutaneous albinism affects its photoreceptor properties. In retinal flatmounts, cone photoreceptors were identified by opsin immunostaining, and their numbers, spectral types, and distributions across the retina were determined. Rod photoreceptors were counted using differential interference contrast microscopy. Pigmented P. maniculatus have a rod-dominated retina with rod densities of about 450.000/mm(2 and cone densities of 3000-6500/mm(2. Two cone opsins, shortwave sensitive (S and middle-to-longwave sensitive (M, are present and expressed in distinct cone types. Partial sequencing of the S opsin gene strongly supports UV sensitivity of the S cone visual pigment. The S cones constitute a 5-15% minority of the cones. Different from house mouse, S and M cone distributions do not have dorsoventral gradients, and coexpression of both opsins in single cones is exceptional (<2% of the cones. In albino P. maniculatus, rod densities are reduced by approximately 40% (270.000/mm(2. Overall, cone density and the density of cones exclusively expressing S opsin are not significantly different from pigmented P. maniculatus. However, in albino retinas S opsin is coexpressed with M opsin in 60-90% of the cones and therefore the population of cones expressing only M opsin is significantly reduced to 5-25%. In conclusion, deer mouse cone properties largely conform to the general mammalian pattern, hence the deer mouse may be better suited than the house mouse for the study of certain basic cone properties, including the effects of albinism on cone opsin expression.

Genetics Home Reference: cone-rod dystrophy

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... common cause of autosomal recessive cone-rod dystrophy , accounting for 30 to 60 percent of cases. At ... dystrophy play essential roles in the structure and function of specialized light receptor cells (photoreceptors) in the ...

Genetic Dissection of Dual Roles for the Transcription Factor six7 in Photoreceptor Development and Patterning in Zebrafish.

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Mailin Sotolongo-Lopez

2016-04-01

Full Text Available The visual system of a particular species is highly adapted to convey detailed ecological and behavioral information essential for survival. The consequences of structural mutations of opsins upon spectral sensitivity and environmental adaptation have been studied in great detail, but lacking is knowledge of the potential influence of alterations in gene regulatory networks upon the diversity of cone subtypes and the variation in the ratio of rods and cones observed in numerous diurnal and nocturnal species. Exploiting photoreceptor patterning in cone-dominated zebrafish, we uncovered two independent mechanisms by which the sine oculis homeobox homolog 7 (six7 regulates photoreceptor development. In a genetic screen, we isolated the lots-of-rods-junior (ljrp23ahub mutation that resulted in an increased number and uniform distribution of rods in otherwise normal appearing larvae. Sequence analysis, genome editing using TALENs and knockdown strategies confirm ljrp23ahub as a hypomorphic allele of six7, a teleost orthologue of six3, with known roles in forebrain patterning and expression of opsins. Based on the lack of predicted protein-coding changes and a deletion of a conserved element upstream of the transcription start site, a cis-regulatory mutation is proposed as the basis of the reduced expression of six7 in ljrp23ahub. Comparison of the phenotypes of the hypomorphic and knock-out alleles provides evidence of two independent roles in photoreceptor development. EdU and PH3 labeling show that the increase in rod number is associated with extended mitosis of photoreceptor progenitors, and TUNEL suggests that the lack of green-sensitive cones is the result of cell death of the cone precursor. These data add six7 to the small but growing list of essential genes for specification and patterning of photoreceptors in non-mammalian vertebrates, and highlight alterations in transcriptional regulation as a potential source of photoreceptor variation

Localization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice

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Sahly, Iman; Dufour, Eric; Schietroma, Cataldo; Michel, Vincent; Bahloul, Amel; Perfettini, Isabelle; Pepermans, Elise; Estivalet, Amrit; Carette, Diane; Aghaie, Asadollah; Ebermann, Inga; Lelli, Andrea; Iribarne, Maria; Hardelin, Jean-Pierre; Weil, Dominique; Sahel, José-Alain

2012-01-01

The mechanisms underlying retinal dystrophy in Usher syndrome type I (USH1) remain unknown because mutant mice lacking any of the USH1 proteins—myosin VIIa, harmonin, cadherin-23, protocadherin-15, sans—do not display retinal degeneration. We found here that, in macaque photoreceptor cells, all USH1 proteins colocalized at membrane interfaces (i) between the inner and outer segments in rods and (ii) between the microvillus-like calyceal processes and the outer segment basolateral region in rods and cones. This pattern, conserved in humans and frogs, was mediated by the formation of an USH1 protein network, which was associated with the calyceal processes from the early embryonic stages of outer segment growth onwards. By contrast, mouse photoreceptors lacked calyceal processes and had no USH1 proteins at the inner–outer segment interface. We suggest that USH1 proteins form an adhesion belt around the basolateral region of the photoreceptor outer segment in humans, and that defects in this structure cause the retinal degeneration in USH1 patients. PMID:23045546

Different effects of valproic acid on photoreceptor loss in Rd1 and Rd10 retinal degeneration mice.

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Mitton, Kenneth P; Guzman, Alvaro E; Deshpande, Mrinalini; Byrd, David; DeLooff, Camryn; Mkoyan, Kristina; Zlojutro, Paul; Wallace, Adrianne; Metcalf, Brandon; Laux, Kirsten; Sotzen, Jason; Tran, Trung

2014-01-01

The histone-deacetylase inhibitor activity of valproic acid (VPA) was discovered after VPA's adoption as an anticonvulsant. This generated speculation for VPA's potential to increase the expression of neuroprotective genes. Clinical trials for retinitis pigmentosa (RP) are currently active, testing VPA's potential to reduce photoreceptor loss; however, we lack information regarding the effects of VPA on available mammalian models of retinal degeneration, nor do we know if retinal gene expression is perturbed by VPA in a predictable way. Thus, we examined the effects of systemic VPA on neurotrophic factor and Nrl-related gene expression in the mouse retina and compared VPA's effects on the rate of photoreceptor loss in two strains of mice, Pde6b(rd1/rd1) and Pde6b(rd10/rd10) . The expression of Bdnf, Gdnf, Cntf, and Fgf2 was measured by quantitative PCR after single and multiple doses of VPA (intraperitoneal) in wild-type and Pde6b(rd1/rd1) mice. Pde6b(rd1/rd1) mice were treated with daily doses of VPA during the period of rapid photoreceptor loss. Pde6b(rd10/rd10) mice were also treated with systemic VPA to compare in a partial loss-of-function model. Retinal morphology was assessed by virtual microscopy or spectral-domain optical coherence tomography (SD-OCT). Full-field and focal electroretinography (ERG) analysis were employed with Pde6b(rd10/rd10) mice to measure retinal function. In wild-type postnatal mice, a single VPA dose increased the expression of Bdnf and Gdnf in the neural retina after 18 h, while the expression of Cntf was reduced by 70%. Daily dosing of wild-type mice from postnatal day P17 to P28 resulted in smaller increases in Bdnf and Gdnf expression, normal Cntf expression, and reduced Fgf2 expression (25%). Nrl gene expression was decreased by 50%, while Crx gene expression was not affected. Rod-specific expression of Mef2c and Nr2e3 was decreased substantially by VPA treatment, while Rhodopsin and Pde6b gene expression was normal at P28. Daily

Efficient photoreceptor-targeted gene expression in vivo by recombinant adeno-associated virus.

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Flannery, J G; Zolotukhin, S; Vaquero, M I; LaVail, M M; Muzyczka, N; Hauswirth, W W

1997-06-24

We describe a general approach for achieving efficient and cell type-specific expression of exogenous genes in photoreceptor cells of the mammalian retina. Recombinant adeno-associated virus (rAAV) vectors were used to transfer the bacterial lacZ gene or a synthetic green fluorescent protein gene (gfp) to mouse or rat retinas after injection into the subretinal space. Using a proximal murine rod opsin promoter (+86 to -385) to drive expression, reporter gene product was found exclusively in photoreceptors, not in any other retinal cell type or in the adjacent retinal pigment epithelium. GFP-expressing photoreceptors typically encompassed 10-20% of the total retinal area after a single 2-microl injection. Photoreceptors were transduced with nearly 100% efficiency in the region directly surrounding the injection site. We estimate approximately 2.5 million photoreceptors were transduced as a result of the single subretinal inoculation. This level of gene transfer and expression suggests the feasibility of genetic therapy for retinal disease. The gfp-containing rAAV stock was substantially free of both adenovirus and wild-type AAV, as judged by plaque assay and infectious center assay, respectively. Thus, highly purified, helper virus-free rAAV vectors can achieve high-frequency tissue-specific transduction of terminally differentiated, postmitotic photoreceptor cells.

Rod photoreceptors express GPR55 in the adult vervet monkey retina

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Bouskila, Joseph; Javadi, Pasha; Casanova, Christian

2013-01-01

. Yet, its formal classification is still a matter of debate. CB1R and CB2R expression patterns are well described for rodent and monkey retinas. In the monkey retina, CB1R has been localized in its neural (cone photoreceptor, horizontal, bipolar, amacrine and ganglion cells) and CB2R in glial...

Bipolar Cell-Photoreceptor Connectivity in the Zebrafish (Danio rerio) Retina

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Li, Yong N.; Tsujimura, Taro; Kawamura, Shoji; Dowling, John E.

2013-01-01

Bipolar cells convey luminance, spatial and color information from photoreceptors to amacrine and ganglion cells. We studied the photoreceptor connectivity of 321 bipolar cells in the adult zebrafish retina. 1,1'-Dioctadecyl-3,3,3',3'-tetramethylindocarbocyanine perchlorate (DiI) was inserted into whole-mounted transgenic zebrafish retinas to label bipolar cells. The photoreceptors that connect to these DiI-labeled cells were identified by transgenic fluorescence or their positions relative to the fluorescent cones, as cones are arranged in a highly-ordered mosaic: rows of alternating blue- (B) and ultraviolet-sensitive (UV) single cones alternate with rows of red- (R) and green-sensitive (G) double cones. Rod terminals intersperse among cone terminals. As many as 18 connectivity subtypes were observed, 9 of which – G, GBUV, RG, RGB, RGBUV, RGRod, RGBRod, RGBUVRod and RRod bipolar cells – accounted for 96% of the population. Based on their axon terminal stratification, these bipolar cells could be further sub-divided into ON, OFF, and ON-OFF cells. The dendritic spread size, soma depth and size, and photoreceptor connections of the 308 bipolar cells within the 9 common connectivity subtypes were determined, and their dendritic tree morphologies and axonal stratification patterns compared. We found that bipolar cells with the same axonal stratification patterns could have heterogeneous photoreceptor connectivity whereas bipolar cells with the same dendritic tree morphology usually had the same photoreceptor connectivity, although their axons might stratify on different levels. PMID:22907678

Pineal photoreceptor cells are required for maintaining the circadian rhythms of behavioral visual sensitivity in zebrafish.

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Xinle Li

Full Text Available In non-mammalian vertebrates, the pineal gland functions as the central pacemaker that regulates the circadian rhythms of animal behavior and physiology. We generated a transgenic zebrafish line [Tg(Gnat2:gal4-VP16/UAS:nfsB-mCherry] in which the E. coli nitroreductase is expressed in pineal photoreceptor cells. In developing embryos and young adults, the transgene is expressed in both retinal and pineal photoreceptor cells. During aging, the expression of the transgene in retinal photoreceptor cells gradually diminishes. By 8 months of age, the Gnat2 promoter-driven nitroreductase is no longer expressed in retinal photoreceptor cells, but its expression in pineal photoreceptor cells persists. This provides a tool for selective ablation of pineal photoreceptor cells, i.e., by treatments with metronidazole. In the absence of pineal photoreceptor cells, the behavioral visual sensitivity of the fish remains unchanged; however, the circadian rhythms of rod and cone sensitivity are diminished. Brief light exposures restore the circadian rhythms of behavioral visual sensitivity. Together, the data suggest that retinal photoreceptor cells respond to environmental cues and are capable of entraining the circadian rhythms of visual sensitivity; however, they are insufficient for maintaining the rhythms. Cellular signals from the pineal photoreceptor cells may be required for maintaining the circadian rhythms of visual sensitivity.

Loss and gain of cone types in vertebrate ciliary photoreceptor evolution.

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Musser, Jacob M; Arendt, Detlev

2017-11-01

Ciliary photoreceptors are a diverse cell type family that comprises the rods and cones of the retina and other related cell types such as pineal photoreceptors. Ciliary photoreceptor evolution has been dynamic during vertebrate evolution with numerous gains and losses of opsin and phototransduction genes, and changes in their expression. For example, early mammals lost all but two cone opsins, indicating loss of cone receptor types in response to nocturnal lifestyle. Our review focuses on the comparison of specifying transcription factors and cell type-specific transcriptome data in vertebrate retinae to build and test hypotheses on ciliary photoreceptor evolution. Regarding cones, recent data reveal that a combination of factors specific for long-wavelength sensitive opsin (Lws)- cones in non-mammalian vertebrates (Thrb and Rxrg) is found across all differentiating cone photoreceptors in mice. This suggests that mammalian ancestors lost all but one ancestral cone type, the Lws-cone. We test this hypothesis by a correlation analysis of cone transcriptomes in mouse and chick, and find that, indeed, transcriptomes of all mouse cones are most highly correlated to avian Lws-cones. These findings underscore the importance of specifying transcription factors in tracking cell type evolution, and shed new light on the mechanisms of cell type loss and gain in retina evolution. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Integrity of the cone photoreceptor mosaic in oligocone trichromacy

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Michaelides, Michel; Rha, Jungtae; Dees, Elise W

2011-01-01

Oligocone trichromacy (OT) is an unusual cone dysfunction syndrome characterized by reduced visual acuity, mild photophobia, reduced amplitude of the cone electroretinogram with normal rod responses, normal fundus appearance, and normal or near-normal color vision. It has been proposed that these...... that these patients have a reduced number of normal functioning cones (oligocone). This paper has sought to evaluate the integrity of the cone photoreceptor mosaic in four patients previously described as having OT....

NADPH Oxidase Contributes to Photoreceptor Degeneration in Constitutively Active RAC1 Mice

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Song, Hongman; Vijayasarathy, Camasamudram; Zeng, Yong; Marangoni, Dario; Bush, Ronald A.; Wu, Zhijian; Sieving, Paul A.

2016-01-01

Purpose The active form of small GTPase RAC1 is required for activation of NADPH oxidase (NOX), which in turn generates reactive oxygen species (ROS) in nonphagocytic cells. We explored whether NOX-induced oxidative stress contributes to rod degeneration in retinas expressing constitutively active (CA) RAC1. Methods Transgenic (Tg)–CA-RAC1 mice were given apocynin (10 mg/kg, intraperitoneal), a NOX inhibitor, or vehicle daily for up to 13 weeks. Superoxide production and oxidative damage were assessed by dihydroethidium staining and by protein carbonyls and malondialdehyde levels, respectively. Outer nuclear layer (ONL) cells were counted and electroretinogram (ERG) amplitudes measured in Tg-CA-RAC1 mice. Outer nuclear layer cells were counted in wild-type (WT) mice after transfer of CA-Rac1 gene by subretinal injection of AAV8-pOpsin-CA Rac1-GFP. Results Transgenic-CA-RAC1 retinas had significantly fewer photoreceptor cells and more apoptotic ONL cells than WT controls from postnatal week (Pw) 3 to Pw13. Superoxide accumulation and protein and lipid oxidation were increased in Tg-CA-RAC1 retinas and were reduced in mice treated with apocynin. Apocynin reduced the loss of photoreceptors and increased the rod ERG a- and b-wave amplitudes when compared with vehicle-injected transgenic controls. Photoreceptor loss was also observed in regions of adult WT retina transduced with AAV8-pOpsin-CA Rac1-GFP but not in neighboring regions that were not transduced or in AAV8-pOpsin-GFP–transduced retinas. Conclusions Constitutively active RAC1 promotes photoreceptor cell death by oxidative damage that occurs, at least partially, through NOX-induced ROS. Reactive oxygen species are likely involved in multiple forms of retinal degenerations, and our results support investigating RAC1 inhibition as a therapeutic approach that targets this disease pathway. PMID:27233035

Correlated and uncorrelated invisible temporal white noise alters mesopic rod signaling.

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Hathibelagal, Amithavikram R; Feigl, Beatrix; Kremers, Jan; Zele, Andrew J

2016-03-01

We determined how rod signaling at mesopic light levels is altered by extrinsic temporal white noise that is correlated or uncorrelated with the activity of one (magnocellular, parvocellular, or koniocellular) postreceptoral pathway. Rod and cone photoreceptor excitations were independently controlled using a four-primary photostimulator. Psychometric (Weibull) functions were measured for incremental rod pulses (50 to 250 ms) in the presence (or absence; control) of perceptually invisible subthreshold extrinsic noise. Uncorrelated (rod) noise facilitates rod detection. Correlated postreceptoral pathway noise produces differential changes in rod detection thresholds and decreases the slope of the psychometric functions. We demonstrate that invisible extrinsic noise changes rod-signaling characteristics within the three retinogeniculate pathways at mesopic illumination depending on the temporal profile of the rod stimulus and the extrinsic noise type.

Bifurcation analysis of a photoreceptor interaction model for Retinitis Pigmentosa

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Camacho, Erika T.; Radulescu, Anca; Wirkus, Stephen

2016-09-01

Retinitis Pigmentosa (RP) is the term used to describe a diverse set of degenerative eye diseases affecting the photoreceptors (rods and cones) in the retina. This work builds on an existing mathematical model of RP that focused on the interaction of the rods and cones. We non-dimensionalize the model and examine the stability of the equilibria. We then numerically investigate other stable modes that are present in the system for various parameter values and relate these modes to the original problem. Our results show that stable modes exist for a wider range of parameter values than the stability of the equilibrium solutions alone, suggesting that additional approaches to preventing cone death may exist.

Analysis of transcriptional regulatory pathways of photoreceptor genes by expression profiling of the Otx2-deficient retina.

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Omori, Yoshihiro; Katoh, Kimiko; Sato, Shigeru; Muranishi, Yuki; Chaya, Taro; Onishi, Akishi; Minami, Takashi; Fujikado, Takashi; Furukawa, Takahisa

2011-01-01

In the vertebrate retina, the Otx2 transcription factor plays a crucial role in the cell fate determination of both rod and cone photoreceptors. We previously reported that Otx2 conditional knockout (CKO) mice exhibited a total absence of rods and cones in the retina due to their cell fate conversion to amacrine-like cells. In order to investigate the entire transcriptome of the Otx2 CKO retina, we compared expression profile of Otx2 CKO and wild-type retinas at P1 and P12 using microarray. We observed that expression of 101- and 1049-probe sets significantly decreased in the Otx2 CKO retina at P1 and P12, respectively, whereas, expression of 3- and 4149-probe sets increased at P1 and P12, respectively. We found that expression of genes encoding transcription factors involved in photoreceptor development, including Crx, Nrl, Nr2e3, Esrrb, and NeuroD, was markedly down-regulated in the Otx2 CKO at both P1 and P12. Furthermore, we identified three human retinal disease loci mapped in close proximity to certain down-regulated genes in the Otx2 CKO retina including Ccdc126, Tnfsf13 and Pitpnm1, suggesting that these genes are possibly responsible for these diseases. These transcriptome data sets of the Otx2 CKO retina provide a resource on developing rods and cones to further understand the molecular mechanisms underlying photoreceptor development, function and disease.

Analysis of transcriptional regulatory pathways of photoreceptor genes by expression profiling of the Otx2-deficient retina.

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Yoshihiro Omori

Full Text Available In the vertebrate retina, the Otx2 transcription factor plays a crucial role in the cell fate determination of both rod and cone photoreceptors. We previously reported that Otx2 conditional knockout (CKO mice exhibited a total absence of rods and cones in the retina due to their cell fate conversion to amacrine-like cells. In order to investigate the entire transcriptome of the Otx2 CKO retina, we compared expression profile of Otx2 CKO and wild-type retinas at P1 and P12 using microarray. We observed that expression of 101- and 1049-probe sets significantly decreased in the Otx2 CKO retina at P1 and P12, respectively, whereas, expression of 3- and 4149-probe sets increased at P1 and P12, respectively. We found that expression of genes encoding transcription factors involved in photoreceptor development, including Crx, Nrl, Nr2e3, Esrrb, and NeuroD, was markedly down-regulated in the Otx2 CKO at both P1 and P12. Furthermore, we identified three human retinal disease loci mapped in close proximity to certain down-regulated genes in the Otx2 CKO retina including Ccdc126, Tnfsf13 and Pitpnm1, suggesting that these genes are possibly responsible for these diseases. These transcriptome data sets of the Otx2 CKO retina provide a resource on developing rods and cones to further understand the molecular mechanisms underlying photoreceptor development, function and disease.

Material Exchange in Photoreceptor Transplantation: Updating Our Understanding of Donor/Host Communication and the Future of Cell Engraftment Science.

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Nickerson, Philip E B; Ortin-Martinez, Arturo; Wallace, Valerie A

2018-01-01

Considerable research effort has been invested into the transplantation of mammalian photoreceptors into healthy and degenerating mouse eyes. Several platforms of rod and cone fluorescent reporting have been central to refining the isolation, purification and transplantation of photoreceptors. The tracking of engrafted cells, including identifying the position, morphology and degree of donor cell integration post-transplant is highly dependent on the use of fluorescent protein reporters. Improvements in imaging and analysis of transplant recipients have revealed that donor cell fluorescent reporters can transfer into host tissue though a process termed material exchange (ME). This recent discovery has chaperoned a new era of interpretation when reviewing the field's use of dissociated donor cell preparations, and has prompted scientists to re-examine how we use and interpret the information derived from fluorescence-based tracking tools. In this review, we describe the status of our understanding of ME in photoreceptor transplantation. In addition, we discuss the impact of this discovery on several aspects of historical rod and cone transplantation data, and provide insight into future standards and approaches to advance the field of cell engraftment.

Protein and signaling networks in vertebrate photoreceptor cells

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Karl-Wilhelm eKoch

2015-11-01

Full Text Available Vertebrate photoreceptor cells are exquisite light detectors operating under very dim and bright illumination. The photoexcitation and adaptation machinery in photoreceptor cells consists of protein complexes that can form highly ordered supramolecular structures and control the homeostasis and mutual dependence of the secondary messengers cGMP and Ca2+. The visual pigment in rod photoreceptors, the G protein-coupled receptor rhodopsin is organized in tracks of dimers thereby providing a signaling platform for the dynamic scaffolding of the G protein transducin. Illuminated rhodopsin is turned off by phosphorylation catalyzed by rhodopsin kinase GRK1 under control of Ca2+-recoverin. The GRK1 protein complex partly assembles in lipid raft structures, where shutting off rhodopsin seems to be more effective. Re-synthesis of cGMP is another crucial step in the recovery of the photoresponse after illumination. It is catalyzed by membrane bound sensory guanylate cyclases and is regulated by specific neuronal Ca2+-sensor proteins called GCAPs. At least one guanylate cyclase (ROS-GC1 was shown to be part of a multiprotein complex having strong interactions with the cytoskeleton and being controlled in a multimodal Ca2+-dependent fashion. The final target of the cGMP signaling cascade is a cyclic nucleotide-gated channel that is a hetero-oligomeric protein located in the plasma membrane and interacting with accessory proteins in highly organized microdomains. We summarize results and interpretations of findings related to the inhomogeneous organization of signaling units in photoreceptor outer segments.

Acute Zonal Cone Photoreceptor Outer Segment Loss.

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Aleman, Tomas S; Sandhu, Harpal S; Serrano, Leona W; Traband, Anastasia; Lau, Marisa K; Adamus, Grazyna; Avery, Robert A

2017-05-01

The diagnostic path presented narrows down the cause of acute vision loss to the cone photoreceptor outer segment and will refocus the search for the cause of similar currently idiopathic conditions. To describe the structural and functional associations found in a patient with acute zonal occult photoreceptor loss. A case report of an adolescent boy with acute visual field loss despite a normal fundus examination performed at a university teaching hospital. Results of a complete ophthalmic examination, full-field flash electroretinography (ERG) and multifocal ERG, light-adapted achromatic and 2-color dark-adapted perimetry, and microperimetry. Imaging was performed with spectral-domain optical coherence tomography (SD-OCT), near-infrared (NIR) and short-wavelength (SW) fundus autofluorescence (FAF), and NIR reflectance (REF). The patient was evaluated within a week of the onset of a scotoma in the nasal field of his left eye. Visual acuity was 20/20 OU, and color vision was normal in both eyes. Results of the fundus examination and of SW-FAF and NIR-FAF imaging were normal in both eyes, whereas NIR-REF imaging showed a region of hyporeflectance temporal to the fovea that corresponded with a dense relative scotoma noted on light-adapted static perimetry in the left eye. Loss in the photoreceptor outer segment detected by SD-OCT co-localized with an area of dense cone dysfunction detected on light-adapted perimetry and multifocal ERG but with near-normal rod-mediated vision according to results of 2-color dark-adapted perimetry. Full-field flash ERG findings were normal in both eyes. The outer nuclear layer and inner retinal thicknesses were normal. Localized, isolated cone dysfunction may represent the earliest photoreceptor abnormality or a distinct entity within the acute zonal occult outer retinopathy complex. Acute zonal occult outer retinopathy should be considered in patients with acute vision loss and abnormalities on NIR-REF imaging, especially if

Nrl-Cre transgenic mouse mediates loxP recombination in developing rod photoreceptors.

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Brightman, Diana S; Razafsky, David; Potter, Chloe; Hodzic, Didier; Chen, Shiming

2016-03-01

The developing mouse retina is a tractable model for studying neurogenesis and differentiation. Although transgenic Cre mouse lines exist to mediate conditional genetic manipulations in developing mouse retinas, none of them act specifically in early developing rods. For conditional genetic manipulations of developing retinas, a Nrl-Cre mouse line in which the Nrl promoter drives expression of Cre in rod precursors was created. The results showed that Nrl-Cre expression was specific to the retina where it drives rod-specific recombination with a temporal pattern similar to endogenous Nrl expression during retinal development. This Nrl-Cre transgene does not negatively impact retinal structure and function. Taken together, the data suggested that the Nrl-Cre mouse line was a valuable tool to drive Cre-mediated recombination specifically in developing rods. © 2016 Wiley Periodicals, Inc.

Correlated cone noise decreases rod signal contributions to the post-receptoral pathways.

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Hathibelagal, Amithavikram R; Feigl, Beatrix; Zele, Andrew J

2018-04-01

This study investigated how invisible extrinsic temporal white noise that correlates with the activity of one of the three [magnocellular (MC), parvocellular (PC), or koniocellular (KC)] post-receptoral pathways alters mesopic rod signaling. A four-primary photostimulator provided independent control of the rod and three cone photoreceptor excitations. The rod contributions to the three post-receptoral pathways were estimated by perceptually matching a 20% contrast rod pulse by independently varying the LMS (MC pathway), +L-M (PC pathway), and S-cone (KC pathway) excitations. We show that extrinsic cone noise caused a predominant decrease in the overall magnitude and ratio of the rod contributions to each pathway. Thus, the relative cone activity in the post-receptoral pathways determines the relative mesopic rod inputs to each pathway.

Cone and Rod Loss in Stargardt Disease Revealed by Adaptive Optics Scanning Light Ophthalmoscopy

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Song, Hongxin; Rossi, Ethan A.; Latchney, Lisa; Bessette, Angela; Stone, Edwin; Hunter, Jennifer J.; Williams, David R.; Chung, Mina

2015-01-01

Importance Stargardt disease (STGD1) is characterized by macular atrophy and flecks in the retinal pigment epithelium. The causative ABCA4 gene encodes a protein localizing to photoreceptor outer segments. The pathologic steps by which ABCA4 mutations lead to clinically detectable retinal pigment epithelium changes remain unclear. We investigated early STGD1 using adaptive optics scanning light ophthalmoscopy. Observations Adaptive optics scanning light ophthalmoscopy imaging of 2 brothers with early STGD1 and their unaffected parents was compared with conventional imaging. Cone and rod spacing were increased in both patients (P optics scanning light ophthalmoscopy reveals increased cone and rod spacing in areas that appear normal in conventional images, suggesting that photoreceptor loss precedes clinically detectable retinal pigment epithelial disease in STGD1. PMID:26247787

Visual cycle and its metabolic support in gecko photoreceptors.

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Kolesnikov, A V; Ala-Laurila, P; Shukolyukov, S A; Crouch, R K; Wiggert, B; Estevez, M E; Govardovskii, V I; Cornwall, M C

2007-02-01

Photoreceptors of nocturnal geckos are transmuted cones that acquired rod morphological and physiological properties but retained cone-type phototransduction proteins. We have used microspectrophotometry and microfluorometry of solitary isolated green-sensitive photoreceptors of Tokay gecko to study the initial stages of the visual cycle within these cells. These stages are the photolysis of the visual pigment, the reduction of all-trans retinal to all-trans retinol, and the clearance of all-trans retinol from the outer segment (OS) into the interphotoreceptor space. We show that the rates of decay of metaproducts (all-trans retinal release) and retinal-to-retinol reduction are intermediate between those of typical rods and cones. Clearance of retinol from the OS proceeds at a rate that is typical of rods and is greatly accelerated by exposure to interphotoreceptor retinoid-binding protein, IRBP. The rate of retinal release from metaproducts is independent of the position within the OS, while its conversion to retinol is strongly spatially non-uniform, being the fastest at the OS base and slowest at the tip. This spatial gradient of retinol production is abolished by dialysis of saponin-permeabilized OSs with exogenous NADPH or substrates for its production by the hexose monophosphate pathway (NADP+glucose-6-phosphate or 6-phosphogluconate, glucose-6-phosphate alone). Following dialysis by these agents, retinol production is accelerated by several-fold compared to the fastest rates observed in intact cells in standard Ringer solution. We propose that the speed of retinol production is set by the availability of NADPH which in turn depends on ATP supply within the outer segment. We also suggest that principal source of this ATP is from mitochondria located within the ellipsoid region of the inner segment.

Cone rod dystrophies

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Hamel, Christian P

2007-01-01

Cone rod dystrophies (CRDs) (prevalence 1/40,000) are inherited retinal dystrophies that belong to the group of pigmentary retinopathies. CRDs are characterized by retinal pigment deposits visible on fundus examination, predominantly localized to the macular region. In contrast to typical retinitis pigmentosa (RP), also called the rod cone dystrophies (RCDs) resulting from the primary loss in rod photoreceptors and later followed by the secondary loss in cone photoreceptors, CRDs reflect the opposite sequence of events. CRD is characterized by primary cone involvement, or, sometimes, by concomitant loss of both cones and rods that explains the predominant symptoms of CRDs: decreased visual acuity, color vision defects, photoaversion and decreased sensitivity in the central visual field, later followed by progressive loss in peripheral vision and night blindness. The clinical course of CRDs is generally more severe and rapid than that of RCDs, leading to earlier legal blindness and disability. At end stage, however, CRDs do not differ from RCDs. CRDs are most frequently non syndromic, but they may also be part of several syndromes, such as Bardet Biedl syndrome and Spinocerebellar Ataxia Type 7 (SCA7). Non syndromic CRDs are genetically heterogeneous (ten cloned genes and three loci have been identified so far). The four major causative genes involved in the pathogenesis of CRDs are ABCA4 (which causes Stargardt disease and also 30 to 60% of autosomal recessive CRDs), CRX and GUCY2D (which are responsible for many reported cases of autosomal dominant CRDs), and RPGR (which causes about 2/3 of X-linked RP and also an undetermined percentage of X-linked CRDs). It is likely that highly deleterious mutations in genes that otherwise cause RP or macular dystrophy may also lead to CRDs. The diagnosis of CRDs is based on clinical history, fundus examination and electroretinogram. Molecular diagnosis can be made for some genes, genetic counseling is always advised. Currently

Cone rod dystrophies

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Hamel Christian P

2007-02-01

Full Text Available Abstract Cone rod dystrophies (CRDs (prevalence 1/40,000 are inherited retinal dystrophies that belong to the group of pigmentary retinopathies. CRDs are characterized by retinal pigment deposits visible on fundus examination, predominantly localized to the macular region. In contrast to typical retinitis pigmentosa (RP, also called the rod cone dystrophies (RCDs resulting from the primary loss in rod photoreceptors and later followed by the secondary loss in cone photoreceptors, CRDs reflect the opposite sequence of events. CRD is characterized by primary cone involvement, or, sometimes, by concomitant loss of both cones and rods that explains the predominant symptoms of CRDs: decreased visual acuity, color vision defects, photoaversion and decreased sensitivity in the central visual field, later followed by progressive loss in peripheral vision and night blindness. The clinical course of CRDs is generally more severe and rapid than that of RCDs, leading to earlier legal blindness and disability. At end stage, however, CRDs do not differ from RCDs. CRDs are most frequently non syndromic, but they may also be part of several syndromes, such as Bardet Biedl syndrome and Spinocerebellar Ataxia Type 7 (SCA7. Non syndromic CRDs are genetically heterogeneous (ten cloned genes and three loci have been identified so far. The four major causative genes involved in the pathogenesis of CRDs are ABCA4 (which causes Stargardt disease and also 30 to 60% of autosomal recessive CRDs, CRX and GUCY2D (which are responsible for many reported cases of autosomal dominant CRDs, and RPGR (which causes about 2/3 of X-linked RP and also an undetermined percentage of X-linked CRDs. It is likely that highly deleterious mutations in genes that otherwise cause RP or macular dystrophy may also lead to CRDs. The diagnosis of CRDs is based on clinical history, fundus examination and electroretinogram. Molecular diagnosis can be made for some genes, genetic counseling is

A novel culture method reveals unique neural stem/progenitors in mature porcine iris tissues that differentiate into neuronal and rod photoreceptor-like cells.

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Royall, Lars N; Lea, Daniel; Matsushita, Tamami; Takeda, Taka-Aki; Taketani, Shigeru; Araki, Masasuke

2017-11-15

Iris neural stem/progenitor cells from mature porcine eyes were investigated using a new protocol for tissue culture, which consists of dispase treatment and Matrigel embedding. We used a number of culture conditions and found an intense differentiation of neuronal cells from both the iris pigmented epithelial (IPE) cells and the stroma tissue cells. Rod photoreceptor-like cells were also observed but mostly in a later stage of culture. Neuronal differentiation does not require any additives such as fetal bovine serum or FGF2, although FGF2 and IGF2 appeared to promote neural differentiation in the IPE cultures. Furthermore, the stroma-derived cells were able to be maintained in vitro indefinitely. The evolutionary similarity between humans and domestic pigs highlight the potential for this methodology in the modeling of human diseases and characterizing human ocular stem cells. Copyright © 2017 Elsevier B.V. All rights reserved.

Material Exchange in Photoreceptor Transplantation: Updating Our Understanding of Donor/Host Communication and the Future of Cell Engraftment Science

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Philip E. B. Nickerson

2018-03-01

Full Text Available Considerable research effort has been invested into the transplantation of mammalian photoreceptors into healthy and degenerating mouse eyes. Several platforms of rod and cone fluorescent reporting have been central to refining the isolation, purification and transplantation of photoreceptors. The tracking of engrafted cells, including identifying the position, morphology and degree of donor cell integration post-transplant is highly dependent on the use of fluorescent protein reporters. Improvements in imaging and analysis of transplant recipients have revealed that donor cell fluorescent reporters can transfer into host tissue though a process termed material exchange (ME. This recent discovery has chaperoned a new era of interpretation when reviewing the field’s use of dissociated donor cell preparations, and has prompted scientists to re-examine how we use and interpret the information derived from fluorescence-based tracking tools. In this review, we describe the status of our understanding of ME in photoreceptor transplantation. In addition, we discuss the impact of this discovery on several aspects of historical rod and cone transplantation data, and provide insight into future standards and approaches to advance the field of cell engraftment.

Mfsd2a Is a Transporter for the Essential ω-3 Fatty Acid Docosahexaenoic Acid (DHA) in Eye and Is Important for Photoreceptor Cell Development.

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Wong, Bernice H; Chan, Jia Pei; Cazenave-Gassiot, Amaury; Poh, Rebecca W; Foo, Juat Chin; Galam, Dwight L A; Ghosh, Sujoy; Nguyen, Long N; Barathi, Veluchamy A; Yeo, Sia W; Luu, Chi D; Wenk, Markus R; Silver, David L

2016-05-13

Eye photoreceptor membrane discs in outer rod segments are highly enriched in the visual pigment rhodopsin and the ω-3 fatty acid docosahexaenoic acid (DHA). The eye acquires DHA from blood, but transporters for DHA uptake across the blood-retinal barrier or retinal pigment epithelium have not been identified. Mfsd2a is a newly described sodium-dependent lysophosphatidylcholine (LPC) symporter expressed at the blood-brain barrier that transports LPCs containing DHA and other long-chain fatty acids. LPC transport via Mfsd2a has been shown to be necessary for human brain growth. Here we demonstrate that Mfsd2a is highly expressed in retinal pigment epithelium in embryonic eye, before the development of photoreceptors, and is the primary site of Mfsd2a expression in the eye. Eyes from whole body Mfsd2a-deficient (KO) mice, but not endothelium-specific Mfsd2a-deficient mice, were DHA-deficient and had significantly reduced LPC/DHA transport in vivo Fluorescein angiography indicated normal blood-retinal barrier function. Histological and electron microscopic analysis indicated that Mfsd2a KO mice exhibited a specific reduction in outer rod segment length, disorganized outer rod segment discs, and mislocalization of and reduction in rhodopsin early in postnatal development without loss of photoreceptors. Minor photoreceptor cell loss occurred in adult Mfsd2a KO mice, but electroretinography indicated visual function was normal. The developing eyes of Mfsd2a KO mice had activated microglia and up-regulation of lipogenic and cholesterogenic genes, likely adaptations to loss of LPC transport. These findings identify LPC transport via Mfsd2a as an important pathway for DHA uptake in eye and for development of photoreceptor membrane discs. © 2016 by The American Society for Biochemistry and Molecular Biology, Inc.

Calcium channel-dependent molecular maturation of photoreceptor synapses.

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Nawal Zabouri

Full Text Available Several studies have shown the importance of calcium channels in the development and/or maturation of synapses. The Ca(V1.4(α(1F knockout mouse is a unique model to study the role of calcium channels in photoreceptor synapse formation. It features abnormal ribbon synapses and aberrant cone morphology. We investigated the expression and targeting of several key elements of ribbon synapses and analyzed the cone morphology in the Ca(V1.4(α(1F knockout retina. Our data demonstrate that most abnormalities occur after eye opening. Indeed, scaffolding proteins such as Bassoon and RIM2 are properly targeted at first, but their expression and localization are not maintained in adulthood. This indicates that either calcium or the Ca(V1.4 channel, or both are necessary for the maintenance of their normal expression and distribution in photoreceptors. Other proteins, such as Veli3 and PSD-95, also display abnormal expression in rods prior to eye opening. Conversely, vesicle related proteins appear normal. Our data demonstrate that the Ca(V1.4 channel is important for maintaining scaffolding proteins in the ribbon synapse but less vital for proteins related to vesicular release. This study also confirms that in adult retinae, cones show developmental features such as sprouting and synaptogenesis. Overall we present evidence that in the absence of the Ca(V1.4 channel, photoreceptor synapses remain immature and are unable to stabilize.

Calcium channel-dependent molecular maturation of photoreceptor synapses.

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Zabouri, Nawal; Haverkamp, Silke

2013-01-01

Several studies have shown the importance of calcium channels in the development and/or maturation of synapses. The Ca(V)1.4(α(1F)) knockout mouse is a unique model to study the role of calcium channels in photoreceptor synapse formation. It features abnormal ribbon synapses and aberrant cone morphology. We investigated the expression and targeting of several key elements of ribbon synapses and analyzed the cone morphology in the Ca(V)1.4(α(1F)) knockout retina. Our data demonstrate that most abnormalities occur after eye opening. Indeed, scaffolding proteins such as Bassoon and RIM2 are properly targeted at first, but their expression and localization are not maintained in adulthood. This indicates that either calcium or the Ca(V)1.4 channel, or both are necessary for the maintenance of their normal expression and distribution in photoreceptors. Other proteins, such as Veli3 and PSD-95, also display abnormal expression in rods prior to eye opening. Conversely, vesicle related proteins appear normal. Our data demonstrate that the Ca(V)1.4 channel is important for maintaining scaffolding proteins in the ribbon synapse but less vital for proteins related to vesicular release. This study also confirms that in adult retinae, cones show developmental features such as sprouting and synaptogenesis. Overall we present evidence that in the absence of the Ca(V)1.4 channel, photoreceptor synapses remain immature and are unable to stabilize.

Early photoreceptor outer segment loss and retinoschisis in Cohen syndrome.

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Uyhazi, Katherine E; Binenbaum, Gil; Carducci, Nicholas; Zackai, Elaine H; Aleman, Tomas S

2018-06-01

To describe early structural and functional retinal changes in a patient with Cohen syndrome. A 13-month-old Caucasian girl of Irish and Spanish ancestry was noted to have micrognathia and laryngomalacia at birth, which prompted a genetic evaluation that revealed biallelic deletions in COH1 (VPS13B) (a maternally inherited 60-kb deletion involving exons 26-32 and a paternally inherited 3.5-kb deletion within exon 17) consistent with Cohen syndrome. She underwent a complete ophthalmic examination, full-field flash electroretinography and retinal imaging with spectral domain optical coherence tomography. Central vision was central, steady, and maintained. There was bilateral myopic astigmatic refractive error. Fundus exam was notable for dark foveolar pigmentation, but no obvious abnormalities of either eye. Spectral domain optical coherence tomography cross sections through the fovea revealed a normal appearing photoreceptor outer nuclear layer but loss of the interdigitation signal between the photoreceptor outer segments and the apical retinal pigment epithelium. Retinoschisis involving the inner nuclear layer of both eyes and possible ganglion cell layer thinning were also noted. There was a detectable electroretinogram with similarly reduced amplitudes of rod- (white, 0.01 cd.s.m -2 ) and cone-mediated (3 cd.s.m -2 , 30 Hz) responses. Photoreceptor outer segment abnormalities and retinoschisis may represent the earliest structural retinal change detected by spectral domain optical coherence tomography in patients with Cohen syndrome, suggesting a complex pathophysiology with primary involvement of the photoreceptor cilium and disorganization of the structural integrity of the inner retina.

Suppressing thyroid hormone signaling preserves cone photoreceptors in mouse models of retinal degeneration

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Ma, Hongwei; Thapa, Arjun; Morris, Lynsie; Redmond, T. Michael; Baehr, Wolfgang; Ding, Xi-Qin

2014-01-01

Photoreceptors degenerate in a wide array of hereditary retinal diseases and age-related macular degeneration. There is currently no treatment available for retinal degenerations. While outnumbered roughly 20:1 by rods in the human retina, it is the cones that mediate color vision and visual acuity, and their survival is critical for vision. In this communication, we investigate whether thyroid hormone (TH) signaling affects cone viability in retinal degeneration mouse models. TH signaling is...

The influence of photoreceptor size and distribution on optical sensitivity in the eyes of lanternfishes (Myctophidae)

KAUST Repository

Busserolles, Fanny de; Fitzpatrick, John L.; Marshall, N. Justin; Collin, Shaun P.

2014-01-01

The mesopelagic zone of the deep-sea (200-1000 m) is characterised by exponentially diminishing levels of downwelling sunlight and by the predominance of bioluminescence emissions. The ability of mesopelagic organisms to detect and behaviourally react to downwelling sunlight and/or bioluminescence will depend on the visual task and ultimately on the eyes and their capacity for detecting low levels of illumination and intermittent point sources of bioluminescent light. In this study, we investigate the diversity of the visual system of the lanternfish (Myctophidae). We focus specifically on the photoreceptor cells by examining their size, arrangement, topographic distribution and contribution to optical sensitivity in 53 different species from 18 genera. We also examine the influence(s) of both phylogeny and ecology on these photoreceptor variables using phylogenetic comparative analyses in order to understand the constraints placed on the visual systems of this large group of mesopelagic fishes at the first stage of retinal processing. We report great diversity in the visual system of the Myctophidae at the level of the photoreceptors. Photoreceptor distribution reveals clear interspecific differences in visual specialisations (areas of high rod photoreceptor density), indicating potential interspecific differences in interactions with prey, predators and/or mates. A great diversity in photoreceptor design (length and diameter) and density is also present. Overall, the myctophid eye is very sensitive compared to other teleosts and each species seems to be specialised for the detection of a specific signal (downwelling light or bioluminescence), potentially reflecting different visual demands for survival. Phylogenetic comparative analyses highlight several relationships between photoreceptor characteristics and the ecological variables tested (depth distribution and luminous tissue patterns). Depth distribution at night was a significant factor in most of the

The influence of photoreceptor size and distribution on optical sensitivity in the eyes of lanternfishes (Myctophidae)

KAUST Repository

Busserolles, Fanny de

2014-06-13

The mesopelagic zone of the deep-sea (200-1000 m) is characterised by exponentially diminishing levels of downwelling sunlight and by the predominance of bioluminescence emissions. The ability of mesopelagic organisms to detect and behaviourally react to downwelling sunlight and/or bioluminescence will depend on the visual task and ultimately on the eyes and their capacity for detecting low levels of illumination and intermittent point sources of bioluminescent light. In this study, we investigate the diversity of the visual system of the lanternfish (Myctophidae). We focus specifically on the photoreceptor cells by examining their size, arrangement, topographic distribution and contribution to optical sensitivity in 53 different species from 18 genera. We also examine the influence(s) of both phylogeny and ecology on these photoreceptor variables using phylogenetic comparative analyses in order to understand the constraints placed on the visual systems of this large group of mesopelagic fishes at the first stage of retinal processing. We report great diversity in the visual system of the Myctophidae at the level of the photoreceptors. Photoreceptor distribution reveals clear interspecific differences in visual specialisations (areas of high rod photoreceptor density), indicating potential interspecific differences in interactions with prey, predators and/or mates. A great diversity in photoreceptor design (length and diameter) and density is also present. Overall, the myctophid eye is very sensitive compared to other teleosts and each species seems to be specialised for the detection of a specific signal (downwelling light or bioluminescence), potentially reflecting different visual demands for survival. Phylogenetic comparative analyses highlight several relationships between photoreceptor characteristics and the ecological variables tested (depth distribution and luminous tissue patterns). Depth distribution at night was a significant factor in most of the

Lack of protein-tyrosine sulfation disrupts photoreceptor outer segment morphogenesis, retinal function and retinal anatomy.

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Sherry, David M; Murray, Anne R; Kanan, Yogita; Arbogast, Kelsey L; Hamilton, Robert A; Fliesler, Steven J; Burns, Marie E; Moore, Kevin L; Al-Ubaidi, Muayyad R

2010-11-01

To investigate the role(s) of protein-tyrosine sulfation in the retina, we examined retinal function and structure in mice lacking tyrosylprotein sulfotransferases (TPST) 1 and 2. Tpst double knockout (DKO; Tpst1(-/-) /Tpst2 (-/-) ) retinas had drastically reduced electroretinographic responses, although their photoreceptors exhibited normal responses in single cell recordings. These retinas appeared normal histologically; however, the rod photoreceptors had ultrastructurally abnormal outer segments, with membrane evulsions into the extracellular space, irregular disc membrane spacing and expanded intradiscal space. Photoreceptor synaptic terminals were disorganized in Tpst DKO retinas, but established ultrastructurally normal synapses, as did bipolar and amacrine cells; however, the morphology and organization of neuronal processes in the inner retina were abnormal. These results indicate that protein-tyrosine sulfation is essential for proper outer segment morphogenesis and synaptic function, but is not critical for overall retinal structure or synapse formation, and may serve broader functions in neuronal development and maintenance. © 2010 The Authors. European Journal of Neuroscience © 2010 Federation of European Neuroscience Societies and Blackwell Publishing Ltd.

Luminescence- and nanoparticle-mediated increase of light absorption by photoreceptor cells: Converting UV light to visible light.

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Li, Lei; Sahi, Sunil K; Peng, Mingying; Lee, Eric B; Ma, Lun; Wojtowicz, Jennifer L; Malin, John H; Chen, Wei

2016-02-10

We developed new optic devices - singly-doped luminescence glasses and nanoparticle-coated lenses that convert UV light to visible light - for improvement of visual system functions. Tb(3+) or Eu(3+) singly-doped borate glasses or CdS-quantum dot (CdS-QD) coated lenses efficiently convert UV light to 542 nm or 613 nm wavelength narrow-band green or red light, or wide-spectrum white light, and thereby provide extra visible light to the eye. In zebrafish (wild-type larvae and adult control animals, retinal degeneration mutants, and light-induced photoreceptor cell degeneration models), the use of Tb(3+) or Eu(3+) doped luminescence glass or CdS-QD coated glass lenses provide additional visible light to the rod and cone photoreceptor cells, and thereby improve the visual system functions. The data provide proof-of-concept for the future development of optic devices for improvement of visual system functions in patients who suffer from photoreceptor cell degeneration or related retinal diseases.

Switch in rod opsin gene expression in the European eel, Anguilla anguilla (L.).

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Hope, A J; Partridge, J C; Hayes, P K

1998-01-01

The rod photoreceptors of the European eel, Anguilla anguilla (L.), alter their wavelength of maximum sensitivity (lambda max) from c.a. 523 nm to c.a. 482 nm at maturation, a switch involving the synthesis of a new visual pigment protein (opsin) that is inserted into the outer segments of existing rods. We artificially induced the switch in rod opsin production by the administration of hormones, and monitored the switch at the level of mRNA accumulation using radiolabelled oligonuleotides that hybridized differently to the two forms of eel rod opsin. The production of the deep-sea form of rod opsin was detected 6 h after the first hormone injection, and the switch in rod opsin expression was complete within four weeks, at which time only the mRNA for the deep-sea opsin was detectable in the retinal cells. It is suggested that this system could be used as a tractable model for studying the regulatory control of opsin gene expression. PMID:9633112

THE STRUCTURE AND CONCENTRATION OF SOLIDS IN PHOTORECEPTOR CELLS STUDIED BY REFRACTOMETRY AND INTERFERENCE MICROSCOPY

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Sidman, Richard L.

1957-01-01

Fragments of freshly obtained retinas of several vertebrate species were studied by refractometry, with reference to the structure of the rods and cones. The findings allowed a reassessment of previous descriptions based mainly on fixed material. The refractometric method was used also to measure the refractice indices and to calculate the concentrations of solids and water in the various cell segments. The main quantitative data were confirmed by interference microscopy. When examined by the method of refractometry the outer segments of freshly prepared retinal rods appear homogeneous. Within a few minutes a single eccentric longitudinal fiber appears, and transverse striations may develop. These changes are attributed to imbibition of water and swelling in structures normally too small for detection by light microscopy. The central "core" of outer segments and the chromophobic disc between outer and inner segments appear to be artifacts resulting from shrinkage during dehydration. The fresh outer segments of cones, and the inner segments of rods and cones also are described and illustrated. The volumes, refractive indices, concentrations of solids, and wet and dry weights of various segments of the photoreceptor cells were tabulated. Rod outer segments of the different species vary more than 100-fold in volume and mass but all have concentrations of solids of 40 to 43 per cent. Cone outer segments contain only about 30 per cent solids. The myoids, paraboloids, and ellipsoids of the inner segments likewise have characteristic refractive indices and concentrations of solids. Some of the limitations and particular virtues of refractometry as a method for quantitative analysis of living cells are discussed in comparison with more conventional biochemical techniques. Also the shapes and refractive indices of the various segments of photoreceptor cells are considered in relation to the absorption and transmission of light. The Stiles-Crawford effect can be accounted

Long-term effects of retinopathy of prematurity (ROP) on rod and rod-driven function.

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Harris, Maureen E; Moskowitz, Anne; Fulton, Anne B; Hansen, Ronald M

2011-02-01

The purpose of this study was to determine whether recovery of scotopic sensitivity occurs in human ROP, as it does in the rat models of ROP. Following a cross-sectional design, scotopic electroretinographic (ERG) responses to full-field stimuli were recorded from 85 subjects with a history of preterm birth. In 39 of these subjects, dark adapted visual threshold was also measured. Subjects were tested post-term as infants (median age 2.5 months) or at older ages (median age 10.5 years) and stratified by severity of ROP: severe, mild, or none. Rod photoreceptor sensitivity, S (ROD), was derived from the a-wave, and post-receptor sensitivity, log σ, was calculated from the b-wave stimulus-response function. Dark adapted visual threshold was measured using a forced-choice preferential procedure. For S (ROD), the deficit from normal for age varied significantly with ROP severity but not with age group. For log σ, in mild ROP, the deficit was smaller in older subjects than in infants, while in severe ROP, the deficit was quite large in both age groups. In subjects who never had ROP, S (ROD) and log σ in both age groups were similar to those in term born controls. Deficits in dark adapted threshold and log σ were correlated in mild but not in severe ROP. The data are evidence that sensitivity of the post-receptor retina improves in those with a history of mild ROP. We speculate that beneficial reorganization of the post-receptor neural circuitry occurs in mild but not in severe ROP.

Machine learning approaches to supporting the identification of photoreceptor-enriched genes based on expression data

Directory of Open Access Journals (Sweden)

Simpson David

2006-03-01

Full Text Available Abstract Background Retinal photoreceptors are highly specialised cells, which detect light and are central to mammalian vision. Many retinal diseases occur as a result of inherited dysfunction of the rod and cone photoreceptor cells. Development and maintenance of photoreceptors requires appropriate regulation of the many genes specifically or highly expressed in these cells. Over the last decades, different experimental approaches have been developed to identify photoreceptor enriched genes. Recent progress in RNA analysis technology has generated large amounts of gene expression data relevant to retinal development. This paper assesses a machine learning methodology for supporting the identification of photoreceptor enriched genes based on expression data. Results Based on the analysis of publicly-available gene expression data from the developing mouse retina generated by serial analysis of gene expression (SAGE, this paper presents a predictive methodology comprising several in silico models for detecting key complex features and relationships encoded in the data, which may be useful to distinguish genes in terms of their functional roles. In order to understand temporal patterns of photoreceptor gene expression during retinal development, a two-way cluster analysis was firstly performed. By clustering SAGE libraries, a hierarchical tree reflecting relationships between developmental stages was obtained. By clustering SAGE tags, a more comprehensive expression profile for photoreceptor cells was revealed. To demonstrate the usefulness of machine learning-based models in predicting functional associations from the SAGE data, three supervised classification models were compared. The results indicated that a relatively simple instance-based model (KStar model performed significantly better than relatively more complex algorithms, e.g. neural networks. To deal with the problem of functional class imbalance occurring in the dataset, two data re

The TRPM1 Channel Is Required for Development of the Rod ON Bipolar Cell-AII Amacrine Cell Pathway in the Retinal Circuit.

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Kozuka, Takashi; Chaya, Taro; Tamalu, Fuminobu; Shimada, Mariko; Fujimaki-Aoba, Kayo; Kuwahara, Ryusuke; Watanabe, Shu-Ichi; Furukawa, Takahisa

2017-10-11

Neurotransmission plays an essential role in neural circuit formation in the central nervous system (CNS). Although neurotransmission has been recently clarified as a key modulator of retinal circuit development, the roles of individual synaptic transmissions are not yet fully understood. In the current study, we investigated the role of neurotransmission from photoreceptor cells to ON bipolar cells in development using mutant mouse lines of both sexes in which this transmission is abrogated. We found that deletion of the ON bipolar cation channel TRPM1 results in the abnormal contraction of rod bipolar terminals and a decreased number of their synaptic connections with amacrine cells. In contrast, these histological alterations were not caused by a disruption of total glutamate transmission due to loss of the ON bipolar glutamate receptor mGluR6 or the photoreceptor glutamate transporter VGluT1. In addition, TRPM1 deficiency led to the reduction of total dendritic length, branch numbers, and cell body size in AII amacrine cells. Activated Goα, known to close the TRPM1 channel, interacted with TRPM1 and induced the contraction of rod bipolar terminals. Furthermore, overexpression of Channelrhodopsin-2 partially rescued rod bipolar cell development in the TRPM1 -/- retina, whereas the rescue effect by a constitutively closed form of TRPM1 was lower than that by the native form. Our results suggest that TRPM1 channel opening is essential for rod bipolar pathway establishment in development. SIGNIFICANCE STATEMENT Neurotransmission has been recognized recently as a key modulator of retinal circuit development in the CNS. However, the roles of individual synaptic transmissions are not yet fully understood. In the current study, we focused on neurotransmission between rod photoreceptor cells and rod bipolar cells in the retina. We used genetically modified mouse models which abrogate each step of neurotransmission: presynaptic glutamate release, postsynaptic glutamate

Morphological characterization and topographic analysis of multiple photoreceptor types in the retinae of mesopelagic hatchetfishes with tubular eyes

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Lauren Michelle Biagioni

2016-03-01

Full Text Available Marine hatchetfishes, Argyropelecus spp., are one of the 14 genera of mesopelagic teleosts, which possess tubular eyes. The tubular eyes are positioned dorsally on the head and consist of a main retina, which subtends a large dorsal binocular field, and an accessory retina, which subtends the lateral monocular visual field. The topographic distribution of photoreceptors in the retina of Argyropelecus sladeni, A. affinis and A. aculeatus was determined using a random, unbiased and systematic stereological approach, which consistently revealed a region of high density (area centralis in the central region of the main retina (up to a peak of 96,000 receptors per mm2 and a relatively homogeneous density of photoreceptors in the accessory retina (of approximately 20,000 receptors per mm2. The position of the area centralis in the main retina indicates this retinal region subserves greater spatial resolution in the centre of the dorsal binocular visual field. Light microscopy and transmission electron microscopy also revealed the presence of multiple photoreceptor types (two rod-like and one cone-like based on the size and shape of the inner and outer segments and ultrastructural differences in the ellipsoidal region. The presence of multiple photoreceptor types in these tubular-eyed, mesopelagic hatchetfishes may reflect the need for the visual system to function under different lighting conditions during vertical migratory behavior, especially given their unique dorsally-facing eyes.

Photoreceptor dysplasia (pd) in miniature schnauzer dogs: evaluation of candidate genes by molecular genetic analysis.

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Zhang, Q; Baldwin, V J; Acland, G M; Parshall, C J; Haskel, J; Aguirre, G D; Ray, K

1999-01-01

Photoreceptor dysplasia (pd) is one of a group of at least six distinct autosomal and one X-linked retinal disorders identified in dogs which are collectively known as progressive retinal atrophy (PRA). It is an early onset retinal disease identified in miniature schnauzer dogs, and pedigree analysis and breeding studies have established autosomal recessive inheritance of the disease. Using a gene-based approach, a number of retina-expressed genes, including some members of the phototransduction pathway, have been causally implicated in retinal diseases of humans and other animals. Here we examined seven such potential candidate genes (opsin, RDS/peripherin, ROM1, rod cGMP-gated cation channel alpha-subunit, and three subunits of transducin) for their causal association with the pd locus by testing segregation of intragenic markers with the disease locus, or, in the absence of informative polymorphisms, sequencing of the coding regions of the genes. Based on these results, we have conclusively excluded four photoreceptor-specific genes as candidates for pd by linkage analysis. For three other photoreceptor-specific genes, we did not find any mutation in the coding sequences of the genes and have excluded them provisionally. Formal exclusion would require investigation of the levels of expression of the candidate genes in pd-affected dogs relative to age-matched controls. At present we are building suitable informative pedigrees for the disease locus with a sufficient number of meiosis to be useful for genomewide screening. This should identify markers linked to the disease locus and eventually permit progress toward the identification of the photoreceptor dysplasia gene and the disease-causing mutation.

Bcl-xL-mediated remodeling of rod and cone synaptic mitochondria after postnatal lead exposure: electron microscopy, tomography and oxygen consumption.

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Perkins, Guy A; Scott, Ray; Perez, Alex; Ellisman, Mark H; Johnson, Jerry E; Fox, Donald A

2012-01-01

Postnatal lead exposure produces rod-selective and Bax-mediated apoptosis, decreased scotopic electroretinograms (ERGs), and scotopic and mesopic vision deficits in humans and/or experimental animals. Rod, but not cone, inner segment mitochondria were considered the primary site of action. However, photoreceptor synaptic mitochondria were not examined. Thus, our experiments investigated the structural and functional effects of environmentally relevant postnatal lead exposure on rod spherule and cone pedicle mitochondria and whether Bcl-xL overexpression provided neuroprotection. C57BL/6N mice pups were exposed to lead only during lactation via dams drinking water containing lead acetate. The blood [Pb] at weaning was 20.6±4.7 µg/dl, which decreased to the control value by 2 months. To assess synaptic mitochondrial structural differences and vulnerability to lead exposure, wild-type and transgenic mice overexpressing Bcl-xL in photoreceptors were used. Electron microscopy, three-dimensional electron tomography, and retinal and photoreceptor synaptic terminal oxygen consumption (QO(2)) studies were conducted in adult control, Bcl-xL, lead, and Bcl-xL/lead mice. The spherule and pedicle mitochondria in lead-treated mice were swollen, and the cristae structure was markedly changed. In the lead-treated mice, the mitochondrial cristae surface area and volume (abundance: measure correlated with ATP (ATP) synthesis) were decreased in the spherules and increased in the pedicles. Pedicles also had an increased number of crista segments per volume. In the lead-treated mice, the number of segments/crista and fraction of cristae with multiple segments (branching) similarly increased in spherule and pedicle mitochondria. Lead-induced remodeling of spherule mitochondria produced smaller cristae with more branching, whereas pedicle mitochondria had larger cristae with more branching and increased crista junction (CJ) diameter. Lead decreased dark- and light-adapted photoreceptor

Light activation of one rhodopsin molecule causes the phosphorylation of hundreds of others. A reaction observed in electropermeabilized frog rod outer segments exposed to dim illumination

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Binder, B.M.; Biernbaum, M.S.; Bownds, M.D.

1990-01-01

A rhodopsin phosphorylation reaction that occurs with high-gain is observed if measurements are made in electropermeabilized frog rod outer segments (ROS) stimulated by a dim flash of light in the operating range of the photoreceptor. Flashes of light exciting 1000 or fewer of the 3 x 10(9) rhodopsins present/ROS results in the incorporation of 1400 phosphates from ATP into the rhodopsin pool for each excited rhodopsin (Rho*). This amplification decreases with increasing light intensity, falling most sharply after each disk has absorbed one photon. The high-gain reaction is lost if the ROS are broken into vesicles by shearing, leaving a low-gain rhodopsin phosphorylation characterized in previous studies using brighter illumination. The high-gain but not the low-gain phosphorylation appears to be regulated by G-protein and by calcium levels in the range over which intracellular calcium changes when rod photoreceptors are illuminated. Kinetic measurements made on the phosphorylation observed at higher light intensities shows that it initially occurs rapidly enough for a role in terminating the photoresponse. The high-gain phosphorylation observed at lower light intensities may play a global role in regulating light-adaptation of the rod photoreceptor, and its existence suggests that a search for a similar high-gain modification in systems using the homologous beta-adrenergic or muscarinic acetylcholine receptors might be rewarding

Hif1a inactivation rescues photoreceptor degeneration induced by a chronic hypoxia-like stress.

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Barben, Maya; Ail, Divya; Storti, Federica; Klee, Katrin; Schori, Christian; Samardzija, Marijana; Michalakis, Stylianos; Biel, Martin; Meneau, Isabelle; Blaser, Frank; Barthelmes, Daniel; Grimm, Christian

2018-04-17

Reduced choroidal blood flow and tissue changes in the ageing human eye impair oxygen delivery to photoreceptors and the retinal pigment epithelium. As a consequence, mild but chronic hypoxia may develop and disturb cell metabolism, function and ultimately survival, potentially contributing to retinal pathologies such as age-related macular degeneration (AMD). Here, we show that several hypoxia-inducible genes were expressed at higher levels in the aged human retina suggesting increased activity of hypoxia-inducible transcription factors (HIFs) during the physiological ageing process. To model chronically elevated HIF activity and investigate ensuing consequences for photoreceptors, we generated mice lacking von Hippel Lindau (VHL) protein in rods. This activated HIF transcription factors and led to a slowly progressing retinal degeneration in the ageing mouse retina. Importantly, this process depended mainly on HIF1 with only a minor contribution of HIF2. A gene therapy approach using AAV-mediated RNA interference through an anti-Hif1a shRNA significantly mitigated the degeneration suggesting a potential intervention strategy that may be applicable to human patients.

Spatiotemporal regulation of ATP and Ca2+ dynamics in vertebrate rod and cone ribbon synapses.

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Johnson, Jerry E; Perkins, Guy A; Giddabasappa, Anand; Chaney, Shawntay; Xiao, Weimin; White, Andrew D; Brown, Joshua M; Waggoner, Jenna; Ellisman, Mark H; Fox, Donald A

2007-06-15

In conventional neurons, Ca2+ enters presynaptic terminals during an action potential and its increased local concentration triggers transient exocytosis. In contrast, vertebrate photoreceptors are nonspiking neurons that maintain sustained depolarization and neurotransmitter release from ribbon synapses in darkness and produce light-dependent graded hyperpolarizing responses. Rods transmit single photon responses with high fidelity, whereas cones are less sensitive and exhibit faster response kinetics. These differences are likely due to variations in presynaptic Ca2+ dynamics. Metabolic coupling and cross-talk between mitochondria, endoplasmic reticulum (ER), plasma membrane Ca2+ ATPase (PMCA), and Na+-Ca2+ exchanger (NCX) coordinately control presynaptic ATP production and Ca2+ dynamics. The goal of our structural and functional studies was to determine the spatiotemporal regulation of ATP and Ca2+ dynamics in rod spherules and cone pedicles. Central retina tissue from C57BL/6 mice was used. Laser scanning confocal microscopy (LSCM) experiments were conducted on fixed-frozen vertical sections. Primary antibodies were selected for their tissue/cellular specificity and ability to recognize single, multiple or all splice variants of selected isoforms. Electron microscopy (EM) and 3-D electron tomography (ET) studies used our standard procedures on thin- and thick-sectioned retinas, respectively. Calibrated fluo-3-Ca2+ imaging experiments of dark- and light-adapted rod and cone terminals in retinal slices were conducted. Confocal microscopy showed that mitochondria, ER, PMCA, and NCX1 exhibited distinct retinal lamination patterns and differential distribution in photoreceptor synapses. Antibodies for three distinct mitochondrial compartments differentially labeled retinal areas with high metabolic demand: rod and cone inner segments, previously undescribed cone juxtanuclear mitochondria and the two plexiform layers. Rod spherule membranes uniformly and intensely

Structure and function of the interphotoreceptor matrix surrounding retinal photoreceptor cells.

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Ishikawa, Makoto; Sawada, Yu; Yoshitomi, Takeshi

2015-04-01

The interphotoreceptor matrix (IPM) is a highly organized structure with interconnected domains surrounding cone and rod photoreceptor cells and extends throughout the subretinal space. Based on known roles of the extracellular matrix in other tissues, the IPM is thought to have several prominent functions including serving as a receptor for growth factors, regulating retinoid transport, participating in cytoskeletal organization in surrounding cells, and regulation of oxygen and nutrient transport. In addition, a number of studies suggest that the IPM also may play a significant role in the etiology of retinal degenerative disorders. In this review, we describe the present knowledge concerning the structure and function of the IPM under physiological and pathological conditions. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

Dynamical adaptation in photoreceptors.

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Damon A Clark

Full Text Available Adaptation is at the heart of sensation and nowhere is it more salient than in early visual processing. Light adaptation in photoreceptors is doubly dynamical: it depends upon the temporal structure of the input and it affects the temporal structure of the response. We introduce a non-linear dynamical adaptation model of photoreceptors. It is simple enough that it can be solved exactly and simulated with ease; analytical and numerical approaches combined provide both intuition on the behavior of dynamical adaptation and quantitative results to be compared with data. Yet the model is rich enough to capture intricate phenomenology. First, we show that it reproduces the known phenomenology of light response and short-term adaptation. Second, we present new recordings and demonstrate that the model reproduces cone response with great precision. Third, we derive a number of predictions on the response of photoreceptors to sophisticated stimuli such as periodic inputs, various forms of flickering inputs, and natural inputs. In particular, we demonstrate that photoreceptors undergo rapid adaptation of response gain and time scale, over ∼ 300[Formula: see text] ms-i. e., over the time scale of the response itself-and we confirm this prediction with data. For natural inputs, this fast adaptation can modulate the response gain more than tenfold and is hence physiologically relevant.

Aerobic Glycolysis Is Essential for Normal Rod Function and Controls Secondary Cone Death in Retinitis Pigmentosa.

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Petit, Lolita; Ma, Shan; Cipi, Joris; Cheng, Shun-Yun; Zieger, Marina; Hay, Nissim; Punzo, Claudio

2018-05-29

Aerobic glycolysis accounts for ∼80%-90% of glucose used by adult photoreceptors (PRs); yet, the importance of aerobic glycolysis for PR function or survival remains unclear. Here, we further established the role of aerobic glycolysis in murine rod and cone PRs. We show that loss of hexokinase-2 (HK2), a key aerobic glycolysis enzyme, does not affect PR survival or structure but is required for normal rod function. Rods with HK2 loss increase their mitochondrial number, suggesting an adaptation to the inhibition of aerobic glycolysis. In contrast, cones adapt without increased mitochondrial number but require HK2 to adapt to metabolic stress conditions such as those encountered in retinitis pigmentosa, where the loss of rods causes a nutrient shortage in cones. The data support a model where aerobic glycolysis in PRs is not a necessity but rather a metabolic choice that maximizes PR function and adaptability to nutrient stress conditions. Copyright © 2018 The Author(s). Published by Elsevier Inc. All rights reserved.

Photoreceptor Sensory Cilium: Traversing the Ciliary Gate

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Hemant Khanna

2015-10-01

Full Text Available Cilia are antenna-like extensions of the plasma membrane found in nearly all cell types. In the retina of the eye, photoreceptors develop unique sensory cilia. Not much was known about the mechanisms underlying the formation and function of photoreceptor cilia, largely because of technical limitations and the specific structural and functional modifications that cannot be modeled in vitro. With recent advances in microscopy techniques and molecular and biochemical approaches, we are now beginning to understand the molecular basis of photoreceptor ciliary architecture, ciliary function and its involvement in human diseases. Here, I will discuss the studies that have revealed new knowledge of how photoreceptor cilia regulate their identity and function while coping with high metabolic and trafficking demands associated with processing light signal.

Homeostatic Plasticity Mediated by Rod-Cone Gap Junction Coupling in Retinal Degenerative Dystrophic RCS Rats

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Hou, Baoke; Fu, Yan; Weng, Chuanhuang; Liu, Weiping; Zhao, Congjian; Yin, Zheng Qin

2017-01-01

Rod-cone gap junctions open at night to allow rod signals to pass to cones and activate the cone-bipolar pathway. This enhances the ability to detect large, dim objects at night. This electrical synaptic switch is governed by the circadian clock and represents a novel form of homeostatic plasticity that regulates retinal excitability according to network activity. We used tracer labeling and ERG recording in the retinae of control and retinal degenerative dystrophic RCS rats. We found that in the control animals, rod-cone gap junction coupling was regulated by the circadian clock via the modulation of the phosphorylation of the melatonin synthetic enzyme arylalkylamine N-acetyltransferase (AANAT). However, in dystrophic RCS rats, AANAT was constitutively phosphorylated, causing rod-cone gap junctions to remain open. A further b/a-wave ratio analysis revealed that dystrophic RCS rats had stronger synaptic strength between photoreceptors and bipolar cells, possibly because rod-cone gap junctions remained open. This was despite the fact that a decrease was observed in the amplitude of both a- and b-waves as a result of the progressive loss of rods during early degenerative stages. These results suggest that electric synaptic strength is increased during the day to allow cone signals to pass to the remaining rods and to be propagated to rod bipolar cells, thereby partially compensating for the weak visual input caused by the loss of rods. PMID:28473754

Generalized Galileons: instabilities of bouncing and Genesis cosmologies and modified Genesis

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Libanov, M. [Institute for Nuclear Research of the Russian Academy of Sciences,60th October Anniversary Prospect 7a, 117312 Moscow (Russian Federation); Moscow Institute of Physics and Technology,Institutskii per. 9, 141700 Dolgoprudny, Moscow Region (Russian Federation); Mironov, S. [Institute for Nuclear Research of the Russian Academy of Sciences,60th October Anniversary Prospect 7a, 117312 Moscow (Russian Federation); Rubakov, V. [Institute for Nuclear Research of the Russian Academy of Sciences,60th October Anniversary Prospect 7a, 117312 Moscow (Russian Federation); Department of Particle Physics and Cosmology, Physics Faculty,M.V. Lomonosov Moscow State University,Vorobjevy Gory, 119991, Moscow (Russian Federation)

2016-08-18

We study spatially flat bouncing cosmologies and models with the early-time Genesis epoch in a popular class of generalized Galileon theories. We ask whether there exist solutions of these types which are free of gradient and ghost instabilities. We find that irrespectively of the forms of the Lagrangian functions, the bouncing models either are plagued with these instabilities or have singularities. The same result holds for the original Genesis model and its variants in which the scale factor tends to a constant as t→−∞. The result remains valid in theories with additional matter that obeys the Null Energy Condition and interacts with the Galileon only gravitationally. We propose a modified Genesis model which evades our no-go argument and give an explicit example of healthy cosmology that connects the modified Genesis epoch with kination (the epoch still driven by the Galileon field, which is a conventional massless scalar field at that stage).

The dual rod system of amphibians supports colour discrimination at the absolute visual threshold.

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Yovanovich, Carola A M; Koskela, Sanna M; Nevala, Noora; Kondrashev, Sergei L; Kelber, Almut; Donner, Kristian

2017-04-05

The presence of two spectrally different kinds of rod photoreceptors in amphibians has been hypothesized to enable purely rod-based colour vision at very low light levels. The hypothesis has never been properly tested, so we performed three behavioural experiments at different light intensities with toads ( Bufo ) and frogs ( Rana ) to determine the thresholds for colour discrimination. The thresholds of toads were different in mate choice and prey-catching tasks, suggesting that the differential sensitivities of different spectral cone types as well as task-specific factors set limits for the use of colour in these behavioural contexts. In neither task was there any indication of rod-based colour discrimination. By contrast, frogs performing phototactic jumping were able to distinguish blue from green light down to the absolute visual threshold, where vision relies only on rod signals. The remarkable sensitivity of this mechanism comparing signals from the two spectrally different rod types approaches theoretical limits set by photon fluctuations and intrinsic noise. Together, the results indicate that different pathways are involved in processing colour cues depending on the ecological relevance of this information for each task.This article is part of the themed issue 'Vision in dim light'. © 2017 The Authors.

Abrupt onset of mutations in a developmentally regulated gene during terminal differentiation of post-mitotic photoreceptor neurons in mice.

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Ivette M Sandoval

Full Text Available For sensitive detection of rare gene repair events in terminally differentiated photoreceptors, we generated a knockin mouse model by replacing one mouse rhodopsin allele with a form of the human rhodopsin gene that causes a severe, early-onset form of retinitis pigmentosa. The human gene contains a premature stop codon at position 344 (Q344X, cDNA encoding the enhanced green fluorescent protein (EGFP at its 3' end, and a modified 5' untranslated region to reduce translation rate so that the mutant protein does not induce retinal degeneration. Mutations that eliminate the stop codon express a human rhodopsin-EGFP fusion protein (hRho-GFP, which can be readily detected by fluorescence microscopy. Spontaneous mutations were observed at a frequency of about one per retina; in every case, they gave rise to single fluorescent rod cells, indicating that each mutation occurred during or after the last mitotic division. Additionally, the number of fluorescent rods did not increase with age, suggesting that the rhodopsin gene in mature rod cells is less sensitive to mutation than it is in developing rods. Thus, there is a brief developmental window, coinciding with the transcriptional activation of the rhodopsin locus, in which somatic mutations of the rhodopsin gene abruptly begin to appear.

Mutation in rod PDE6 linked to congenital stationary night blindness impairs the enzyme inhibition by its gamma-subunit.

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Muradov, Khakim G; Granovsky, Alexey E; Artemyev, Nikolai O

2003-03-25

Photoreceptor cGMP phosphodiesterase (PDE6) is the effector enzyme in the vertebrate visual transduction cascade. The activity of rod PDE6 catalytic alpha- and beta-subunits is blocked in the dark by two inhibitory Pgamma-subunits. The inhibition is released upon light-stimulation of photoreceptor cells. Mutation H258N in PDE6beta has been linked to congenital stationary night blindness (CSNB) in a large Danish family (Rambusch pedigree) (Gal, A., Orth, U., Baehr, W., Schwinger, E., and Rosenberg, T. (1994) Nat. Genet. 7, 64-67.) We have analyzed the consequences of this mutation for PDE6 function using a Pgamma-sensitive PDE6alpha'/PDE5 chimera, Chi16. Biochemical analysis of the H257N mutant, an equivalent of PDE6betaH258N, demonstrates that this substitution does not alter the ability of chimeric PDE to dimerize or the enzyme's catalytic properties. The sensitivity of H257N to a competitive inhibitor zaprinast was also unaffected. However, the mutant displayed a significant impairment in the inhibitory interaction with Pgamma, which was apparent from a approximately 20-fold increase in the K(i) value (46 nM) and incomplete maximal inhibition. The inhibitory defect of H257N is not due to perturbation of noncatalytic cGMP binding to the PDE6alpha' GAF domains. The noncatalytic cGMP-binding characteristics of the H257N mutant were similar to those of the parent PDE6alpha'/PDE5 chimera. Since rod PDE6 in the Rambusch CSNB is a catalytic heterodimer of the wild-type PDE6alpha and mutant PDE6beta, Chi16 and H257N were coexpressed, and a heterodimeric PDE, Chi16/H257N, was isolated. It displayed two Pgamma inhibitory sites with the K(i) values of 5 and 57 nM. Our results support the hypothesis that mutation H258N in PDE6beta causes CSNB through incomplete inhibition of PDE6 activity by Pgamma, which leads to desensitization of rod photoreceptors.

The Retinal Pigment Epithelium: a Convenient Source of New Photoreceptor cells?

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Shu-Zhen Wang

2014-01-01

Full Text Available Recent success in restoring visual function through photoreceptor replacement in mouse models of photoreceptor degeneration intensifies the need to generate or regenerate photoreceptor cells for the ultimate goal of using cell replacement therapy for blindness caused by photoreceptor degeneration. Current research on deriving new photoreceptors for replacement, as regenerative medicine in general, focuses on the use of embryonic stem cells and induced pluripotent stem (iPS cells to generate transplantable cells. Nonetheless, naturally occurring regeneration, such as wound healing, involves awakening cells at or near a wound site to produce new cells needed to heal the wound. Here we discuss the possibility of tweaking an ocular tissue, the retinal pigment epithelium (RPE, to produce photoreceptor cells in situ in the eye. Unlike the neural retina, the RPE in adult mammals maintains cell proliferation capability. Furthermore, progeny cells from RPE proliferation may differentiate into cells other than RPE. The combination of proliferation and plasticity opens a question of whether they could be channeled by a regulatory gene with pro-photoreceptor activity towards photoreceptor production. Studies using embryonic chick and transgenic mouse showed that indeed photoreceptor-like cells were produced in culture and in vivo in the eye using genedirected reprogramming of RPE cells, supporting the feasibility of using the RPE as a convenient source of new photoreceptor cells for in situ retinal repair without involving cell transplantation.

Increased proliferation of late-born retinal progenitor cells by gestational lead exposure delays rod and bipolar cell differentiation.

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Chaney, Shawnta Y; Mukherjee, Shradha; Giddabasappa, Anand; Rueda, Elda M; Hamilton, W Ryan; Johnson, Jerry E; Fox, Donald A

2016-01-01

Studies of neuronal development in the retina often examine the stages of proliferation, differentiation, and synaptic development, albeit independently. Our goal was to determine if a known neurotoxicant insult to a population of retinal progenitor cells (RPCs) would affect their eventual differentiation and synaptic development. To that end, we used our previously published human equivalent murine model of low-level gestational lead exposure (GLE). Children and animals with GLE exhibit increased scotopic electroretinogram a- and b-waves. Adult mice with GLE exhibit an increased number of late-born RPCs, a prolonged period of RPC proliferation, and an increased number of late-born rod photoreceptors and rod and cone bipolar cells (BCs), with no change in the number of late-born Müller glial cells or early-born neurons. The specific aims of this study were to determine whether increased and prolonged RPC proliferation alters the spatiotemporal differentiation and synaptic development of rods and BCs in early postnatal GLE retinas compared to control retinas. C57BL/6N mouse pups were exposed to lead acetate via drinking water throughout gestation and until postnatal day 10, which is equivalent to the human gestation period for retinal neurogenesis. RT-qPCR, immunohistochemical analysis, and western blots of well-characterized, cell-specific genes and proteins were performed at embryonic and early postnatal ages to assess rod and cone photoreceptor differentiation, rod and BC differentiation and synaptic development, and Müller glial cell differentiation. Real-time quantitative PCR (RT-qPCR) with the rod-specific transcription factors Nrl , Nr2e3 , and Crx and the rod-specific functional gene Rho , along with central retinal confocal studies with anti-recoverin and anti-rhodopsin antibodies, revealed a two-day delay in the differentiation of rod photoreceptors in GLE retinas. Rhodopsin immunoblots supported this conclusion. No changes in glutamine synthetase gene

Mutations in CEP78 Cause Cone-Rod Dystrophy and Hearing Loss Associated with Primary-Cilia Defects.

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Nikopoulos, Konstantinos; Farinelli, Pietro; Giangreco, Basilio; Tsika, Chrysanthi; Royer-Bertrand, Beryl; Mbefo, Martial K; Bedoni, Nicola; Kjellström, Ulrika; El Zaoui, Ikram; Di Gioia, Silvio Alessandro; Balzano, Sara; Cisarova, Katarina; Messina, Andrea; Decembrini, Sarah; Plainis, Sotiris; Blazaki, Styliani V; Khan, Muhammad Imran; Micheal, Shazia; Boldt, Karsten; Ueffing, Marius; Moulin, Alexandre P; Cremers, Frans P M; Roepman, Ronald; Arsenijevic, Yvan; Tsilimbaris, Miltiadis K; Andréasson, Sten; Rivolta, Carlo

2016-09-01

Cone-rod degeneration (CRD) belongs to the disease spectrum of retinal degenerations, a group of hereditary disorders characterized by an extreme clinical and genetic heterogeneity. It mainly differentiates from other retinal dystrophies, and in particular from the more frequent disease retinitis pigmentosa, because cone photoreceptors degenerate at a higher rate than rod photoreceptors, causing severe deficiency of central vision. After exome analysis of a cohort of individuals with CRD, we identified biallelic mutations in the orphan gene CEP78 in three subjects from two families: one from Greece and another from Sweden. The Greek subject, from the island of Crete, was homozygous for the c.499+1G>T (IVS3+1G>T) mutation in intron 3. The Swedish subjects, two siblings, were compound heterozygotes for the nearby mutation c.499+5G>A (IVS3+5G>A) and for the frameshift-causing variant c.633delC (p.Trp212Glyfs(∗)18). In addition to CRD, these three individuals had hearing loss or hearing deficit. Immunostaining highlighted the presence of CEP78 in the inner segments of retinal photoreceptors, predominantly of cones, and at the base of the primary cilium of fibroblasts. Interaction studies also showed that CEP78 binds to FAM161A, another ciliary protein associated with retinal degeneration. Finally, analysis of skin fibroblasts derived from affected individuals revealed abnormal ciliary morphology, as compared to that of control cells. Altogether, our data strongly suggest that mutations in CEP78 cause a previously undescribed clinical entity of a ciliary nature characterized by blindness and deafness but clearly distinct from Usher syndrome, a condition for which visual impairment is due to retinitis pigmentosa. Copyright © 2016 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

Molecular determinants of Guanylate Cyclase Activating Protein subcellular distribution in photoreceptor cells of the retina.

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López-Begines, Santiago; Plana-Bonamaisó, Anna; Méndez, Ana

2018-02-13

Retinal guanylate cyclase (RetGC) and guanylate cyclase activating proteins (GCAPs) play an important role during the light response in photoreceptor cells. Mutations in these proteins are linked to distinct forms of blindness. RetGC and GCAPs exert their role at the ciliary outer segment where phototransduction takes place. We investigated the mechanisms governing GCAP1 and GCAP2 distribution to rod outer segments by expressing selected GCAP1 and GCAP2 mutants as transient transgenes in the rods of GCAP1/2 double knockout mice. We show that precluding GCAP1 direct binding to RetGC (K23D/GCAP1) prevented its distribution to rod outer segments, while preventing GCAP1 activation of RetGC post-binding (W94A/GCAP1) did not. We infer that GCAP1 translocation to the outer segment strongly depends on GCAP1 binding affinity for RetGC, which points to GCAP1 requirement to bind to RetGC to be transported. We gain further insight into the distinctive regulatory steps of GCAP2 distribution, by showing that a phosphomimic at position 201 is sufficient to retain GCAP2 at proximal compartments; and that the bovine equivalent to blindness-causative mutation G157R/GCAP2 results in enhanced phosphorylation in vitro and significant retention at the inner segment in vivo, as likely contributing factors to the pathophysiology.

Photoreceptor layer map using spectral-domain optical coherence tomography.

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Lee, Ji Eun; Lim, Dae Won; Bae, Han Yong; Park, Hyun Jin

2009-12-01

To develop a novel method for analysis of the photoreceptor layer map (PLM) generated using spectral-domain optical coherence tomography (OCT). OCT scans were obtained from 20 eyes, 10 with macular holes (MH) and 10 with central serous chorioretinopathy (CSC) using the Macular Cube (512 x 128) protocol of the Cirrus HD-OCT (Carl Zeiss). The scanned data were processed using embedded tools of the advanced visualization. A partial thickness OCT fundus image of the photoreceptor layer was generated by setting the region of interest to a 50-microm thick layer that was parallel and adjacent to the retinal pigment epithelium. The resulting image depicted the photoreceptor layer as a map of the reflectivity in OCT. The PLM was compared with fundus photography, auto-fluorescence, tomography, and retinal thickness map. The signal from the photoreceptor layer of every OCT scan in each case was demonstrated as a single image of PLM in a fundus photograph fashion. In PLM images, detachment of the sensory retina is depicted as a hypo-reflective area, which represents the base of MH and serous detachment in CSC. Relative hypo-reflectivity, which was also noted at closed MH and at recently reattached retina in CSC, was associated with reduced signal from the junction between the inner and outer segments of photoreceptors in OCT images. Using PLM, changes in the area of detachment and reflectivity of the photoreceptor layer could be efficiently monitored. The photoreceptor layer can be analyzed as a map using spectral-domain OCT. In the treatment of both MH and CSC, PLM may provide new pathological information about the photoreceptor layer to expand our understanding of these diseases.

Neutron diffraction analysis of the structure of rod photoreceptor membranes in intact retinas

Energy Technology Data Exchange (ETDEWEB)

Yaeger, M; Schoenborn, B; Engelman, D; Moore, P; Stryer, L

1980-01-01

Neutron diffraction data have been collected from samples containing ten dark-adapted Rana catesbiana bullfrog retinas in 100, 80, 60, 40, and 30% D/sub 2/O Ringer's solution using a step-scanning Soller slit diffractometer. Diffraction was also recorded from retinas equilibrated in D/sub 2/O solutions with varying osmolarity. Rocking curve experiments demonstrated that the rods are disoriented in a cylindrically symmetrical fashion. Structure factor amplitudes were obtained using semi-automated curve-fitting procedures, and phases were obtained by interpreting the D/sub 2/O-H/sub 2/O and osmotic Patterson maps. In D/sub 2/O Ringer's solution the first four structure factors are -353 +- 25, 246 +- 19, 434 +- 13 and 383 +- 19. Neutron scattering density profiles were calculated to 75 A resolution using these structure factors. These neutron diffraction data are consistent with the view that the lipid bilayer is a major structural motif of the rod outer segment disc membrane. Neutron Fourier syntheses in different mixtures of D/sub 2/O and H/sub 2/O indicate that the intradisc and extradisc spaces are predominantly aqueous, consistent with the increase in the intradisc and extradisc volumes as the Ringer's solution is made more hypotomic. In isotonic Ringer's solution, the thicknesses of the intradisc and extradisc spaces are about 36 A and 160 A, respectively, and the center-to-center separation between the 50 A thick lipid bilayer is 88 A.

Amyloid precursor protein is required for normal function of the rod and cone pathways in the mouse retina.

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Tracy Ho

Full Text Available Amyloid precursor protein (APP is a transmembrane glycoprotein frequently studied for its role in Alzheimer's disease. Our recent study in APP knockout (KO mice identified an important role for APP in modulating normal neuronal development in the retina. However the role APP plays in the adult retina and whether it is required for vision is unknown. In this study we evaluated the role of APP in retinal function and morphology comparing adult wildtype (WT and APP-KO mice. APP was expressed on neuronal cells of the inner retina, including horizontal, cone bipolar, amacrine and ganglion cells in WT mice. The function of the retina was assessed using the electroretinogram and although the rod photoreceptor responses were similar in APP-KO and WT mice, the post-photoreceptor, inner retinal responses of both the rod and cone pathways were reduced in APP-KO mice. These changes in inner retinal function did not translate to a substantial change in visual acuity as assessed using the optokinetic response or to changes in the gross cellular structure of the retina. These findings indicate that APP is not required for basic visual function, but that it is involved in modulating inner retinal circuitry.

76 FR 54454 - Issuance of Loan Guarantee to Genesis Solar, LLC, for the Genesis Solar Energy Project

Science.gov (United States)

2011-09-01

... DEPARTMENT OF ENERGY Issuance of Loan Guarantee to Genesis Solar, LLC, for the Genesis Solar... Energy Project (GSEP), a 250-megawatt (MW) nominal capacity solar power generating facility on.../Final Environmental Impact Statement for the Genesis Solar Energy Project, Riverside County, California...

Usher protein functions in hair cells and photoreceptors.

Science.gov (United States)

Cosgrove, Dominic; Zallocchi, Marisa

2014-01-01

The 10 different genes associated with the deaf/blind disorder, Usher syndrome, encode a number of structurally and functionally distinct proteins, most expressed as multiple isoforms/protein variants. Functional characterization of these proteins suggests a role in stereocilia development in cochlear hair cells, likely owing to adhesive interactions in hair bundles. In mature hair cells, homodimers of the Usher cadherins, cadherin 23 and protocadherin 15, interact to form a structural fiber, the tip link, and the linkages that anchor the taller stereocilia's actin cytoskeleton core to the shorter adjacent stereocilia and the elusive mechanotransduction channels, explaining the deafness phenotype when these molecular interactions are perturbed. The conundrum is that photoreceptors lack a synonymous mechanotransduction apparatus, and so a common theory for Usher protein function in the two neurosensory cell types affected in Usher syndrome is lacking. Recent evidence linking photoreceptor cell dysfunction in the shaker 1 mouse model for Usher syndrome to light-induced protein translocation defects, combined with localization of an Usher protein interactome at the periciliary region of the photoreceptors suggests Usher proteins might regulate protein trafficking between the inner and outer segments of photoreceptors. A distinct Usher protein complex is trafficked to the ribbon synapses of hair cells, and synaptic defects have been reported in Usher mutants in both hair cells and photoreceptors. This review aims to clarify what is known about Usher protein function at the synaptic and apical poles of hair cells and photoreceptors and the prospects for identifying a unifying pathobiological mechanism to explain deaf/blindness in Usher syndrome. Copyright © 2013 Elsevier Ltd. All rights reserved.

Global gene expression analysis in a mouse model for Norrie disease: late involvement of photoreceptor cells.

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Lenzner, Steffen; Prietz, Sandra; Feil, Silke; Nuber, Ulrike A; Ropers, H-Hilger; Berger, Wolfgang

2002-09-01

Mutations in the NDP gene give rise to a variety of eye diseases, including classic Norrie disease (ND), X-linked exudative vitreoretinopathy (EVRX), retinal telangiectasis (Coats disease), and advanced retinopathy of prematurity (ROP). The gene product is a cystine-knot-containing extracellular signaling molecule of unknown function. In the current study, gene expression was determined in a mouse model of ND, to unravel disease-associated mechanisms at the molecular level. Gene transcription in the eyes of 2-year-old Ndp knockout mice was compared with that in the eyes of age-matched wild-type control animals, by means of cDNA subtraction and microarrays. Clones (n = 3072) from the cDNA subtraction libraries were spotted onto glass slides and hybridized with fluorescently labeled RNA-derived targets. More than 230 differentially expressed clones were sequenced, and their expression patterns were verified by virtual Northern blot analysis. Numerous gene transcripts that are absent or downregulated in the eye of Ndp knockout mice are photoreceptor cell specific. In younger Ndp knockout mice (up to 1 year old), however, all these transcripts were found to be expressed at normal levels. The identification of numerous photoreceptor cell-specific transcripts with a reduced expression in 2-year-old, but not in young, Ndp knockout mice indicates that normal gene expression in these light-sensitive cells of mutant mice is established and maintained over a long period and that rods and cones are affected relatively late in the mouse model of ND. Obviously, the absence of the Ndp gene product is not compatible with long-term survival of photoreceptor cells in the mouse.

The phototransduction machinery in the rod outer segment has a strong efficacy gradient

KAUST Repository

Mazzolini, Monica

2015-05-04

Rod photoreceptors consist of an outer segment (OS) and an inner segment. Inside the OS a biochemical machinery transforms the rhodopsin photoisomerization into electrical signal. This machinery has been treated as and is thought to be homogenous with marginal inhomogeneities. To verify this assumption, we developed a methodology based on special tapered optical fibers (TOFs) to deliver highly localized light stimulations. By using these TOFs, specific regions of the rod OS could be stimulated with spots of light highly confined in space. As the TOF is moved from the OS base toward its tip, the amplitude of saturating and single photon responses decreases, demonstrating that the efficacy of the transduction machinery is not uniform and is 5-10 times higher at the base than at the tip. This gradient of efficacy of the transduction machinery is attributed to a progressive depletion of the phosphodiesterase along the rod OS. Moreover we demonstrate that, using restricted spots of light, the duration of the photoresponse along the OS does not increase linearly with the light intensity as with diffuse light. © 2015, National Academy of Sciences. All rights reserved.

Knock-in strategy at 3'-end of Crx gene by CRISPR/Cas9 system shows the gene expression profiles during human photoreceptor differentiation.

Science.gov (United States)

Homma, Kohei; Usui, Sumiko; Kaneda, Makoto

2017-03-01

Fluorescent reporter gene knock-in induced pluripotent stem cell (iPSC) lines have been used to evaluate the efficiency of differentiation into specific cell lineages. Here, we report a knock-in strategy for the generation of human iPSC reporter lines in which a 2A peptide sequence and a red fluorescent protein (E2-Crimson) gene were inserted at the termination codon of the cone-rod homeobox (Crx) gene, a photoreceptor-specific transcriptional factor gene. The knock-in iPSC lines were differentiated into fluorescence-expressing cells in 3D retinal differentiation culture, and the fluorescent cells also expressed Crx specifically in the nucleus. We found that the fluorescence intensity was positively correlated with the expression levels of Crx mRNA and that fluorescent cells expressed rod photoreceptor-specific genes in the later stage of differentiation. Finally, we treated the fluorescent cells with DAPT, a Notch inhibitor, and found that DAPT-enhanced retinal differentiation was associated with up-regulation of Crx, Otx2 and NeuroD1, and down-regulation of Hes5 and Ngn2. These suggest that this knock-in strategy at the 3'-end of the target gene, combined with the 2A peptide linked to fluorescent proteins, offers a useful tool for labeling specific cell lineages or monitoring expression of any marker genes without affecting the function of the target gene. © 2017 Molecular Biology Society of Japan and John Wiley & Sons Australia, Ltd.

[Modification of retinal photoreceptor membranes and Ca ion binding].

Science.gov (United States)

Korchagin, V P; Berman, A L; Shukoliukov, S A; Rychkova, M P; Etingof, R N

1978-10-01

Calcium binding by modified photoreceptor membranes of cattle retina has been studied. Ca2+-binding the membranes significantly changes after C-phospholipase treatment, displaying the initial growth (less than 65% of lipid phosphorus removed) with subsequent decrease (more than 65% of phosphorus removed). Liposomes of the photoreceptor membranes lipids were found to bind more calcium than do the native photoreceptor membranes. Proteolytic enzymes (papaine, pronase) splitting some rhodopsin fragments do not affect the ability of the membrane to bind Ca2+. The increase of light-induced Ca-binding is observed only after the outer segments preincubation under conditions providing for rhodopsin phosphorylation. This effect was observed also after the splitting of the rhodopsin fragment by papaine. It is concluded that calcium binding in the photoreceptor membranes is mainly due to the phosphate groups of phospholipids.

Responses of photoreceptors in Hermissenda.

Science.gov (United States)

Akon, D L; Fuortes, M G

1972-12-01

The five photoreceptors in the eye of the mollusc Hermissenda crassicornis respond to light with depolarization and firing of impulses. The impulses of any one cell inhibit other cells, but the degree of inhibition differs in different pairs. Evidence is presented to show that the interactions occur at terminal branches of the photoreceptor axons, inside the cerebropleural ganglion. Properties of the generator potential are examined and it is shown that the depolarization develops in two phases which are affected differently by extrinsic currents. Finally, it is shown that by enhancing the differences in the responses of individual cells to a variety of stimuli, the interactions may facilitate a number of simple discriminations.

Mutations in the ABCA4 (ABCR) Gene Are the Major Cause of Autosomal Recessive Cone-Rod Dystrophy

OpenAIRE

Maugeri, Alessandra; Klevering, B. Jeroen; Rohrschneider, Klaus; Blankenagel, Anita; Brunner, Han G.; Deutman, August F.; Hoyng, Carel B.; Cremers, Frans P. M.

2000-01-01

The photoreceptor cell–specific ATP-binding cassette transporter gene (ABCA4; previously denoted “ABCR”) is mutated in most patients with autosomal recessive (AR) Stargardt disease (STGD1) or fundus flavimaculatus (FFM). In addition, a few cases with AR retinitis pigmentosa (RP) and AR cone-rod dystrophy (CRD) have been found to have ABCA4 mutations. To evaluate the importance of the ABCA4 gene as a cause of AR CRD, we selected 5 patients with AR CRD and 15 patients with isolated CRD, all fro...

Rhodopsin Forms Nanodomains in Rod Outer Segment Disc Membranes of the Cold-Blooded Xenopus laevis.

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Tatini Rakshit

Full Text Available Rhodopsin forms nanoscale domains (i.e., nanodomains in rod outer segment disc membranes from mammalian species. It is unclear whether rhodopsin arranges in a similar manner in amphibian species, which are often used as a model system to investigate the function of rhodopsin and the structure of photoreceptor cells. Moreover, since samples are routinely prepared at low temperatures, it is unclear whether lipid phase separation effects in the membrane promote the observed nanodomain organization of rhodopsin from mammalian species. Rod outer segment disc membranes prepared from the cold-blooded frog Xenopus laevis were investigated by atomic force microscopy to visualize the organization of rhodopsin in the absence of lipid phase separation effects. Atomic force microscopy revealed that rhodopsin nanodomains form similarly as that observed previously in mammalian membranes. Formation of nanodomains in ROS disc membranes is independent of lipid phase separation and conserved among vertebrates.

Human Usher 1B/mouse shaker-1: the retinal phenotype discrepancy explained by the presence/absence of myosin VIIA in the photoreceptor cells.

Science.gov (United States)

el-Amraoui, A; Sahly, I; Picaud, S; Sahel, J; Abitbol, M; Petit, C

1996-08-01

Usher syndrome type 1 (USH1) associates severe congenital deafness, vestibular dysfunction and progressive retinitis pigmentosa leading to blindness. The gene encoding myosin VIIA is responsible for USH1B. Mutations in the murine orthologous gene lead to the shaker-1 phenotype, which manifests cochlear and vestibular dysfunction, without any retinal defect. To address this phenotypic discrepancy, the expression of myosin VIIA in retinal cells was analyzed in human and mouse during embryonic development and adult life. In the human embryo, myosin VIIA was present first in the pigment epithelium cells, and later in these cells as well as in the photoreceptor cells. In the adult human retina, myosin VIIA was present in both cell types. In contrast, in mouse, only pigment epithelium cells expressed the protein throughout development and adult life. Myosin VIIA was also found to be absent in the photoreceptor cells of other rodents (rat and guinea-pig), whereas these cells expressed the protein in amphibians, avians and primates. These observations suggest that retinitis pigmentosa of USH1B results from a primary rod and cone defect. The USH1B/shaker-1 paradigm illustrates a species-specific cell pattern of gene expression as a possible cause for the discrepancy between phenotypes involving defective orthologous genes in man and mouse. Interestingly, in the photoreceptor cells, myosin VIIA is mainly localized in the inner and base of outer segments as well as in the synaptic ending region where it is co-localized with the synaptic vesicles. Therefore, we suggest that myosin VIIA might play a role in the trafficking of ribbon-synaptic vesicle complexes and the renewal processes of the outer photoreceptor disks.

Generation, purification and transplantation of photoreceptors derived from human induced pluripotent stem cells.

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Deepak A Lamba

2010-01-01

Full Text Available Inherited and acquired retinal degenerations are frequent causes of visual impairment and photoreceptor cell replacement therapy may restore visual function to these individuals. To provide a source of new retinal neurons for cell based therapies, we developed methods to derive retinal progenitors from human ES cells.In this report we have used a similar method to direct induced pluripotent stem cells (iPS from human fibroblasts to a retinal progenitor fate, competent to generate photoreceptors. We also found we could purify the photoreceptors derived from the iPS cells using fluorescence activated cell sorting (FACS after labeling photoreceptors with a lentivirus driving GFP from the IRBP cis-regulatory sequences. Moreover, we found that when we transplanted the FACS purified iPSC derived photoreceptors, they were able to integrate into a normal mouse retina and express photoreceptor markers.This report provides evidence that enriched populations of human photoreceptors can be derived from iPS cells.

Origins, transformations and key foci in instrumental genesis

DEFF Research Database (Denmark)

Tamborg, Andreas Lindenskov

This paper investigates the origins of the instrumental genesis and instrumental orchestrations frameworks. This is done by reviewing instrumented activity situations, instrumental genesis, and instrumental orchestrations with the purpose of identifying their epistemological assumptions, what the...... and instrumental orchestrations have potential shortcomings since the technologies that currently exist in school contexts are rather different from the technologies instrumental genesis was originally developed to study.......This paper investigates the origins of the instrumental genesis and instrumental orchestrations frameworks. This is done by reviewing instrumented activity situations, instrumental genesis, and instrumental orchestrations with the purpose of identifying their epistemological assumptions, what...

Three distinct roles for notch in Drosophila R7 photoreceptor specification.

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Andrew Tomlinson

2011-08-01

Full Text Available Receptor tyrosine kinases (RTKs and Notch (N proteins are different types of transmembrane receptors that transduce extracellular signals and control cell fate. Here we examine cell fate specification in the Drosophila retina and ask how N acts together with the RTKs Sevenless (Sev and the EGF receptor (DER to specify the R7 photoreceptor. The retina is composed of many hundred ommatidia, each of which grows by recruiting surrounding, undifferentiated cells and directing them to particular fates. The R7 photoreceptor derives from a cohort of three cells that are incorporated together following specification of the R2-R5 and R8 photoreceptors. Two cells of the cohort are specified as the R1/6 photoreceptor type by DER activation. These cells then activate N in the third cell (the R7 precursor. By manipulation of N and RTK signaling in diverse combinations we establish three roles for N in specifying the R7 fate. The first role is to impose a block to photoreceptor differentiation; a block that DER activation cannot overcome. The second role, paradoxically, is to negate the first; Notch activation up-regulates Sev expression, enabling the presumptive R7 cell to receive an RTK signal from R8 that can override the block. The third role is to specify the cell as an R7 rather than an R1/6 once RTK signaling has specified the cells as a photoreceptor. We speculate why N acts both to block and to facilitate photoreceptor differentiation, and provide a model for how N and RTK signaling act combinatorially to specify the R1/6 and R7 photoreceptors as well as the surrounding non-neuronal cone cells.

G3 : GENESIS software envrionment update

OpenAIRE

Castagné, Nicolas; Cadoz, Claude; Allaoui, Ali; Tache, Olivier Michel

2009-01-01

GENESIS3 is the new version of the GENESIS software environment for musical creation by means of mass-interaction physics network modeling. It was designed, and developed from scratch, in hindsight of more than 10 years working on and using the previous version. We take the opportunity of this birth to provide in this article (1) an analysis of the peculiarities in GENESIS, aiming at highlighting its core ?software paradigm?; and (2) an update on the features of the new version as compared to...

Intraocular gene transfer of ciliary neurotrophic factor rescues photoreceptor degeneration in RCS rats.

Science.gov (United States)

Huang, Shun-Ping; Lin, Po-Kang; Liu, Jorn-Hon; Khor, Chin-Ni; Lee, Yih-Jing

2004-01-01

Ciliary neurotrophic factor (CNTF) is known as an important factor in the regulation of retinal cell growth. We used both recombinant CNTF and an adenovirus carrying the CNTF gene to regulate retinal photoreceptor expression in a retinal degenerative animal, Royal College of Surgeons (RCS) rats. Cells in the outer nuclear layer of the retinae from recombinant-CNTF-treated, adenoviral-CNTF-treated, saline-operated, and contralateral untreated preparations were examined for those exhibiting CNTF photoreceptor protective effects. Cell apoptosis in the outer nuclear layer of the retinae was also detected. It was found that CNTF had a potent effect on delaying the photoreceptor degeneration process in RCS rats. Furthermore, adenovirus CNTF gene transfer was proven to be better at rescuing photoreceptors than that when using recombinant CNTF, since adenoviral CNTF prolonged the photoreceptor protection effect. The function of the photoreceptors was also examined by taking electroretinograms of different animals. Adenoviral-CNTF-treated eyes showed better retinal function than did the contralateral control eyes. This study indicates that adenoviral CNTF effectively rescues degenerating photoreceptors in RCS rats. Copyright 2004 National Science Council, ROC and S. Karger AG, Basel

PAR-Complex and Crumbs Function During Photoreceptor Morphogenesis and Retinal Degeneration.

Science.gov (United States)

Pichaud, Franck

2018-01-01

The fly photoreceptor has long been used as a model to study sensory neuron morphogenesis and retinal degeneration. In particular, elucidating how these cells are built continues to help further our understanding of the mechanisms of polarized cell morphogenesis, intracellular trafficking and the causes of human retinal pathologies. The conserved PAR complex, which in flies consists of Cdc42-PAR6-aPKC-Bazooka, and the transmembrane protein Crumbs (Crb) are key players during photoreceptor morphogenesis. While the PAR complex regulates polarity in many cell types, Crb function in polarity is relatively specific to epithelial cells. Together Cdc42-PAR6-aPKC-Bazooka and Crb orchestrate the differentiation of the photoreceptor apical membrane (AM) and zonula adherens (ZA) , thus allowing these cells to assemble into a neuro-epithelial lattice. In addition to its function in epithelial polarity, Crb has also been shown to protect fly photoreceptors from light-induced degeneration, a process linked to Rhodopsin expression and trafficking. Remarkably, mutations in the human Crumbs1 (CRB1) gene lead to retinal degeneration, making the fly photoreceptor a powerful disease model system.

Photoreceptor spectral sensitivity in the bumblebee, Bombus impatiens (Hymenoptera: Apidae.

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Peter Skorupski

Full Text Available The bumblebee Bombus impatiens is increasingly used as a model in comparative studies of colour vision, or in behavioural studies relying on perceptual discrimination of colour. However, full spectral sensitivity data on the photoreceptor inputs underlying colour vision are not available for B. impatiens. Since most known bee species are trichromatic, with photoreceptor spectral sensitivity peaks in the UV, blue and green regions of the spectrum, data from a related species, where spectral sensitivity measurements have been made, are often applied to B impatiens. Nevertheless, species differences in spectral tuning of equivalent photoreceptor classes may result in peaks that differ by several nm, which may have small but significant effects on colour discrimination ability. We therefore used intracellular recording to measure photoreceptor spectral sensitivity in B. impatiens. Spectral peaks were estimated at 347, 424 and 539 nm for UV, blue and green receptors, respectively, suggesting that this species is a UV-blue-green trichromat. Photoreceptor spectral sensitivity peaks are similar to previous measurements from Bombus terrestris, although there is a significant difference in the peak sensitivity of the blue receptor, which is shifted in the short wave direction by 12-13 nm in B. impatiens compared to B. terrestris.

Roles of glucose in photoreceptor survival.

Science.gov (United States)

Chertov, Andrei O; Holzhausen, Lars; Kuok, Iok Teng; Couron, Drew; Parker, Ed; Linton, Jonathan D; Sadilek, Martin; Sweet, Ian R; Hurley, James B

2011-10-07

Vertebrate photoreceptor neurons have a high demand for metabolic energy, and their viability is very sensitive to genetic and environmental perturbations. We investigated the relationship between energy metabolism and cell death by evaluating the metabolic effects of glucose deprivation on mouse photoreceptors. Oxygen consumption, lactate production, ATP, NADH/NAD(+), TCA cycle intermediates, morphological changes, autophagy, and viability were evaluated. We compared retinas incubated with glucose to retinas deprived of glucose or retinas treated with a mixture of mitochondrion-specific fuels. Rapid and slow phases of cell death were identified. The rapid phase is linked to reduced mitochondrial activity, and the slower phase reflects a need for substrates for cell maintenance and repair.

Cellular elements for seeing in the dark: voltage-dependent conductances in cockroach photoreceptors

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Salmela Iikka

2012-08-01

Full Text Available Abstract Background The importance of voltage-dependent conductances in sensory information processing is well-established in insect photoreceptors. Here we present the characterization of electrical properties in photoreceptors of the cockroach (Periplaneta americana, a nocturnal insect with a visual system adapted for dim light. Results Whole-cell patch-clamped photoreceptors had high capacitances and input resistances, indicating large photosensitive rhabdomeres suitable for efficient photon capture and amplification of small photocurrents at low light levels. Two voltage-dependent potassium conductances were found in the photoreceptors: a delayed rectifier type (KDR and a fast transient inactivating type (KA. Activation of KDR occurred during physiological voltage responses induced by light stimulation, whereas KA was nearly fully inactivated already at the dark resting potential. In addition, hyperpolarization of photoreceptors activated a small-amplitude inward-rectifying (IR current mediated at least partially by chloride. Computer simulations showed that KDR shapes light responses by opposing the light-induced depolarization and speeding up the membrane time constant, whereas KA and IR have a negligible role in the majority of cells. However, larger KA conductances were found in smaller and rapidly adapting photoreceptors, where KA could have a functional role. Conclusions The relative expression of KA and KDR in cockroach photoreceptors was opposite to the previously hypothesized framework for dark-active insects, necessitating further comparative work on the conductances. In general, the varying deployment of stereotypical K+ conductances in insect photoreceptors highlights their functional flexibility in neural coding.

Modulation of recognition memory performance by light requires both melanopsin and classical photoreceptors

Science.gov (United States)

Tam, Shu K. E.; Hasan, Sibah; Hughes, Steven; Hankins, Mark W.; Foster, Russell G.; Bannerman, David M.

2016-01-01

Acute light exposure exerts various effects on physiology and behaviour. Although the effects of light on brain network activity in humans are well demonstrated, the effects of light on cognitive performance are inconclusive, with the size, as well as direction, of the effect depending on the nature of the task. Similarly, in nocturnal rodents, bright light can either facilitate or disrupt performance depending on the type of task employed. Crucially, it is unclear whether the effects of light on behavioural performance are mediated via the classical image-forming rods and cones or the melanopsin-expressing photosensitive retinal ganglion cells. Here, we investigate the modulatory effects of light on memory performance in mice using the spontaneous object recognition task. Importantly, we examine which photoreceptors are required to mediate the effects of light on memory performance. By using a cross-over design, we show that object recognition memory is disrupted when the test phase is conducted under a bright light (350 lux), regardless of the light level in the sample phase (10 or 350 lux), demonstrating that exposure to a bright light at the time of test, rather than at the time of encoding, impairs performance. Strikingly, the modulatory effect of light on memory performance is completely abolished in both melanopsin-deficient and rodless–coneless mice. Our findings provide direct evidence that melanopsin-driven and rod/cone-driven photoresponses are integrated in order to mediate the effect of light on memory performance. PMID:28003454

Photoreceptor atrophy in acute zonal occult outer retinopathy

DEFF Research Database (Denmark)

Zibrandtsen, N.; Munch, I.C.; Klemp, K.

2008-01-01

appearance were examined using optical coherence tomography (OCT), automated perimetry and electroretinography (ERG). RESULTS: Both patients demonstrated photoreceptor atrophy corresponding to partial or complete scotomata with reduced or extinct electroretinographic responses. Attenuation or complete loss...... of all the segments composing the photoreceptor layer was found by OCT. Full-field ERG revealed affection of the 30 Hz flicker responses and subnormal photopic responses in both patients and subnormal scotopic responses in case 1. Multifocal electroretinography (mERG) revealed localized outer retinal...

Photoreceptor atrophy in acute zonal occult outer retinopathy

DEFF Research Database (Denmark)

Zibrandtsen, N.; Munch, I.C.; Klemp, K.

2008-01-01

examined using optical coherence tomography (OCT), automated perimetry and electroretinography (ERG). Both patients demonstrated photoreceptor atrophy corresponding to partial or complete scotomata with reduced or extinct electroretinographic responses. Attenuation or complete loss of all the segments...... composing the photoreceptor layer was found by OCT. Full-field ERG revealed affection of the 30 Hz flicker responses and subnormal photopic responses in both patients and subnormal scotopic responses in case 1. Multifocal electroretinography (mERG) revealed localized outer retinal dysfunction. The field...

PAR-Complex and Crumbs Function During Photoreceptor Morphogenesis and Retinal Degeneration

Directory of Open Access Journals (Sweden)

Franck Pichaud

2018-03-01

Full Text Available The fly photoreceptor has long been used as a model to study sensory neuron morphogenesis and retinal degeneration. In particular, elucidating how these cells are built continues to help further our understanding of the mechanisms of polarized cell morphogenesis, intracellular trafficking and the causes of human retinal pathologies. The conserved PAR complex, which in flies consists of Cdc42-PAR6-aPKC-Bazooka, and the transmembrane protein Crumbs (Crb are key players during photoreceptor morphogenesis. While the PAR complex regulates polarity in many cell types, Crb function in polarity is relatively specific to epithelial cells. Together Cdc42-PAR6-aPKC-Bazooka and Crb orchestrate the differentiation of the photoreceptor apical membrane (AM and zonula adherens (ZA, thus allowing these cells to assemble into a neuro-epithelial lattice. In addition to its function in epithelial polarity, Crb has also been shown to protect fly photoreceptors from light-induced degeneration, a process linked to Rhodopsin expression and trafficking. Remarkably, mutations in the human Crumbs1 (CRB1 gene lead to retinal degeneration, making the fly photoreceptor a powerful disease model system.

Ultrafast spectroscopy of biological photoreceptors

NARCIS (Netherlands)

Kennis, J.T.M.; Groot, M.L.

2007-01-01

We review recent new insights on reaction dynamics of photoreceptors proteins gained from ultrafast spectroscopy. In Blue Light sensing Using FAD (BLUF) domains, a hydrogen-bond rearrangement around the flavin chromophore proceeds through a radical-pair mechanism, by which light-induced electron and

Laser induced photoreceptor damage and recovery in the high numerical aperture eye of the garter snake.

Science.gov (United States)

Zwick, H; Edsall, P; Stuck, B E; Wood, E; Elliott, R; Cheramie, R; Hacker, H

2008-02-01

The garter snake provides a unique model for in-vivo imaging of photoreceptor damage induced by laser retinal exposure. Laser thermal/mechanical retinal injury induced alterations in photoreceptor structure and leukocyte cellular behavior. Photoreceptors turned white, lost mode structure, and swelled; leukocyte activity was observed in the vicinity of photoreceptor cells. Non-thermal alterations were identified with a bio-tag for oxidative stress. Mechanisms of photoreceptor recovery and replacement were observed and evaluated for active cytoskeletal systems by using an anti-actin tag that could detect the presence of active cytoskeletal systems resident in photoreceptors as well as other retinal systems.

LONGITUDINAL QUANTITATIVE EVALUATION OF PHOTORECEPTOR VOLUME FOLLOWING REPAIR OF MACULA-OFF RETINAL DETACHMENT.

Science.gov (United States)

Narala, Ramsudha; Scarinci, Fabio; Shaarawy, Amr; Simonett, Joseph M; Flaxel, Christina J; Fawzi, Amani A

2016-08-01

To quantify photoreceptor volume changes after successful surgical repair of macula-off retinal detachment and to correlate these volumetric changes to postoperative best-corrected visual acuity (BCVA). Retrospective study of 15 eyes of 15 patients with macula-off retinal detachment who underwent successful surgical repair. A minimum of 4 optical coherence tomography scans that straddled the foveal center was used to quantify the central photoreceptor volume (central 1 mm). Mean photoreceptor volume at the first postoperative visit was 0.451 mm, increasing to 0.523 mm at the final postoperative visit (P = 0.004). Mean BCVA improved from 1.13 ± 0.59 logarithm of the minimum angle of resolution units (∼20/270) preoperatively to 0.52 ± 0.42 logarithm of the minimum angle of resolution units (∼20/66) at the final postoperative visit (P = 0.001). Mean photoreceptor volume at either the initial or final visit demonstrated significant correlations with final postoperative BCVA (r = -0.670, P = 0.017 and r = -0.753, P = 0.005, respectively). Shorter time interval from diagnosis to surgery was significantly associated with greater mean final postoperative photoreceptor volume (r = -0.588, P = 0.021) and better mean final postoperative BCVA (r = 0.709, P = 0.003). We observed a significant increase in photoreceptor volume after successful retinal detachment repair; photoreceptor volume was positively associated with BCVA and time to surgery. Our series emphasizes the importance of prompt surgical repair and shows that photoreceptor recovery and volumetric improvement correlate significantly with BCVA.

Control rod

International Nuclear Information System (INIS)

Kawakami, Kazuo; Shimoshige, Takanori; Nishimura, Akira

1979-01-01

Purpose: A control rod has been developed, which provided a plurality of through-holes in the vicinity of the sheath fitting position, in order to flatten burn-up, of fuel rods in positions confronting a control rod. Thereby to facilitate the manufacture of the control rods and prevent fuel rod failures. Constitution: A plurality of through-holes are formed in the vicinity of the sheath fitting position of a central support rod to which a sheath for the control rod is fitted. These through-holes are arranged in the axial direction of the central support rod. Accordingly, burn-up of fuel rods confronting the control rods can be reduced by through-holes and fuel rod failures can be prevented. (Yoshino, Y.)

New Results from the Flare Genesis Experiment

Science.gov (United States)

Rust, D. M.; Bernasconi, P. N.; Eaton, H. A.; Keller, C.; Murphy, G. A.; Schmieder, B.

2000-05-01

From January 10 to 27, 2000, the Flare Genesis solar telescope observed the Sun while suspended from a balloon in the stratosphere above Antarctica. The goal of the mission was to acquire long time series of high-resolution images and vector magnetograms of the solar photosphere and chromosphere. Images were obtained in the magnetically sensitive Ca I line at 6122 Angstroms and at H-alpha (6563 Angstroms). The FGE data were obtained in the context of Max Millennium Observing Campaign #004, the objective of which was to study the ``Genesis of Solar Flares and Active Filaments/Sigmoids." Flare Genesis obtained about 26,000 usable images on the 8 targeted active regions. A preliminary examination reveals a good sequence on an emerging flux region and data on the M1 flare on January 22, as well as a number of sequences on active filaments. We will present the results of our first analysis efforts. Flare Genesis was supported by NASA grants NAG5-4955, NAG5-5139, and NAG5-8331 and by NSF grant OPP-9615073. The Air Force Office of Scientific Research and the Ballistic Missile Defense Organization supported early development of the Flare Genesis Experiment.

Rod phototransduction determines the trade-off of temporal integration and speed of vision in dark-adapted toads.

Science.gov (United States)

Haldin, Charlotte; Nymark, Soile; Aho, Ann-Christine; Koskelainen, Ari; Donner, Kristian

2009-05-06

Human vision is approximately 10 times less sensitive than toad vision on a cool night. Here, we investigate (1) how far differences in the capacity for temporal integration underlie such differences in sensitivity and (2) whether the response kinetics of the rod photoreceptors can explain temporal integration at the behavioral level. The toad was studied as a model that allows experimentation at different body temperatures. Sensitivity, integration time, and temporal accuracy of vision were measured psychophysically by recording snapping at worm dummies moving at different velocities. Rod photoresponses were studied by ERG recording across the isolated retina. In both types of experiments, the general timescale of vision was varied by using two temperatures, 15 and 25 degrees C. Behavioral integration times were 4.3 s at 15 degrees C and 0.9 s at 25 degrees C, and rod integration times were 4.2-4.3 s at 15 degrees C and 1.0-1.3 s at 25 degrees C. Maximal behavioral sensitivity was fivefold lower at 25 degrees C than at 15 degrees C, which can be accounted for by inability of the "warm" toads to integrate light over longer times than the rods. However, the long integration time at 15 degrees C, allowing high sensitivity, degraded the accuracy of snapping toward quickly moving worms. We conclude that temporal integration explains a considerable part of all variation in absolute visual sensitivity. The strong correlation between rods and behavior suggests that the integration time of dark-adapted vision is set by rod phototransduction at the input to the visual system. This implies that there is an inexorable trade-off between temporal integration and resolution.

Fundus Autofluorescence and Photoreceptor Cell Rosettes in Mouse Models

Science.gov (United States)

Flynn, Erin; Ueda, Keiko; Auran, Emily; Sullivan, Jack M.; Sparrow, Janet R.

2014-01-01

Purpose. This study was conducted to study correlations among fundus autofluorescence (AF), RPE lipofuscin accumulation, and photoreceptor cell degeneration and to investigate the structural basis of fundus AF spots. Methods. Fundus AF images (55° lens; 488-nm excitation) and spectral-domain optical coherence tomography (SD-OCT) scans were acquired in pigmented Rdh8−/−/Abca4−/− mice (ages 1–9 months) with a confocal scanning laser ophthalmoscope (cSLO). For quantitative fundus AF (qAF), gray levels (GLs) were calibrated to an internal fluorescence reference. Retinal bisretinoids were measured by quantitative HPLC. Histometric analysis of outer nuclear layer (ONL) thicknesses was performed, and cryostat sections of retina were examined by fluorescence microscopy. Results. Quantified A2E and qAF intensities increased until age 4 months in the Rdh8−/−/Abca4−/− mice. The A2E levels declined after 4 months of age, but qAF intensity values continued to rise. The decline in A2E levels in the Rdh8−/−/Abca4−/− mice paralleled reduced photoreceptor cell viability as reflected in ONL thinning. Hyperautofluorescent puncta in fundus AF images corresponded to photoreceptor cell rosettes in SD-OCT images and histological sections stained with hematoxylin and eosin. The inner segment/outer segment–containing core of the rosette emitted an autofluorescence detected by fluorescence microscopy. Conclusions. When neural retina is disordered, AF from photoreceptor cells can contribute to noninvasive fundus AF images. Hyperautofluorescent puncta in fundus AF images are attributable, in at least some cases, to photoreceptor cell rosettes. PMID:25015357

On Dispersion in Visual Photoreceptors

NARCIS (Netherlands)

Stavenga, D.G.; Barneveld, H.H. van

1975-01-01

An idealized visual pigment absorbance spectrum is used together with a Kramers-Kronig dispersion relation to calculate the contribution of the visual pigment to the refractive index of the fly photoreceptor. It appears that an absorption coefficient of 0.010 µm-1 results in a refractive index

Preservation of photoreceptors in dystrophic RCS rats following allo- and xenotransplantation of IPE cells.

Science.gov (United States)

Thumann, Gabriele; Salz, Anna Katharina; Walter, Peter; Johnen, Sandra

2009-03-01

To examine whether iris pigment epithelial (IPE) cells transplanted into the subretinal space of Royal College of Surgeons (RCS) rats have the ability to rescue photoreceptors. Rat IPE (rIPE) or human IPE (hIPE) cells were transplanted subretinally in 23-day-old RCS rats. Sham injection and transplantation of ARPE-19 cells served as controls. After 12 weeks, eyes were evaluated for photoreceptor survival by morphometric analysis and electron microscopy. Morphometric analysis showed photoreceptor rescue in all transplanted and sham-injected animals (number of photoreceptors/300 microm retina+/-sd: rIPE 41.67 +/- 28; hIPE 29.50 +/- 16; ARPE-19 36.12 +/- 21; sham 16.56 +/- 6) compared to age-matched, control rats (number of photoreceptors/300 microm retina+/-sd: 9.71 +/- 4). Photoreceptor rescue was prominent in IPE cell-transplanted rats and was significantly greater than sham-injected eyes (p = 0.02 for rIPE and p = 0.04 for hIPE). Since IPE cells transplanted into the subretinal space have the ability to rescue photoreceptors from degeneration in the RCS rat without any harmful effects, IPE cells may represent an ideal cell to genetically modify and thus carry essential genetic information for the repair of defects in the subretinal space.

Idiopathic multifocal choroiditis/punctate inner choroidopathy with acute photoreceptor loss or dysfunction out of proportion to clinically visible lesions

Science.gov (United States)

Munk, Marion R.; Jung, Jesse J.; Biggee, Kristin; Tucker, William R.; Sen, H. Nida; Schmidt-Erfurth, Ursula; Fawzi, Amani A.; Jampol, Lee M.

2014-01-01

Purpose To report acute/subacute vision loss and paracentral scotomata in patients with idiopathic multifocal choroiditis/punctate inner choroidopathy (MFC/PIC) due to large zones of acute photoreceptor attenuation surrounding the chorioretinal lesions. Methods Multimodal-imaging case-series Results Six females and 2 males were included (mean age 31.5±5.8 years). Vision ranged from 20/20-1 to hand motion (mean 20/364). SD-OCT demonstrated extensive attenuation of the external limiting membrane (ELM), ellipsoid and interdigitation zones, adjacent to the visible MFC/PIC lesions. The corresponding areas were hyperautofluorescent on fundus-autofluorescence (FAF), and were associated with corresponding visual field defects. Full-field ERG (available in 3 cases) showed markedly decreased cone/rod response and multifocal ERG revealed reduced amplitudes and increased implicit times in 2 cases. Three patients received no treatment, the remaining were treated with oral corticosteroids (n=4), oral acyclovir/valacyclovir (n=2), intravitreal/posterior subtenon triamcinolone-acetate (n=3) and anti-VEGF (n=2). Visual recovery occurred in only 3 cases, of whom 2 were treated. Varying morphological recovery was found in 6 cases, associated with decrease in hyperautofluorescence on FAF. Conclusions MFC/PIC can present with transient or permanent central photoreceptor attenuation/loss. This presentation is likely a variant of MFC/PIC with chorioretinal atrophy. Associated changes are best evaluated using multimodal imaging. PMID:25322466

Role of kinesin heavy chain in Crumbs localization along the rhabdomere elongation in Drosophila photoreceptor.

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Garrett P League

Full Text Available BACKGROUND: Crumbs (Crb, a cell polarity gene, has been shown to provide a positional cue for the extension of the apical membrane domain, adherens junction (AJ, and rhabdomere along the growing proximal-distal axis during Drosophila photoreceptor morphogenesis. In developing Drosophila photoreceptors, a stabilized microtubule structure was discovered and its presence was linked to polarity protein localization. It was therefore hypothesized that the microtubules may provide trafficking routes for the polarity proteins during photoreceptor morphogenesis. This study has examined whether Kinesin heavy chain (Khc, a subunit of the microtubule-based motor Kinesin-1, is essential in polarity protein localization in developing photoreceptors. METHODOLOGY/PRINCIPAL FINDINGS: Because a genetic interaction was found between crb and khc, Crb localization was examined in the developing photoreceptors of khc mutants. khc was dispensable during early eye differentiation and development. However, khc mutant photoreceptors showed a range of abnormalities in the apical membrane domain depending on the position along the proximal-distal axis in pupal photoreceptors. The khc mutant showed a progressive mislocalization in the apical domain along the distal-proximal axis during rhabdomere elongation. The khc mutation also led to a similar progressive defect in the stabilized microtubule structures, strongly suggesting that Khc is essential for microtubule structure and Crb localization during distal to proximal rhabdomere elongation in pupal morphogenesis. This role of Khc in apical domain control was further supported by khc's gain-of-function phenotype. Khc overexpression in photoreceptors caused disruption of the apical membrane domain and the stabilized microtubules in the developing photoreceptors. CONCLUSIONS/SIGNIFICANCE: In summary, we examined the role of khc in the regulation of the apical Crb domain in developing photoreceptors. Since the rhabdomeres in

Lrit1, a Retinal Transmembrane Protein, Regulates Selective Synapse Formation in Cone Photoreceptor Cells and Visual Acuity

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Akiko Ueno

2018-03-01

Full Text Available Summary: In the vertebrate retina, cone photoreceptors play crucial roles in photopic vision by transmitting light-evoked signals to ON- and/or OFF-bipolar cells. However, the mechanisms underlying selective synapse formation in the cone photoreceptor pathway remain poorly understood. Here, we found that Lrit1, a leucine-rich transmembrane protein, localizes to the photoreceptor synaptic terminal and regulates the synaptic connection between cone photoreceptors and cone ON-bipolar cells. Lrit1-deficient retinas exhibit an aberrant morphology of cone photoreceptor pedicles, as well as an impairment of signal transmission from cone photoreceptors to cone ON-bipolar cells. Furthermore, we demonstrated that Lrit1 interacts with Frmpd2, a photoreceptor scaffold protein, and with mGluR6, an ON-bipolar cell-specific glutamate receptor. Additionally, Lrit1-null mice showed visual acuity impairments in their optokinetic responses. These results suggest that the Frmpd2-Lrit1-mGluR6 axis regulates selective synapse formation in cone photoreceptors and is essential for normal visual function. : Ueno et al. finds that Lrit1 plays an important role in regulating the synaptic connection between cone photoreceptors and cone ON-bipolar cells. The Frmpd2-Lrit1-mGluR6 axis is crucial for selective synapse formation in cone photoreceptors and for development of normal visual function. Keywords: retina, circuit, synapse formation, cone photoreceptor cell, ON-bipolar cell, visual acuity

℮-conome: an automated tissue counting platform of cone photoreceptors for rodent models of retinitis pigmentosa

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Clérin Emmanuelle

2011-12-01

Full Text Available Abstract Background Retinitis pigmentosa is characterized by the sequential loss of rod and cone photoreceptors. The preservation of cones would prevent blindness due to their essential role in human vision. Rod-derived Cone Viability Factor is a thioredoxin-like protein that is secreted by rods and is involved in cone survival. To validate the activity of Rod-derived Cone Viability Factors (RdCVFs as therapeutic agents for treating retinitis Pigmentosa, we have developed e-conome, an automated cell counting platform for retinal flat mounts of rodent models of cone degeneration. This automated quantification method allows for faster data analysis thereby accelerating translational research. Methods An inverted fluorescent microscope, motorized and coupled to a CCD camera records images of cones labeled with fluorescent peanut agglutinin lectin on flat-mounted retinas. In an average of 300 fields per retina, nine Z-planes at magnification X40 are acquired after two-stage autofocus individually for each field. The projection of the stack of 9 images is subject to a threshold, filtered to exclude aberrant images based on preset variables. The cones are identified by treating the resulting image using 13 variables empirically determined. The cone density is calculated over the 300 fields. Results The method was validated by comparison to the conventional stereological counting. The decrease in cone density in rd1 mouse was found to be equivalent to the decrease determined by stereological counting. We also studied the spatiotemporal pattern of the degeneration of cones in the rd1 mouse and show that while the reduction in cone density starts in the central part of the retina, cone degeneration progresses at the same speed over the whole retinal surface. We finally show that for mice with an inactivation of the Nucleoredoxin-like genes Nxnl1 or Nxnl2 encoding RdCVFs, the loss of cones is more pronounced in the ventral retina. Conclusion The automated

℮-conome: an automated tissue counting platform of cone photoreceptors for rodent models of retinitis pigmentosa.

Science.gov (United States)

Clérin, Emmanuelle; Wicker, Nicolas; Mohand-Saïd, Saddek; Poch, Olivier; Sahel, José-Alain; Léveillard, Thierry

2011-12-20

Retinitis pigmentosa is characterized by the sequential loss of rod and cone photoreceptors. The preservation of cones would prevent blindness due to their essential role in human vision. Rod-derived Cone Viability Factor is a thioredoxin-like protein that is secreted by rods and is involved in cone survival. To validate the activity of Rod-derived Cone Viability Factors (RdCVFs) as therapeutic agents for treating retinitis Pigmentosa, we have developed e-conome, an automated cell counting platform for retinal flat mounts of rodent models of cone degeneration. This automated quantification method allows for faster data analysis thereby accelerating translational research. An inverted fluorescent microscope, motorized and coupled to a CCD camera records images of cones labeled with fluorescent peanut agglutinin lectin on flat-mounted retinas. In an average of 300 fields per retina, nine Z-planes at magnification X40 are acquired after two-stage autofocus individually for each field. The projection of the stack of 9 images is subject to a threshold, filtered to exclude aberrant images based on preset variables. The cones are identified by treating the resulting image using 13 variables empirically determined. The cone density is calculated over the 300 fields. The method was validated by comparison to the conventional stereological counting. The decrease in cone density in rd1 mouse was found to be equivalent to the decrease determined by stereological counting. We also studied the spatiotemporal pattern of the degeneration of cones in the rd1 mouse and show that while the reduction in cone density starts in the central part of the retina, cone degeneration progresses at the same speed over the whole retinal surface. We finally show that for mice with an inactivation of the Nucleoredoxin-like genes Nxnl1 or Nxnl2 encoding RdCVFs, the loss of cones is more pronounced in the ventral retina. The automated platform ℮-conome used here for retinal disease is a tool that

NMNAT1 variants cause cone and cone-rod dystrophy.

Science.gov (United States)

Nash, Benjamin M; Symes, Richard; Goel, Himanshu; Dinger, Marcel E; Bennetts, Bruce; Grigg, John R; Jamieson, Robyn V

2018-03-01

Cone and cone-rod dystrophies (CD and CRD, respectively) are degenerative retinal diseases that predominantly affect the cone photoreceptors. The underlying disease gene is not known in approximately 75% of autosomal recessive cases. Variants in NMNAT1 cause a severe, early-onset retinal dystrophy called Leber congenital amaurosis (LCA). We report two patients where clinical phenotyping indicated diagnoses of CD and CRD, respectively. NMNAT1 variants were identified, with Case 1 showing an extremely rare homozygous variant c.[271G > A] p.(Glu91Lys) and Case 2 compound heterozygous variants c.[53 A > G];[769G > A] p.(Asn18Ser);(Glu257Lys). The detailed variant analysis, in combination with the observation of an associated macular atrophy phenotype, indicated that these variants were disease-causing. This report demonstrates that the variants in NMNAT1 may cause CD or CRD associated with macular atrophy. Genetic investigations of the patients with CD or CRD should include NMNAT1 in the genes examined.

Lrit1, a Retinal Transmembrane Protein, Regulates Selective Synapse Formation in Cone Photoreceptor Cells and Visual Acuity.

Science.gov (United States)

Ueno, Akiko; Omori, Yoshihiro; Sugita, Yuko; Watanabe, Satoshi; Chaya, Taro; Kozuka, Takashi; Kon, Tetsuo; Yoshida, Satoyo; Matsushita, Kenji; Kuwahara, Ryusuke; Kajimura, Naoko; Okada, Yasushi; Furukawa, Takahisa

2018-03-27

In the vertebrate retina, cone photoreceptors play crucial roles in photopic vision by transmitting light-evoked signals to ON- and/or OFF-bipolar cells. However, the mechanisms underlying selective synapse formation in the cone photoreceptor pathway remain poorly understood. Here, we found that Lrit1, a leucine-rich transmembrane protein, localizes to the photoreceptor synaptic terminal and regulates the synaptic connection between cone photoreceptors and cone ON-bipolar cells. Lrit1-deficient retinas exhibit an aberrant morphology of cone photoreceptor pedicles, as well as an impairment of signal transmission from cone photoreceptors to cone ON-bipolar cells. Furthermore, we demonstrated that Lrit1 interacts with Frmpd2, a photoreceptor scaffold protein, and with mGluR6, an ON-bipolar cell-specific glutamate receptor. Additionally, Lrit1-null mice showed visual acuity impairments in their optokinetic responses. These results suggest that the Frmpd2-Lrit1-mGluR6 axis regulates selective synapse formation in cone photoreceptors and is essential for normal visual function. Copyright © 2018 The Author(s). Published by Elsevier Inc. All rights reserved.

Photocurrents in retinal rods of pigeons (Columba livia): kinetics and spectral sensitivity.

Science.gov (United States)

Palacios, A G; Goldsmith, T H

1993-01-01

1. Membrane photocurrents were recorded from outer segments of isolated retinal rods of pigeons (Columba livia), the first such measurements on the photoreceptors of a bird. The amplitude of the response to 20 ms flashes of narrow wavelength bands of light increases linearly with intensity at low photon fluxes and saturates at higher intensities. The maximum (saturating) photocurrent observed in forty-nine rod cells was 50 pA. Larger responses with less variability in the intensity for half-maximal responses were observed when the physiological saline contained 20 mM bicarbonate (in addition to Hepes buffer). 2. The dependence of peak amplitude on intensity is well fitted by an exponential function; it is usually less well fitted by the Michaelis-Menten (Naka-Rushton) equation. 3. In the presence of bicarbonate, the average sensitivity of pigeon rods to dim flashes was 0.56 pA photon-1 microns -2. The effective collecting area per photon was 1.8 microns 2. About 83 +/- 26 (mean +/- S.D.) photoisomerizations were required for a half-saturating response. 4. The response kinetics of rods to dim flashes can be reasonably well described by a series of four to five either Poisson or independent filters. The time to peak, measured from the mid-point of a 20 ms flash, was 319 +/- 83 ms (mean +/- S.D.). The integration time of the response was 851 +/- 86 ms (mean +/- S.D.) with bicarbonate present and 572 +/- 126 ms in the absence of bicarbonate. The responses of pigeon rods appear to be slower than those of mammals at the same temperature. The fraction of current suppressed by a single photoisomerization is smaller in pigeon than in mammalian rods by a factor of at least two. 5. The spectral sensitivity function was measured between 680 and 330 nm. The maximum at about 505 nm (range 497-508 nm) corresponds to the alpha-band of a vertebrate rhodopsin and agrees with previous behavioural measurements of scotopic sensitivity of pigeons as well as the absorption spectrum of

Evidence for dynamic network regulation of Drosophila photoreceptor function from mutants lacking the neurotransmitter histamine

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An eDau

2016-03-01

Full Text Available Synaptic feedback from interneurons to photoreceptors can help to optimize visual information flow by balancing its allocation on retinal pathways under changing light conditions. But little is known about how this critical network operation is regulated dynamically. Here, we investigate this question by comparing signaling properties and performance of wild-type Drosophila R1-R6 photoreceptors to those of the hdcJK910 mutant, which lacks the neurotransmitter histamine and therefore cannot transmit information to interneurons. Recordings show that hdcJK910 photoreceptors sample similar amounts of information from naturalistic stimulation to wild-type photoreceptors, but this information is packaged in smaller responses, especially under bright illumination. Analyses reveal how these altered dynamics primarily resulted from network overload that affected hdcJK910 photoreceptors in two ways. First, the missing inhibitory histamine input to interneurons almost certainly depolarized them irrevocably, which in turn increased their excitatory feedback to hdcJK910 R1-R6s. This tonic excitation depolarized the photoreceptors to artificially high potentials, reducing their operational range. Second, rescuing histamine input to interneurons in hdcJK910 mutant also restored their normal phasic feedback modulation to R1-R6s, causing photoreceptor output to accentuate dynamic intensity differences at bright illumination, similar to the wild-type. These results provide mechanistic explanations of how synaptic feedback connections optimize information packaging in photoreceptor output and novel insight into the operation and design of dynamic network regulation of sensory neurons.

A new photosensory function for simple photoreceptors, the intrinsically photoresponsive neurons of the sea slug Onchidium

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Tsukasa Gotow

2009-12-01

Full Text Available Simple photoreceptors, namely intrinsically light-sensitive neurons without microvilli and/or cilia, have long been known to exist in the central ganglia of crayfish, Aplysia, Onchidium, and Helix. These simple photoreceptors are not only first-order photosensory cells, but also second-order neurons (interneurons, relaying several kinds of sensory synaptic inputs. Another important issue is that the photoresponses of these simple photoreceptors show very slow kinetics and little adaptation. These characteristics suggest that the simple photoreceptors of the Onchidium have a function in non-image-forming vision, different from classical eye photoreceptors used for cording dynamic images of vision. The cited literature provides evidence that the depolarizing and hyperpolarizing photoresponses of simple photoreceptors play a role in the long-lasting potentiation of synaptic transmission of excitatory and inhibitory sensory inputs, and as well as in the potentiation and the suppression of the subsequent behavioral outputs. In short, we suggest that simple photoreceptors operate in the general potentiation of synaptic transmission and subsequent motor output; i.e., they perform a new photosensory function.

A neuroanatomical and physiological study of the non-image forming visual system of the cone-rod homeobox gene (Crx) knock out mouse

DEFF Research Database (Denmark)

Rovsing, Louise; Rath, Martin F; Lund-Andersen, Casper

2010-01-01

The anatomy and physiology of the non-image forming visual system was investigated in a visually blind cone-rod homeobox gene (Crx) knock-out mouse (Crx(-)(/)(-)), which lacks the outer segments of the photoreceptors. We show that the suprachiasmatic nuclei (SCN) in the Crx(-/-) mouse exhibit...... melanopsin neurons or the SCN may be necessary for a normal function of the non-image forming system of the mouse. However, a change in the SCN of the Crx(-/-) mouse might also explain the observed circadian differences between the knock out mouse and wild type mouse....

Edaravone, an ROS Scavenger, Ameliorates Photoreceptor Cell Death after Experimental Retinal Detachment

Science.gov (United States)

Roh, Mi In; Murakami, Yusuke; Thanos, Aristomenis; Miller, Joan W.

2011-01-01

Purpose. To investigate whether edaravone (3-methyl-1-phenyl-2-pyrazolin-5-one), a free radical scavenger, would be neuroprotective against photoreceptor cell death in a rat model of retinal detachment (RD). Methods. RD was induced in adult Brown Norway rats by subretinal injection of sodium hyaluronate. Edaravone (3, 5, or 10 mg/kg) or physiologic saline was administered intraperitoneally once a day until death on day 3 or 5. Oxidative stress in the retina was assessed by 4-hydroxynonenal staining or ELISA for protein carbonyl content. Photoreceptor death was assessed by TUNEL and measurement of the outer nuclear layer thickness. Western blot analysis and caspase activity assays were performed. Inflammatory cytokine secretion and inflammatory cell infiltration were evaluated by ELISA and immunostaining, respectively. Results. RD resulted in increased generation of ROS. Treatment with 5 mg/kg edaravone significantly reduced the ROS level, along with a decrease in TUNEL-positive cells in the photoreceptor layer. A caspase assay also confirmed decreased activation of caspase-3, -8, and -9 in RD treated with edaravone. The level of the antiapoptotic Bcl-2 was increased in detached retinas after edaravone treatment, whereas the levels of the stress-activated p-ERK1/2 were decreased. In addition, edaravone treatment resulted in a significant decrease in the levels of TNF-α, MCP-1, and macrophage infiltration. Conclusions. Oxidative stress plays an important role in photoreceptor cell death after RD. Edaravone treatment may aid in preventing photoreceptor cell death after RD by suppressing ROS-induced photoreceptor damage. PMID:21310909

Photoreceptor cells with profound structural deficits can support useful vision in mice.

Science.gov (United States)

Thompson, Stewart; Blodi, Frederick R; Lee, Swan; Welder, Chris R; Mullins, Robert F; Tucker, Budd A; Stasheff, Steven F; Stone, Edwin M

2014-03-25

In animal models of degenerative photoreceptor disease, there has been some success in restoring photoreception by transplanting stem cell-derived photoreceptor cells into the subretinal space. However, only a small proportion of transplanted cells develop extended outer segments, considered critical for photoreceptor cell function. The purpose of this study was to determine whether photoreceptor cells that lack a fully formed outer segment could usefully contribute to vision. Retinal and visual function was tested in wild-type and Rds mice at 90 days of age (Rds(P90)). Photoreceptor cells of mice homozygous for the Rds mutation in peripherin 2 never develop a fully formed outer segment. The electroretinogram and multielectrode recording of retinal ganglion cells were used to test retinal responses to light. Three distinct visual behaviors were used to assess visual capabilities: the optokinetic tracking response, the discrimination-based visual water task, and a measure of the effect of vision on wheel running. Rds(P90) mice had reduced but measurable electroretinogram responses to light, and exhibited light-evoked responses in multiple types of retinal ganglion cells, the output neurons of the retina. In optokinetic and discrimination-based tests, acuity was measurable but reduced, most notably when contrast was decreased. The wheel running test showed that Rds(P90) mice needed 3 log units brighter luminance than wild type to support useful vision (10 cd/m(2)). Photoreceptors that lack fully formed outer segments can support useful vision. This challenges the idea that normal cellular structure needs to be completely reproduced for transplanted cells to contribute to useful vision.

Control rod

International Nuclear Information System (INIS)

Igarashi, Takao; Sugawara, Satoshi; Yoshimoto, Yuichiro; Saito, Shozo; Fukumoto, Takashi.

1987-01-01

Purpose: To reduce the weight and thereby obtain satisfactory operationability of control rods by combining absorbing nuclear chain type neutron absorbers and conventional type neutron absorbers in the axial direction of blades. Constitution: Neutron absorber rods and long life type neutron absorber rods are disposed in a tie rod and a sheath. The neutron absorber rod comprises a poison tube made of stainless steels and packed with B 4 C powder. The long life type neutron absorber rod is prepared by packing B-10 enriched boron carbide powder into a hafnium metal rod, hafnium pipe, europium and stainless made poison tube. Since the long life type absorber rod uses HF as the absorbing nuclear chain type neutron absorber, it absorbs neutrons to form new neutron absorbers to increase the nuclear life. (Yoshino, Y.)

Control rods

International Nuclear Information System (INIS)

Maruyama, Hiromi.

1984-01-01

Purpose: To realize effective utilization, cost reduction and weight reduction in neutron absorbing materials. Constitution: Residual amount of neutron absorbing material is averaged between the top end region and other regions of a control rod upon reaching to the control rod working life, by using a single kind of neutron absorbing material and increasing the amount of the neutron absorber material at the top end region of the control rod as compared with that in the other regions. Further, in a case of a control rod having control rod blades such as in a cross-like control rod, the amount of the neutron absorbing material is decreased in the middle portion than in the both end portions of the control rod blade along the transversal direction of the rod, so that the residual amount of the neutron absorbing material is balanced between the central region and both end regions upon reaching the working life of the control rod. (Yoshihara, H.)

Photoreceptor processing speed and input resistance changes during light adaptation correlate with spectral class in the bumblebee, Bombus impatiens.

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Peter Skorupski

Full Text Available Colour vision depends on comparison of signals from photoreceptors with different spectral sensitivities. However, response properties of photoreceptor cells may differ in ways other than spectral tuning. In insects, for example, broadband photoreceptors, with a major sensitivity peak in the green region of the spectrum (>500 nm, drive fast visual processes, which are largely blind to chromatic signals from more narrowly-tuned photoreceptors with peak sensitivities in the blue and UV regions of the spectrum. In addition, electrophysiological properties of the photoreceptor membrane may result in differences in response dynamics of photoreceptors of similar spectral class between species, and different spectral classes within a species. We used intracellular electrophysiological techniques to investigate response dynamics of the three spectral classes of photoreceptor underlying trichromatic colour vision in the bumblebee, Bombus impatiens, and we compare these with previously published data from a related species, Bombus terrestris. In both species, we found significantly faster responses in green, compared with blue- or UV-sensitive photoreceptors, although all 3 photoreceptor types are slower in B. impatiens than in B. terrestris. Integration times for light-adapted B. impatiens photoreceptors (estimated from impulse response half-width were 11.3 ± 1.6 ms for green photoreceptors compared with 18.6 ± 4.4 ms and 15.6 ± 4.4 for blue and UV, respectively. We also measured photoreceptor input resistance in dark- and light-adapted conditions. All photoreceptors showed a decrease in input resistance during light adaptation, but this decrease was considerably larger (declining to about 22% of the dark value in green photoreceptors, compared to blue and UV (41% and 49%, respectively. Our results suggest that the conductances associated with light adaptation are largest in green photoreceptors, contributing to their greater temporal processing speed

Control rod calibration including the rod coupling effect

International Nuclear Information System (INIS)

Szilard, R.; Nelson, G.W.

1984-01-01

In a reactor containing more than one control rod, which includes all reactors licensed in the United States, there will be a 'coupling' or 'shadowing' of control rod flux at the location of a control rod as a result of the flux depression caused by another control rod. It was decided to investigate this phenomenon further, and eventually to put calibration table data or formulae in a small computer in the control room, so once could insert the positions of the three control rods and receive the excess reactivity without referring to separate tables. For this to be accomplished, a 'three control- rod reactivity function' would be used which would include the flux coupling between the rods. The function is design and measured data was fitted into it to determine the calibration constants. The input data for fitting the trial functions consisted of 254 data points, each consisting of the position of the reg, shim, and transient rods, and the total excess reactivity. (About 200 of these points were 'critical balance points', that is the rod positions for which reactor was critical, and the remainder were determined by positive period measurements.) Although this may be unrealistic from a physical viewpoint, the function derived gave a very accurate recalculation of the input data, and thus would faithfully give the excess reactivity for any possible combination of the locations of the three control rods. The next step, incorporation of the three-rod function into the minicomputer, will be pursued in the summer and fall of 1984

Ultrastructural and ERG findings in progressive rod-cone dystrophy in a litter of Labrador retrievers.

Science.gov (United States)

Raitta, C; Kommonen, B; Ulshafer, R; Karhunen, U

1991-02-01

Early ultrastructural findings of a progressive photoreceptor dystrophy and corresponding ERG findings are reported in 3 Labrador Retrievers from a litter of 7 pups bred from 2 dogs clinically and electroretinographically affected with generalized progressive retinal dystrophy. The pups were euthanized at 5, 11 and 15 months post partum. The most prominent ultrastructural finding was photoreceptor dystrophy. At 5 months the outer nuclear layer (ONL) consisted of 8-10 layers and seemed reduced in thickness, pyknotic nuclei were seen in this layer. The receptor outer segments (OS) were short and swollen. Some disorientation of OS discs occurred. In the 11-months specimen 7-8 ONL layers were identified. Overall thinning of the neuro-retina had occurred and fewer receptors compared to the 5-months specimen were present. By 15 months the ONL was further reduced to about 4 layers. Enlarged internuclear spaces were present in the ONL as well as around inner segments (IS). Phagocytic cells were frequent among remains of OS. The pigment epithelium appeared normal. The dark adapted ERG b-wave amplitudes and photopic 30 Hz flicker responses were low in comparison to controls of the same breed, and decreased with age. The condition represents a progressive rod-cone dystrophy which shares similarities with primary receptor dystrophy in man such as retinitis pigmentosa.

Chemically induced and light-independent cryptochrome photoreceptor activation.

Science.gov (United States)

Rosenfeldt, Gesa; Viana, Rafael Muñoz; Mootz, Henning D; von Arnim, Albrecht G; Batschauer, Alfred

2008-01-01

The cryptochrome photoreceptors of higher plants are dimeric proteins. Their N-terminal photosensory domain mediates dimerization, and the unique C-terminal extension (CCT) mediates signaling. We made use of the human FK506-binding protein (FKBP) that binds with high affinity to rapamycin or rapamycin analogs (rapalogs). The FKBP-rapamycin complex is recognized by another protein, FRB, thus allowing rapamycin-induced dimerization of two target proteins. Here we demonstrate by bioluminescence resonance energy transfer (BRET) assays the applicability of this regulated dimerization system to plants. Furthermore, we show that fusion proteins consisting of the C-terminal domain of Arabidopsis cryptochrome 2 fused to FKBP and FRB and coexpressed in Arabidopsis cells specifically induce the expression of cryptochrome-controlled reporter and endogenous genes in darkness upon incubation with the rapalog. These results demonstrate that the activation of cryptochrome signal transduction can be chemically induced in a dose-dependent fashion and uncoupled from the light signal, and provide the groundwork for gain-of-function experiments to study specifically the role of photoreceptors in darkness or in signaling cross-talk even under light conditions that activate members of all photoreceptor families.

Cytogenesis in the monkey retina

International Nuclear Information System (INIS)

La Vail, M.M.; Rapaport, D.H.; Rakic, P.

1991-01-01

Time of cell origin in the retina of the rhesus monkey (Macaca mulatta) was studied by plotting the number of heavily radiolabeled nuclei in autoradiograms prepared from 2- to 6-month-old animals, each of which was exposed to a pulse of 3H-thymidine (3H-TdR) on a single embryonic (E) or postnatal (P) day. Cell birth in the monkey retina begins just after E27, and approximately 96% of cells are generated by E120. The remaining cells are produced during the last (approximately 45) prenatal days and into the first several weeks after birth. Cell genesis begins near the fovea, and proceeds towards the periphery. Cell division largely ceases in the foveal and perifoveal regions by E56. Despite extensive overlap, a class-specific sequence of cell birth was observed. Ganglion and horizontal cells, which are born first, have largely congruent periods of cell genesis with the peak between E38 and E43, and termination around E70. The first labeled cones were apparent by E33, and their highest density was achieved between E43 and E56, tapering to low values at E70, although some cones are generated in the far periphery as late as E110. Amacrine cells are next in the cell birth sequence and begin genesis at E43, reach a peak production between E56 and E85, and cease by E110. Bipolar cell birth begins at the same time as amacrines, but appears to be separate from them temporally since their production reaches a peak between E56 and E102, and persists beyond the day of birth. Mueller cells and rod photoreceptors, which begin to be generated at E45, achieve a peak, and decrease in density at the same time as bipolar cells, but continue genesis at low density on the day of birth. Thus, bipolar, Mueller, and rod cells have a similar time of origin

Presynaptic dystroglycan-pikachurin complex regulates the proper synaptic connection between retinal photoreceptor and bipolar cells.

Science.gov (United States)

Omori, Yoshihiro; Araki, Fumiyuki; Chaya, Taro; Kajimura, Naoko; Irie, Shoichi; Terada, Koji; Muranishi, Yuki; Tsujii, Toshinori; Ueno, Shinji; Koyasu, Toshiyuki; Tamaki, Yasuhiro; Kondo, Mineo; Amano, Shiro; Furukawa, Takahisa

2012-05-02

Dystroglycan (DG) is a key component of the dystrophin-glycoprotein complex (DGC) at the neuromuscular junction postsynapse. In the mouse retina, the DGC is localized at the presynapse of photoreceptor cells, however, the function of presynaptic DGC is poorly understood. Here, we developed and analyzed retinal photoreceptor-specific DG conditional knock-out (DG CKO) mice. We found that the DG CKO retina showed a reduced amplitude and a prolonged implicit time of the ERG b-wave. Electron microscopic analysis revealed that bipolar dendrite invagination into the photoreceptor terminus is perturbed in the DG CKO retina. In the DG CKO retina, pikachurin, a DG ligand in the retina, is markedly decreased at photoreceptor synapses. Interestingly, in the Pikachurin(-/-) retina, the DG signal at the ribbon synaptic terminus was severely reduced, suggesting that pikachurin is required for the presynaptic accumulation of DG at the photoreceptor synaptic terminus, and conversely DG is required for pikachurin accumulation. Furthermore, we found that overexpression of pikachurin induces formation and clustering of a DG-pikachurin complex on the cell surface. The Laminin G repeats of pikachurin, which are critical for its oligomerization and interaction with DG, were essential for the clustering of the DG-pikachurin complex as well. These results suggest that oligomerization of pikachurin and its interaction with DG causes DG assembly on the synapse surface of the photoreceptor synaptic terminals. Our results reveal that the presynaptic interaction of pikachurin with DG at photoreceptor terminals is essential for both the formation of proper photoreceptor ribbon synaptic structures and normal retinal electrophysiology.

Retinal Thickening and Photoreceptor Loss in HIV Eyes without Retinitis.

Directory of Open Access Journals (Sweden)

Cheryl A Arcinue

Full Text Available To determine the presence of structural changes in HIV retinae (i.e., photoreceptor density and retinal thickness in the macula compared with age-matched HIV-negative controls.Cohort of patients with known HIV under CART (combination Antiretroviral Therapy treatment were examined with a flood-illuminated retinal AO camera to assess the cone photoreceptor mosaic and spectral-domain optical coherence tomography (SD-OCT to assess retinal layers and retinal thickness.Twenty-four eyes of 12 patients (n = 6 HIV-positive and 6 HIV-negative were imaged with the adaptive optics camera. In each of the regions of interest studied (nasal, temporal, superior, inferior, the HIV group had significantly less mean cone photoreceptor density compared with age-matched controls (difference range, 4,308-6,872 cones/mm2. A different subset of forty eyes of 20 patients (n = 10 HIV-positive and 10 HIV-negative was included in the retinal thickness measurements and retinal layer segmentation with the SD-OCT. We observed significant thickening in HIV positive eyes in the total retinal thickness at the foveal center, and in each of the three horizontal B-scans (through the macular center, superior, and inferior to the fovea. We also noted that the inner retina (combined thickness from ILM through RNFL to GCL layer was also significantly thickened in all the different locations scanned compared with HIV-negative controls.Our present study shows that the cone photoreceptor density is significantly reduced in HIV retinae compared with age-matched controls. HIV retinae also have increased macular retinal thickness that may be caused by inner retinal edema secondary to retinovascular disease in HIV. The interaction of photoreceptors with the aging RPE, as well as possible low-grade ocular inflammation causing diffuse inner retinal edema, may be the key to the progressive vision changes in HIV-positive patients without overt retinitis.

Cosmological bounce and Genesis beyond Horndeski

Energy Technology Data Exchange (ETDEWEB)

Kolevatov, R.; Mironov, S.; Volkova, V. [Institute for Nuclear Research of the Russian Academy of Sciences, 60th October Anniversary Prospect, 7a, 117312 Moscow (Russian Federation); Sukhov, N., E-mail: kolevatov@ms2.inr.ac.ru, E-mail: sa.mironov_1@physics.msu.ru, E-mail: nd.sukhov@physics.msu.ru, E-mail: volkova.viktoriya@physics.msu.ru [Department of Particle Physics and Cosmology, Physics Faculty, M.V. Lomonosov Moscow State University, Vorobjevy Gory, 119991 Moscow (Russian Federation)

2017-08-01

We study 'classical' bouncing and Genesis models in beyond Horndeski theory. We give an example of spatially flat bouncing solution that is non-singular and stable throughout the whole evolution. We also provide an example of stable geodesically complete Genesis with similar features. The model is arranged in such a way that the scalar field driving the cosmological evolution initially behaves like full-fledged beyond Horndeski, whereas at late times it becomes a massless scalar field minimally coupled to gravity.

Chemically-induced photoreceptor degeneration and protection in mouse iPSC-derived three-dimensional retinal organoids

Directory of Open Access Journals (Sweden)

Shin-ichiro Ito

2017-10-01

Full Text Available Induced pluripotent stem cells (iPSCs, which can be differentiated into various tissues and cell types, have been used for clinical research and disease modeling. Self-organizing three-dimensional (3D tissue engineering has been established within the past decade and enables researchers to obtain tissues and cells that almost mimic in vivo development. However, there are no reports of practical experimental procedures that reproduce photoreceptor degeneration. In this study, we induced photoreceptor cell death in mouse iPSC-derived 3D retinal organoids (3D-retinas by 4-hydroxytamoxifen (4-OHT, which induces photoreceptor degeneration in mouse retinal explants, and then established a live-cell imaging system to measure degeneration-related properties. Furthermore, we quantified the protective effects of representative ophthalmic supplements for treating the photoreceptor degeneration. This drug evaluation system enables us to monitor drug effects in photoreceptor cells and could be useful for drug screening.

Calcium homeostasis in fly photoreceptor cells

NARCIS (Netherlands)

Oberwinkler, J

2002-01-01

In fly photoreceptor cells, two processes dominate the Ca2+ homeostasis: light-induced Ca2+ influx through members of the TRP family of ion channels, and Ca2+ extrusion by Na+/Ca2+ exchange.Ca2+ release from intracellular stores is quantitatively insignificant. Both, the light-activated channels and

Responses of crayfish photoreceptor cells following intense light adaptation.

Science.gov (United States)

Cummins, D R; Goldsmith, T H

1986-01-01

After intense orange adapting exposures that convert 80% of the rhodopsin in the eye to metarhodopsin, rhabdoms become covered with accessory pigment and appear to lose some microvillar order. Only after a delay of hours or even days is the metarhodopsin replaced by rhodopsin (Cronin and Goldsmith 1984). After 24 h of dark adaptation, when there has been little recovery of visual pigment, the photoreceptor cells have normal resting potentials and input resistances, and the reversal potential of the light response is 10-15 mV (inside positive), unchanged from controls. The log V vs log I curve is shifted about 0.6 log units to the right on the energy axis, quantitatively consistent with the decrease in the probability of quantum catch expected from the lowered concentration of rhodopsin in the rhabdoms. Furthermore, at 24 h the photoreceptors exhibit a broader spectral sensitivity than controls, which is also expected from accumulations of metarhodopsin in the rhabdoms. In three other respects, however, the transduction process appears to be light adapted: The voltage responses are more phasic than those of control photoreceptors. The relatively larger effect (compared to controls) of low extracellular Ca++ (1 mmol/l EGTA) in potentiating the photoresponses suggests that the photoreceptors may have elevated levels of free cytoplasmic Ca++. The saturating depolarization is only about 30% as large as the maximal receptor potentials of contralateral, dark controls, and by that measure the log V-log I curve is shifted downward by 0.54 log units.(ABSTRACT TRUNCATED AT 250 WORDS)

Control rod drives

International Nuclear Information System (INIS)

Nakamura, Akira.

1984-01-01

Purpose: To enable to monitor the coupling state between a control rod and a control rod drive. Constitution: After the completion of a control rod withdrawal, a coolant pressure is applied to a control rod drive being adjusted so as to raise only the control rod drive and, in a case where the coupling between the control rod drive and the control rod is detached, the former is elevated till it contacts the control rod and then stopped. The actual stopping position is detected by an actual position detection circuit and compared with a predetermined position stored in a predetermined position detection circuit. If both of the positions are not aligned with each other, it is judged by a judging circuit that the control rod and the control rod drives are not combined. (Sekiya, K.)

Analysis of macular cone photoreceptors in a case of occult macular dystrophy

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Tojo N

2013-05-01

Full Text Available Naoki Tojo Tomoko Nakamura Hironori Ozaki Miyako Oka Toshihiko Oiwake Atsushi HayashiDepartment of Ophthalmology, University of Toyama, Toyama, JapanPurpose: To investigate changes in cone photoreceptors with adaptive optics (AO fundus imaging and spectral domain optical coherence tomography (SD-OCT in a case of occult macular dystrophy (OMD.Patient and methods: Both eyes of a 42-year-old woman diagnosed with OMD were examined. We used an AO fundus camera to obtain images of cone photoreceptors in the macula of the OMD subject and five healthy control subjects. Correlations between the AO images and the SD-OCT images were examined. Cone photoreceptors in eight areas in the macula of OMD and healthy control subjects were analyzed and compared.Results: SD-OCT showed a loss of the cone outer-segment tips line outside of the fovea in both eyes of the subject with OMD. The left eye with decreased visual acuity showed a discontinuous photoreceptor inner-segment and outer-segment line and cone outer-segment tips line at the fovea in SD-OCT and loss of cone mosaics as a dark spot in the AO image. In panoramic AO images and cone-density maps, less cone density was observed in a ring-like region outside the fovea than in the peripheral retina. In most of the areas examined, the cone densities were lower in the OMD eyes than in the healthy control eyes.Conclusions: Cone densities in the macula of the OMD patient were greatly decreased. AO images were found to be useful to evaluate morphologic changes in cone photoreceptors in patients with OMD.Keywords: occult macular dystrophy, adaptive optics, cone photoreceptor, cone analysis, optical coherence tomography

Necrotic enlargement of cone photoreceptor cells and the release of high-mobility group box-1 in retinitis pigmentosa

Science.gov (United States)

Murakami, Y; Ikeda, Y; Nakatake, S; Tachibana, T; Fujiwara, K; Yoshida, N; Notomi, S; Nakao, S; Hisatomi, T; Miller, J W; Vavvas, DG; Sonoda, KH; Ishibashi, T

2015-01-01

Retinitis pigmentosa (RP) refers to a group of inherited retinal degenerations resulting form rod and cone photoreceptor cell death. The rod cell death due to deleterious genetic mutations has been shown to occur mainly through apoptosis, whereas the mechanisms and features of the secondary cone cell death have not been fully elucidated. Our previous study showed that the cone cell death in rd10 mice, an animal model of RP, involves necrotic features and is partly mediated by the receptor interacting protein kinase. However, the relevancy of necrotic cone cell death in human RP patients remains unknown. In the present study, we showed that dying cone cells in rd10 mice exhibited cellular enlargement, along with necrotic changes such as cellular swelling and mitochondrial rupture. In human eyes, live imaging of cone cells by adaptive optics scanning laser ophthalmoscopy revealed significantly increased percentages of enlarged cone cells in the RP patients compared with the control subjects. The vitreous of the RP patients contained significantly higher levels of high-mobility group box-1, which is released extracellularly associated with necrotic cell death. These findings suggest that necrotic enlargement of cone cells is involved in the process of cone degeneration, and that necrosis may be a novel target to prevent or delay the loss of cone-mediated central vision in RP. PMID:27551484

Control rod assembly

International Nuclear Information System (INIS)

Takahashi, Akio.

1982-01-01

Purpose: To enable reliable insertion and drops of control rods, as well as insure a sufficient flow rate of coolants flowing through the control rods for attaining satisfactory cooling thereof to enable relexation of thermal stress resulted to rectifying mechanisms or the likes. Constitution: To the outer circumference of a control rod contained vertically movably within a control rod guide tube, resistive members are retractably provided in such a way as to project to close the gap between outer circumference of the control rod and the inner surface of the control rod guide tube upon engagement of a gripper of control rod drives, and retract upon release of the engagement of the gripper. Thus, since the resistive members project to provide a greater resistance to the coolants flowing between them and the control rod guide tube in the normal operation where the gripper is engaged to drive the control rod by the control rod drives, a major part of the coolant flowing into the control rod guide tube flows into the control rod. This enables to cool the control rod effectively and make the temperature distribution uniform for the coolant flowing from the upper end of the control rod guide tube to thereby attain the relaxation of the thermal stress resulted in the rectifying mechanisms or the likes. (Moriyama, K.)

In vitro transdifferentiation of human peripheral blood mononuclear cells to photoreceptor-like cells

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Yukari Komuta

2016-06-01

Full Text Available Direct reprogramming is a promising, simple and low-cost approach to generate target cells from somatic cells without using induced pluripotent stem cells. Recently, peripheral blood mononuclear cells (PBMCs have attracted considerable attention as a somatic cell source for reprogramming. As a cell source, PBMCs have an advantage over dermal fibroblasts with respect to the ease of collecting tissues. Based on our studies involving generation of photosensitive photoreceptor cells from human iris cells and human dermal fibroblasts by transduction of photoreceptor-related transcription factors via retrovirus vectors, we transduced these transcription factors into PBMCs via Sendai virus vectors. We found that retinal disease-related genes were efficiently detected in CRX-transduced cells, most of which are crucial to photoreceptor functions. In functional studies, a light-induced inward current was detected in some CRX-transduced cells. Moreover, by modification of the culture conditions including additional transduction of RAX1 and NEUROD1, we found a greater variety of retinal disease-related genes than that observed in CRX-transduced PBMCs. These data suggest that CRX acts as a master control gene for reprogramming PBMCs into photoreceptor-like cells and that our induced photoreceptor-like cells might contribute to individualized drug screening and disease modeling of inherited retinal degeneration.

Device for coupling a control rod and control rod drive

International Nuclear Information System (INIS)

Nishioka, Kazuya.

1975-01-01

Object: To obtain simple and reliable coupling between a control rod and control rod drive by equipping the lower end of the control rod with an extension provided with lateral protuberances and forming the upper end of an index tube with a recess provided with lateral holes. Structure: The tapering central extension of the control rod is inserted into the recess by lowering the control rod, and then it is further inserted by causing frictional movement of the inclined surfaces of lateral protuberances in frictional contact with guide surfaces. When the lateral protuberances are brought into contact with a stepped portion, the control rod is rotated to fit the lateral protuberances into the lateral holes. In this way, the control rod is coupled to the index tube of the control rod drive. (Yoshino, Y.)

Gibberellin and auxin influence the diurnal transcription pattern of photoreceptor genes via CRY1a in tomato.

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Paolo Facella

Full Text Available Plant photoreceptors, phytochromes and cryptochromes, regulate many aspects of development and growth, such as seed germination, stem elongation, seedling de-etiolation, cotyledon opening, flower induction and circadian rhythms. There are several pieces of evidence of interaction between photoreceptors and phyto-hormones in all of these physiological processes, but little is known about molecular and genetic mechanisms underlying hormone-photoreceptor crosstalk.In this work, we investigated the molecular effects of exogenous phyto-hormones to photoreceptor gene transcripts of tomato wt, as well as transgenic and mutant lines with altered cryptochromes, by monitoring day/night transcript oscillations. GA and auxin alter the diurnal expression level of different photoreceptor genes in tomato, especially in mutants that lack a working form of cryptochrome 1a: in those mutants the expression of some (IAA or most (GA photoreceptor genes is down regulated by these hormones.Our results highlight the presence of molecular relationships among cryptochrome 1a protein, hormones, and photoreceptors' gene expression in tomato, suggesting that manipulation of cryptochromes could represent a good strategy to understand in greater depth the role of phyto-hormones in the plant photoperceptive mechanism.

Control rod displacement

International Nuclear Information System (INIS)

Nakazato, S.

1987-01-01

This patent describes a nuclear reactor including a core, cylindrical control rods, a single support means supporting the control rods from their upper ends in spaced apart positions and movable for displacing the control rods in their longitudinal direction between a first end position in which the control rods are fully inserted into the core and a second end position in which the control rods are retracted from the core, and guide means contacting discrete regions of the outer surface of each control rod at least when the control rods are in the vicinity of the second end position. The control rods are supported by the support means for longitudinal movement without rotation into and out of the core relative to the guide means to thereby cause the outer surface of the control rods to experience wear as a result of sliding contact with the guide means. The support means are so arranged with respect to the core and the guide means that it is incapable of rotation relative to the guide means. The improvement comprises displacement means being operatively coupled to a respective one of the control rods for periodically rotating the control rod in a single angular direction through an angle selected to change the locations on the outer surfaces of the control rods at which the control rods are contacted by the guide means during subsequent longitudinal movement of the control rods

Morphoelastic rods. Part I: A single growing elastic rod

KAUST Repository

Moulton, D.E.

2013-02-01

A theory for the dynamics and statics of growing elastic rods is presented. First, a single growing rod is considered and the formalism of three-dimensional multiplicative decomposition of morphoelasticity is used to describe the bulk growth of Kirchhoff elastic rods. Possible constitutive laws for growth are discussed and analysed. Second, a rod constrained or glued to a rigid substrate is considered, with the mismatch between the attachment site and the growing rod inducing stress. This stress can eventually lead to instability, bifurcation, and buckling. © 2012 Elsevier Ltd. All rights reserved.

Morphoelastic rods. Part I: A single growing elastic rod

KAUST Repository

Moulton, D.E.; Lessinnes, T.; Goriely, A.

2013-01-01

A theory for the dynamics and statics of growing elastic rods is presented. First, a single growing rod is considered and the formalism of three-dimensional multiplicative decomposition of morphoelasticity is used to describe the bulk growth of Kirchhoff elastic rods. Possible constitutive laws for growth are discussed and analysed. Second, a rod constrained or glued to a rigid substrate is considered, with the mismatch between the attachment site and the growing rod inducing stress. This stress can eventually lead to instability, bifurcation, and buckling. © 2012 Elsevier Ltd. All rights reserved.

Shaggy Photoreceptors with Subfoveal Fluid Associated with a Distant Choroidal Melanoma

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Ann Q. Tran

2015-01-01

Full Text Available Purpose. To describe the enhanced depth imaging optical coherence tomography (EDI-OCT findings in a patient with an extra macula choroidal melanoma before and after treatment. Methods. Observational case report. Results. A 45 year-old Caucasian male patient was referred to retina clinic for management of choroidal melanoma. Examination revealed a nasal choroidal melanoma while EDI-OCT illustrated subfoveal fluid pocket with elongated shaggy photoreceptors distant and separate from the tumor. The patient was treated with plaque brachytherapy and intravitreal bevacizumab. One week after plaque removal, there was a dramatic reduction in the shaggy photoreceptors. Conclusion. Choroidal melanomas have effects that are not localized to the area of the tumor. This loculated pocket of subretinal fluid and coinciding changes to photoreceptor morphology may be related to global changes in choroidal function or release of tumor related cytokines.

Control rod drive

International Nuclear Information System (INIS)

Hawke, B.C.

1986-01-01

A reactor core, one or more control rods, and a control rod drive are described for selectively inserting and withdrawing the one or more control rods into and from the reactor core, which consists of: a support structure secured beneath the reactor core; control rod positioning means supported by the support structure for movably supporting the control rod for movement between a lower position wherein the control rod is located substantially beneath the reactor core and an upper position wherein at least an upper portion of the control rod extends into the reactor core; transmission means; primary drive means connected with the control rod positioning means by the transmission means for positioning the control rod under normal operating conditions; emergency drive means for moving the control rod from the lower position to the upper position under emergency conditions, the emergency drive means including a weight movable between an upper and a lower position, means for movably supporting the weight, and means for transmitting gravitational force exerted on the weight to the control rod positioning means to move the control rod upwardly when the weight is pulled downwardly by gravity; the transmission means connecting the control rod positioning means with the emergency drive means so that the primary drive means effects movement of the weight and the control rod in opposite directions under normal conditions, thus providing counterbalancing to reduce the force required for upward movement of the control rod under normal conditions; and restraint means for restraining the fall of the weight under normal operating conditions and disengaging the primary drive means to release the weight under emergency conditions

Retinal neuroprotection by hypoxic preconditioning is independent of hypoxia-inducible factor-1 alpha expression in photoreceptors.

Science.gov (United States)

Thiersch, Markus; Lange, Christina; Joly, Sandrine; Heynen, Severin; Le, Yun Zheng; Samardzija, Marijana; Grimm, Christian

2009-06-01

Hypoxic preconditioning stabilizes hypoxia-inducible factor (HIF) 1 alpha in the retina and protects photoreceptors against light-induced cell death. HIF-1 alpha is one of the major transcription factors responding to low oxygen tension and can differentially regulate a large number of target genes. To analyse whether photoreceptor-specific expression of HIF-1 alpha is essential to protect photoreceptors by hypoxic preconditioning, we knocked down expression of HIF-1 alpha specifically in photoreceptor cells, using the cyclization recombinase (Cre)-lox system. The Cre-mediated knockdown caused a 20-fold reduced expression of Hif-1 alpha in the photoreceptor cell layer. In the total retina, RNA expression was reduced by 65%, and hypoxic preconditioning led to only a small increase in HIF-1 alpha protein levels. Accordingly, HIF-1 target gene expression after hypoxia was significantly diminished. Retinas of Hif-1 alpha knockdown animals did not show any pathological alterations, and tolerated hypoxic exposure in a comparable way to wild-type retinas. Importantly, the strong neuroprotective effect of hypoxic preconditioning against light-induced photoreceptor degeneration persisted in knockdown mice, suggesting that hypoxia-mediated survival of light exposure does not depend on an autocrine action of HIF-1 alpha in photoreceptor cells. Hypoxia-mediated stabilization of HIF-2 alpha and phosphorylation of signal transducer and activator of transcription 3 (STAT 3) were not affected in the retinas of Hif-1 alpha knockdown mice. Thus, these factors are candidates for regulating the resistance of photoreceptors to light damage after hypoxic preconditioning, along with several potentially neuroprotective genes that were similarly induced in hypoxic knockdown and control mice.

Genesis and Development of Consolidated Financial Statements

OpenAIRE

Kostyantyn Bezverkhiy

2015-01-01

In the context of economy globalization it is the mission of consolidated financial statements to meet demands of users for financial information about activities of a company group as one company. The article is devoted to study of genesis and development of consolidated financial statements of companies. Historical prerequisites of genesis of consolidated financial statements are shown as well as factors which conduced to their spreading. The approaches are elucidated to preparation of cons...

Short-term psychosocial stress protects photoreceptors from damage via corticosterone-mediated activation of the AKT pathway.

Science.gov (United States)

Forkwa, Tembei K; Neumann, Inga D; Tamm, Ernst R; Ohlmann, Andreas; Reber, Stefan O

2014-02-01

Apoptotic death of photoreceptors in hereditary retinal degenerations can be prevented by neuroprotective molecules. Here, we report that adrenal glucocorticoids (GC) released during psychosocial stress protect photoreceptors from apoptosis after light damage. Psychosocial stress is known to be the main type of stressor humans are exposed to and was induced here in mice by 10h of chronic subordinate colony housing (CSC). Photoreceptor damage was generated by subsequent exposure to white light. Short-term psychosocial stress prior to illumination significantly reduced the number of apoptotic photoreceptors, an effect that was absent in adrenalectomized (ADX) mice. The neuroprotective effect was completely restored in ADX mice substituted with GC. Moreover, phosphorylation of retinal AKT increased following CSC or exogenous GC treatment, an effect that was again absent in ADX mice exposed to CSC. Finally, inhibition of AKT signaling with triciribine blocked the stress- and GC-mediated neuroprotective effects on photoreceptors. In summary, we provide evidence that 1) short-term psychosocial stress protects photoreceptors from light-induced damage and 2) the protective effect is most likely mediated by GC-induced activation of the AKT signaling pathway. Copyright © 2013 Elsevier Inc. All rights reserved.

Genesis machines

CERN Document Server

Amos, Martyn

2014-01-01

Silicon chips are out. Today's scientists are using real, wet, squishy, living biology to build the next generation of computers. Cells, gels and DNA strands are the 'wetware' of the twenty-first century. Much smaller and more intelligent, these organic computers open up revolutionary possibilities. Tracing the history of computing and revealing a brave new world to come, Genesis Machines describes how this new technology will change the way we think not just about computers - but about life itself.

Optical properties of photoreceptor and retinal pigment epithelium cells investigated with adaptive optics optical coherence tomography

Science.gov (United States)

Liu, Zhuolin

Human vision starts when photoreceptors collect and respond to light. Photoreceptors do not function in isolation though, but share close interdependence with neighboring photoreceptors and underlying retinal pigment epithelium (RPE) cells. These cellular interactions are essential for normal function of the photoreceptor-RPE complex, but methods to assess these in the living human eye are limited. One approach that has gained increased promise is high-resolution retinal imaging that has undergone tremendous technological advances over the last two decades to probe the living retina at the cellular level. Pivotal in these advances has been adaptive optics (AO) and optical coherence tomography (OCT) that together allow unprecedented spatial resolution of retinal structures in all three dimensions. Using these high-resolution systems, cone photoreceptor are now routinely imaged in healthy and diseased retina enabling fundamental structural properties of cones to be studied such as cell spacing, packing arrangement, and alignment. Other important cell properties, however, have remained elusive to investigation as even better imaging performance is required and thus has resulted in an incomplete understanding of how cells in the photoreceptor-RPE complex interact with light. To address this technical bottleneck, we expanded the imaging capability of AO-OCT to detect and quantify more accurately and completely the optical properties of cone photoreceptor and RPE cells at the cellular level in the living human retina. The first objective of this thesis was development of a new AO-OCT method that is more precise and sensitive, thus enabling a more detailed view of the 3D optical signature of the photoreceptor-RPE complex than was previously possible (Chapter 2). Using this new system, the second objective was quantifying the waveguide properties of individual cone photoreceptor inner and outer segments across the macula (Chapter 3). The third objective extended the AO

Interaction of two photoreceptors in the regulation of bacterial photosynthesis genes

OpenAIRE

Metz, Sebastian; Haberzettl, Kerstin; Frühwirth, Sebastian; Teich, Kristin; Hasewinkel, Christian; Klug, Gabriele

2012-01-01

The expression of photosynthesis genes in the facultatively photosynthetic bacterium Rhodobacter sphaeroides is controlled by the oxygen tension and by light quantity. Two photoreceptor proteins, AppA and CryB, have been identified in the past, which are involved in this regulation. AppA senses light by its N-terminal BLUF domain, its C-terminal part binds heme and is redox-responsive. Through its interaction to the transcriptional repressor PpsR the AppA photoreceptor controls expression of ...

Extracellular electrical activity from the photoreceptors of midge

Indian Academy of Sciences (India)

Unknown

349. Keywords. Chironomus; electroretinogram; insect development; midge; photoreceptor ... ceran insects, only larval ocelli of mosquito (Family: Culi- cidae) have been ... and Ball (1995) studied the influence of light in Chiro- nomus tentans ...

Replacement rod

International Nuclear Information System (INIS)

Hatfield, S.C.

1989-01-01

This patent describes in an elongated replacement rod for use with fuel assemblies of the type having two end fittings connected by guide tubes with a plurality of rod and guide tube cell defining spacer grids containing rod support features and mixing vanes. The grids secured to the guide tubes in register between the end fittings at spaced intervals. The fuel rod comprising: an asymmetrically beveled tip; a shank portion having a straight centerline; and a permanently diverging portion between the tip and the shank portion

Water rod

International Nuclear Information System (INIS)

Kashiwai, Shin-ichi; Yokomizo, Osamu; Orii, Akihito.

1992-01-01

In a reactor core of a BWR type reactor, the area of a flow channel in a lower portion of a downcoming pipe for downwardly releasing steams present at the top portion in a water rod is increased. Further, a third coolant flow channel (an inner water rod) is disposed in an uprising having an exit opened near the inlet of the water rod and an inlet opened at the outside near the top portion of the water and having an increase flow channel area in the upper portion. The downcoming pipe in the water rod is filled with steams, and the void ratio is increased by so much as the flow channel area of the downcoming pipe is increased. Since the pressure difference between the inlet and the exit of the inner water rod is greater than the pressure difference between the inlet and the exit of the water rod, most of water flown into the inner water rod is discharged out of the exit in the form of water as it is. Since the area of the flow channel is increased in the portion of the inner water rod, void efficiency in the upper portion of the reactor core is decreased by so much. Since the void ratio is thus increased in the lower portion and the void efficiency is decreased in the upper portion of the reactor core, axial void distribution can be flattened. (N.H.)

Discussion on the genesis of Zhongchuan uranium deposit

International Nuclear Information System (INIS)

Zhang Yulong; Zhang Chengzhong

2008-01-01

Through elaborating the geological setting, deposit and orebody geological charactors and hydrological features, the ore controlling factors are analysed and the genesis of Zhongchuan uranium deposit is discussed in the way of deposit occurrence, mineral asembleage and matalization ages. It is believed that uranium deposit was formed under the regional uplifting background with the exogenous mechanism and its genesis belongs to surface leaching. (authors)

Genesis / Juta Kivimäe

Index Scriptorium Estoniae

Kivimäe, Juta, 1952-

2003-01-01

Poola skulptori Magdalena Schmidt-G̤ra installatsioonid "Genesis I, II" rahvusraamatukogu fuajees ja peanäitusesaalis, kus oli samal ajal eksponeeritud eesti nahakunstnike Luule Maari ja Ruuda Maarandi köitenäitus

Multimodal Imaging of Photoreceptor Structure in Choroideremia.

Science.gov (United States)

Sun, Lynn W; Johnson, Ryan D; Williams, Vesper; Summerfelt, Phyllis; Dubra, Alfredo; Weinberg, David V; Stepien, Kimberly E; Fishman, Gerald A; Carroll, Joseph

2016-01-01

Choroideremia is a progressive X-linked recessive dystrophy, characterized by degeneration of the retinal pigment epithelium (RPE), choroid, choriocapillaris, and photoreceptors. We examined photoreceptor structure in a series of subjects with choroideremia with particular attention to areas bordering atrophic lesions. Twelve males with clinically-diagnosed choroideremia and confirmed hemizygous mutations in the CHM gene were examined. High-resolution images of the retina were obtained using spectral domain optical coherence tomography (SD-OCT) and both confocal and non-confocal split-detector adaptive optics scanning light ophthalmoscope (AOSLO) techniques. Eleven CHM gene mutations (3 novel) were identified; three subjects had the same mutation and one subject had two mutations. SD-OCT findings included interdigitation zone (IZ) attenuation or loss in 10/12 subjects, often in areas with intact ellipsoid zones; RPE thinning in all subjects; interlaminar bridges in the imaged areas of 10/12 subjects; and outer retinal tubulations (ORTs) in 10/12 subjects. Only split-detector AOSLO could reliably resolve cones near lesion borders, and such cones were abnormally heterogeneous in morphology, diameter and density. On split-detector imaging, the cone mosaic terminated sharply at lesion borders in 5/5 cases examined. Split-detector imaging detected remnant cone inner segments within ORTs, which were generally contiguous with a central patch of preserved retina. Early IZ dropout and RPE thinning on SD-OCT are consistent with previously published results. Evidence of remnant cone inner segments within ORTs and the continuity of the ORTs with preserved retina suggests that these may represent an intermediate state of retinal degeneration prior to complete atrophy. Taken together, these results supports a model of choroideremia in which the RPE degenerates before photoreceptors.

Retinitis pigmentosa: genes and disease mechanisms.

Science.gov (United States)

Ferrari, Stefano; Di Iorio, Enzo; Barbaro, Vanessa; Ponzin, Diego; Sorrentino, Francesco S; Parmeggiani, Francesco

2011-06-01

Retinitis pigmentosa (RP) is a group of inherited disorders affecting 1 in 3000-7000 people and characterized by abnormalities of the photoreceptors (rods and cones) or the retinal pigment epithelium of the retina which lead to progressive visual loss. RP can be inherited in an autosomal dominant, autosomal recessive or X-linked manner. While usually limited to the eye, RP may also occur as part of a syndrome as in the Usher syndrome and Bardet-Biedl syndrome. Over 40 genes have been associated with RP so far, with the majority of them expressed in either the photoreceptors or the retinal pigment epithelium. The tremendous heterogeneity of the disease makes the genetics of RP complicated, thus rendering genotype-phenotype correlations not fully applicable yet. In addition to the multiplicity of mutations, in fact, different mutations in the same gene may cause different diseases. We will here review which genes are involved in the genesis of RP and how mutations can lead to retinal degeneration. In the future, a more thorough analysis of genetic and clinical data together with a better understanding of the genotype-phenotype correlation might allow to reveal important information with respect to the likelihood of disease development and choices of therapy.

Identification of the sodium-calcium exchanger as the major ricin-binding glycoprotein of bovine rod outer segments and its localization to the plasma membrane

International Nuclear Information System (INIS)

Reid, D.M.; Molday, R.S.; Friedel, U.; Cook, N.J.

1990-01-01

After neuraminidase treatment the Na + /Ca 2+ exchanger of bovine rod outer segments was found to specifically bind Ricinus communis agglutinin. SDS gel electrophoresis and Western blotting of ricin-binding proteins purified from rod outer segment membranes by lectin affinity chromatography revealed the existence of two major polypeptides of M r 215K and 103K, the former of which was found to specifically react with PMe 1B3, a monoclonal antibody specific for the 230-kDa non-neuraminidase-treated Na + /Ca 2+ exchanger. Reconstitution of the ricin affinity-purified exchanger into calcium-containing liposomes revealed that neuraminidase treatment had no significant effect on the kinetics of Na + /Ca 2+ exchange activation by sodium. The authors further investigated the density of the Na + /Ca 2+ exchanger in disk and plasma membrane preparations using Western blotting, radioimmunoassays, immunoelectron microscopy, and reconstitution procedures. The results indicate that the Na + /Ca 2+ exchanger is localized in the rod photoreceptor plasma membrane and is absent or present in extremely low concentrations in disk membranes, as they have previously shown to be the case for the cGMP-gated cation channel. Previous reports describing the existence of Na + /Ca 2+ exchange activity in rod outer segment disk membrane preparations may be due to the fusion of plasma membrane components and/or the presence of contaminating plasma membrane vesicles

Microarray-based mutation analysis of the ABCA4 (ABCR) gene in autosomal recessive cone-rod dystrophy and retinitis pigmentosa.

Science.gov (United States)

Klevering, B Jeroen; Yzer, Suzanne; Rohrschneider, Klaus; Zonneveld, Marijke; Allikmets, Rando; van den Born, L Ingeborgh; Maugeri, Alessandra; Hoyng, Carel B; Cremers, Frans P M

2004-12-01

Mutations in the ABCA4 gene have been associated with autosomal recessive Stargardt disease (STGD1), cone-rod dystrophy (CRD), and retinitis pigmentosa (RP). We employed a recently developed genotyping microarray, the ABCR400-chip, to search for known ABCA4 mutations in patients with isolated or autosomal recessive CRD (54 cases) or RP (90 cases). We performed detailed ophthalmologic examinations and identified at least one ABCA4 mutation in 18 patients (33%) with CRD and in five patients (5.6%) with RP. Single-strand conformation polymorphism (SSCP) analysis and subsequent DNA sequencing revealed four novel missense mutations (R24C, E161K, P597S, G618E) and a novel 1-bp deletion (5888delG). Ophthalmoscopic abnormalities in CRD patients ranged from minor granular pigmentary changes in the posterior pole to widespread atrophy. In 12 patients with recordable electroretinogram (ERG) tracings, a cone-rod pattern was detected. Three patients demonstrated progression from a retinal dystrophy resembling STGD1 to a more widespread degeneration, and were subsequently diagnosed as CRD. In addition to a variable degree of atrophy, all RP patients displayed ophthalmologic characteristics of classic RP. When detectable, ERG recordings in these patients demonstrated rod-cone patterns of photoreceptor degeneration. In conclusion, in this study, we show that the ABCA4 mutation chip is an efficient first screening tool for arCRD.

Control rod

International Nuclear Information System (INIS)

Igarashi, Takao; Yoshimoto, Yuichiro; Sugawara, Satoshi; Fukumoto, Takashi; Endo, Zen-ichiro; Saito, Shozo; Shinpo, Katsutoshi; Nishimura, Akira; Ozawa, Michihiro

1988-01-01

Purpose: To provide a sufficient shutdown margin upon reactor shutdown, prevent sheath deformation without decreasing neutron absorbents and prevent impact shocks exerted to structural materials. Constitution: The control rod of the present invention comprises a neutron absorption region, a sheath deformation means attached to the side wall and means for restricting and supporting axial movement of the neutron absorbent rod. Then, the amount of absorptive nuclei chained absorbents in the lower region is reduced than that in the upper region. In this way, effective neutron absorbing performance can be obtained relative to the neutron importance distribution during reactor shutdown. In addition, since the operationability is improved by reducing the weight of the control rod and the absorptive nuclei chained neutron abosrbers are used, mechanical nuclear life of the control rod can be increased. Thus, it is possible to prevent the outward deformation of the sheath, as well as prevent collision between the neutron absorber rod and the structural material on the side of inserting the control rod generated upon reactor scram by a simple structure. (Kamimura, M.)

Characterization of Multiple Light Damage Paradigms Reveals Regional Differences in Photoreceptor Loss

OpenAIRE

Thomas, Jennifer L.; Nelson, Craig M.; Luo, Xixia; Hyde, David R.; Thummel, Ryan

2012-01-01

Zebrafish provide an attractive model to study the retinal response to photoreceptor apoptosis due to its remarkable ability to spontaneously regenerate retinal neurons following damage. There are currently two widely used light-induced retinal degeneration models to damage photoreceptors in the adult zebrafish. One model uses constant bright light, whereas the other uses a short exposure to extremely intense ultraviolet light. Although both models are currently used, it is unclear whether th...

Control rod drives

International Nuclear Information System (INIS)

Futatsugi, Masao.

1980-01-01

Purpose: To secure the reactor operation safety by the provision of a fluid pressure detecting section for control rod driving fluid and a control rod interlock at the midway of the flow pass for supplying driving fluid to the control rod drives. Constitution: Between a driving line and a direction control valve are provided a pressure detecting portion, an alarm generating device, and a control rod inhibition interlock. The driving fluid from a driving fluid source is discharged by way of a pump and a manual valve into the reactor in which the control rods and reactor fuels are contained. In addition, when the direction control valve is switched and the control rods are inserted and extracted by the control rod drives, the pressure in the driving line is always detected by the pressure detection section, whereby if abnormal pressure is resulted, the alarm generating device is actuated to warn the abnormality and the control rod inhibition interlock is actuated to lock the direction control valve thereby secure the safety operation of the reactor. (Seki, T.)

Control rod drives

International Nuclear Information System (INIS)

Oonuki, Koji.

1981-01-01

Purpose: To increase the driving speed of control rods at rapid insertion with an elongate control rod and an extension pipe while ensuring sufficient buffering performance in a short buffering distance, by providing a plurality of buffers to an extension pipe between a control rod drive source and a control rod in LMFBR type reactor. Constitution: First, second and third buffers are respectively provided to an acceleration piston, an extension pipe and a control rod respectively and the insertion positions for each of the buffers are displaced orderly from above to below. Upon disconnection of energizing current for an electromagnet, the acceleration piston, the extension pipe and the control rod are rapidly inserted in one body. The first, second and third buffers are respectively actuated at each of their falling strokes upon rapid insertion respectively, and the acceleration piston, the extension pipe and the control rod receive the deceleration effect in the order correspondingly. Although the compression force is applied to the control rod only near the stroke end, it does not cause deformation. (Kawakami, Y.)

Automated Photoreceptor Cell Identification on Nonconfocal Adaptive Optics Images Using Multiscale Circular Voting.

Science.gov (United States)

Liu, Jianfei; Jung, HaeWon; Dubra, Alfredo; Tam, Johnny

2017-09-01

Adaptive optics scanning light ophthalmoscopy (AOSLO) has enabled quantification of the photoreceptor mosaic in the living human eye using metrics such as cell density and average spacing. These rely on the identification of individual cells. Here, we demonstrate a novel approach for computer-aided identification of cone photoreceptors on nonconfocal split detection AOSLO images. Algorithms for identification of cone photoreceptors were developed, based on multiscale circular voting (MSCV) in combination with a priori knowledge that split detection images resemble Nomarski differential interference contrast images, in which dark and bright regions are present on the two sides of each cell. The proposed algorithm locates dark and bright region pairs, iteratively refining the identification across multiple scales. Identification accuracy was assessed in data from 10 subjects by comparing automated identifications with manual labeling, followed by computation of density and spacing metrics for comparison to histology and published data. There was good agreement between manual and automated cone identifications with overall recall, precision, and F1 score of 92.9%, 90.8%, and 91.8%, respectively. On average, computed density and spacing values using automated identification were within 10.7% and 11.2% of the expected histology values across eccentricities ranging from 0.5 to 6.2 mm. There was no statistically significant difference between MSCV-based and histology-based density measurements (P = 0.96, Kolmogorov-Smirnov 2-sample test). MSCV can accurately detect cone photoreceptors on split detection images across a range of eccentricities, enabling quick, objective estimation of photoreceptor mosaic metrics, which will be important for future clinical trials utilizing adaptive optics.

Control rod cluster with removable rods for nuclear fuel assembly

International Nuclear Information System (INIS)

Denizou, J.P.

1989-01-01

For each removable control rod, the open end section of the sleeve has a certain length of reduced diameter with openings in its wall. The top end of the rod is joined to an extension tube that surrounds the shaft over part of its lenght. This extension tube fits over the reduced part of the sleeve when the shaft is screwed into the bore of the sleeve. Rotation of the rod in the sleeve is prevented by deforming the extension tube locally in the openings of the end part of the sleeve. The rod is dismantled by exerting a torque on it using a gripping area near the end of the rod [fr

Photonic crystal light collectors in fish retina improve vision in turbid water.

Science.gov (United States)

Kreysing, Moritz; Pusch, Roland; Haverkate, Dorothee; Landsberger, Meik; Engelmann, Jacob; Ruiter, Janina; Mora-Ferrer, Carlos; Ulbricht, Elke; Grosche, Jens; Franze, Kristian; Streif, Stefan; Schumacher, Sarah; Makarov, Felix; Kacza, Johannes; Guck, Jochen; Wolburg, Hartwig; Bowmaker, James K; von der Emde, Gerhard; Schuster, Stefan; Wagner, Hans-Joachim; Reichenbach, Andreas; Francke, Mike

2012-06-29

Despite their diversity, vertebrate retinae are specialized to maximize either photon catch or visual acuity. Here, we describe a functional type that is optimized for neither purpose. In the retina of the elephantnose fish (Gnathonemus petersii), cone photoreceptors are grouped together within reflecting, photonic crystal-lined cups acting as macroreceptors, but rod photoreceptors are positioned behind these reflectors. This unusual arrangement matches rod and cone sensitivity for detecting color-mixed stimuli, whereas the photoreceptor grouping renders the fish insensitive to spatial noise; together, this enables more reliable flight reactions in the fish's dim and turbid habitat as compared with fish lacking this retinal specialization.

Control rod drive mechanism

International Nuclear Information System (INIS)

Nakamura, Akira.

1981-01-01

Purpose: To ensure the scram operation of a control rod by the reliable detection for the position of control rods. Constitution: A permanent magnet is provided to the lower portion of a connecting rod in engagement with a control rod and a tube having a plurality of lead switches arranged axially therein in a predetermined pitch is disposed outside of the control rod drives. When the control rod moves upwardly in the scram operation, the lead switches are closed successively upon passage of the permanent magnet to operate the electrical circuit provided by way of each of the lead switches. Thus, the position for the control rod during the scram can reliably be determined and the scram characteristic of the control rod can be recognized. (Furukawa, Y.)

Finestructure of the retina in Garra rufa (cypriniae, Teleostei)

International Nuclear Information System (INIS)

Al-Adhami, A. M.; Mir, S.

1999-01-01

The light - and dark-adapted retina of the freshwater, bottom-dweller tele ost, Ga rra rufa (Heck el, 1843) was studied under light and electron microscopes. The fish is a fist record in having both falcifrom process and vit real blood circulation and the hyaloid artery from which it developers. A number of acute vision areas represented by increased density of ganglion cell soma ta are evident. The dark-adapted retina is characterized by notably large photoreceptor terminals (rod spherules and cone placidas). A rod spherules has single synaptic ribbon, whereas a cone pedicle has three to four. The inner nuclear layer is composed of the so meta of horizontal, bipolar and amsacrine cells in addition to nuclei of Muller cells. The outer nuclear layer, on the other hand, is composed of two-three rows of rod nuclei and one row of cone nuclei. The photoreceptor cells include rods and single and double cones. The rod outer segments have deep and/or shallow incisor. Cone ellipsoid may have ellipsosomes. These are shown to develop from one of the apical mitochondria of the ellipsoid- Retinomotor movement involves both the photoreceptor cells and the pigment epithelium. (authors). 11 refs., 14 figs

Photobiomodulation reduces photoreceptor death and regulates cytoprotection in early states of P23H retinal dystrophy

Science.gov (United States)

Kirk, Diana K.; Gopalakrishnan, Sandeep; Schmitt, Heather; Abroe, Betsy; Stoehr, Michele; Dubis, Adam; Carroll, Joseph; Stone, Jonathan; Valter, Krisztina; Eells, Janis

2013-03-01

Irradiation by light in the far-red to near-infrared (NIR) region of the spectrum (photobiomodulation, PBM) has been demonstrated to attenuate the severity of neurodegenerative disease in experimental and clinical studies. The purpose of this study was to test the hypothesis that 670 nm PBM would protect against the loss of retinal function and improve photoreceptor survival in a rodent model of retinitis pigmentosa, the P23H transgenic rat. P23H rat pups were treated once per day with a 670 nm LED array (180 sec treatments at 50 mW/cm2; fluence 9 joules/cm2) (Quantum Devices Inc., Barneveld WI) from postnatal day (p) 16-20 or from p10-20. Sham-treated rats were restrained, but not exposed to NIR light. The status of the retina was determined at p22 by assessment of mitochondrial function, oxidative stress and cell death. In a second series of studies, retinal status was assessed at p30 by measuring photoreceptor function by ERG and retinal morphology by Spectral Domain Optical Coherence Tomography (SD-OCT). 670 nm PBM increased retinal mitochondrial cytochrome oxidase activity and upregulated the retina's production of the key mitochondrial antioxidant enzyme, MnSOD. PBM also attenuated photoreceptor cell loss and improved photoreceptor function. PBM protects photoreceptors in the developing P23H retina, by augmenting mitochondrial function and stimulating antioxidant protective pathways. Photobiomodulation may have therapeutic potential, where mitochondrial damage is a step in the death of photoreceptors.

Mef2d is essential for the maturation and integrity of retinal photoreceptor and bipolar cells.

Science.gov (United States)

Omori, Yoshihiro; Kitamura, Tamiki; Yoshida, Satoyo; Kuwahara, Ryusuke; Chaya, Taro; Irie, Shoichi; Furukawa, Takahisa

2015-05-01

Mef2 transcription factors play a crucial role in cardiac and skeletal muscle differentiation. We found that Mef2d is highly expressed in the mouse retina and its loss causes photoreceptor degeneration similar to that observed in human retinitis pigmentosa patients. Electroretinograms (ERGs) were severely impaired in Mef2d-/- mice. Immunohistochemistry showed that photoreceptor and bipolar cell synapse protein levels severely decreased in the Mef2d-/- retina. Expression profiling by microarray analysis showed that Mef2d is required for the expression of various genes in photoreceptor and bipolar cells, including cone arrestin, Guca1b, Pde6h and Cacna1s, which encode outer segment and synapse proteins. We also observed that Mef2d synergistically activates the cone arrestin (Arr3) promoter with Crx, suggesting that functional cooperation between Mef2d and Crx is important for photoreceptor cell gene regulation. Taken together, our results show that Mef2d is essential for photoreceptor and bipolar cell gene expression, either independently or cooperatively with Crx. © 2015 Institution for Protein Research. Genes to Cells published by Wiley Publishing Asia Pty Ltd and the Molecular Biology Society of Japan.

On the genesis of the uraniferous deposits I; Consideraciones sobre la genesis de los yacimientos uraniferos. I.

Energy Technology Data Exchange (ETDEWEB)

Mingarro, E

1964-07-01

The main problems of the genesis of uranium deposits as hydro thermals are objectively considered here under three aspects: uranium source transport and deposition. The transport of uranium can be effected under a tetravalent form, or as complex ions of hexavalent uranium: as uranyl ion (UO{sub 2}){sup 2}+ or under complex carbonic or sulfuric forms, such as UO{sub 2}(XO{sub n}){sub 2}{sup 2}- or UO{sub 2}(XO{sub n}){sub 3}{sup 4}-. These three ways of transport correspond to the three basic geochemical para genesis of uranium: uranium-titanium, uranium-cobalt, uranium. Deposition is currently made by reduction and in some way is no dependent of mineralogical association. (Author) 61 refs.

Method of inserting fuel rod

International Nuclear Information System (INIS)

Kamimoto, Shuji; Imoo, Makoto; Tsuchida, Kenji.

1991-01-01

The present invention concerns a method of inserting a fuel rod upon automatic assembling, automatic dismantling and reassembling of a fuel assembly in a light water moderated reactor, as well as a device and components used therefor. That is, a fuel rod is inserted reliably to an aimed point of insertion by surrounding the periphery of the fuel rod to be inserted with guide rods, and thereby suppressing the movement of the fuel rod during insertion. Alternatively, a fuel rod is inserted reliably to a point of insertion by inserting guide rods at the periphery of the point of insertion for the fuel rod to be inserted thereby surrounding the point of insertion with the guide rods or fuel rods. By utilizing fuel rods already present in the fuel assembly as the guide rods described above, the fuel rod can be inserted reliably to the point of insertion with no additional devices. Dummy fuel rods are previously inserted in a fuel assembly which are then utilized as the above-mentioned guide rods to accurately insert the fuel rod to the point of insertion. (I.S.)

Absence of synaptic regulation by phosducin in retinal slices.

Directory of Open Access Journals (Sweden)

James H Long

Full Text Available Phosducin is an abundant photoreceptor protein that binds G-protein βγ subunits and plays a role in modulating synaptic transmission at photoreceptor synapses under both dark-adapted and light-adapted conditions in vivo. To examine the role of phosducin at the rod-to-rod bipolar cell (RBC synapse, we used whole-cell voltage clamp recordings to measure the light-evoked currents from both wild-type (WT and phosducin knockout (Pd(-/- RBCs, in dark- and light-adapted retinal slices. Pd(-/- RBCs showed smaller dim flash responses and steeper intensity-response relationships than WT RBCs, consistent with the smaller rod responses being selectively filtered out by the non-linear threshold at the rod-to-rod bipolar synapse. In addition, Pd(-/- RBCs showed a marked delay in the onset of the light-evoked currents, similar to that of a WT response to an effectively dimmer flash. Comparison of the changes in flash sensitivity in the presence of steady adapting light revealed that Pd(-/- RBCs desensitized less than WT RBCs to the same intensity. These results are quantitatively consistent with the smaller single photon responses of Pd(-/- rods, owing to the known reduction in rod G-protein expression levels in this line. The absence of an additional synaptic phenotype in these experiments suggests that the function of phosducin at the photoreceptor synapse is abolished by the conditions of retinal slice recordings.

Control rod drives

International Nuclear Information System (INIS)

Hayakawa, Hiroyasu.

1979-01-01

Purpose: To enable rapid control in a simple circuit by providing a motor control device having an electric capacity capable of simultaneously driving all of the control rods rapidly only in the inserting direction as well as a motor controlling device capable of fine control for the insertion and extraction at usual operation. Constitution: The control rod drives comprise a first motor control device capable of finely controlling the control rods both in inserting and extracting directions, a second motor control device capable of rapidly driving the control rods only in the inserting direction, and a first motor switching circuit and a second motor switching circuit switched by switches. Upon issue of a rapid insertion instruction for the control rods, the second motor switching circuit is closed by the switch and the second motor control circuit and driving motors are connected. Thus, each of the control rod driving motors is driven at a high speed in the inserting direction to rapidly insert all of the control rods. (Yoshino, Y.)

Fission reactor control rod

International Nuclear Information System (INIS)

Irie, Tomoo.

1991-01-01

The present invention concerns a control rod in a PWR type reactor. A control rod has an inner cladding tube and an outer cladding tube disposed coaxially, and a water draining hole is formed at the inside of the inner cladding tube. Neutron absorbers are filled in an annular gap between the outer cladding tube and the inner cladding tube. The water draining hole opens at the lower end thereof to the top end of the control rod and at the upper end thereof to the side of the upper end plug of the control rod. If the control rod is dropped to a control rod guide thimble for reactor scram, coolants from the control rod guide thimble are flown from the lower end of the water draining hole and discharged from the upper end passing through the water draining hole. In this way, water from the control rod guide thimble is removed easily when the control rod is dropped. Further, the discharging amount of water itself is reduced by the provision of the water draining hole. Accordingly, sufficient control rod dropping speed can be attained. (I.N.)

Fuel rod simulator effects in flooding experiments single rod tests

International Nuclear Information System (INIS)

Nishida, M.

1984-09-01

The influence of a gas filled gap between cladding and pellet on the quenching behavior of a PWR fuel rod during the reflood phase of a LOCA has been investigated. Flooding experiments were conducted with a short length electrically heated single fuel rod simulator surrounded by glass housing. The gap of 0.05 mm width between the Zircaloy cladding and the internal Al 2 O 3 pellets of the rod was filled either wit helium or with argon to vary the radial heat resistance across the gap. This report presents some typical data and an evaluation of the reflood behavior of the fuel rod simulator used. The results show that the quench front propagates faster for increasing heat resistance in the gap between cladding and heat source of the rod. (orig.) [de

Activin Signals through SMAD2/3 to Increase Photoreceptor Precursor Yield during Embryonic Stem Cell Differentiation.

Science.gov (United States)

Lu, Amy Q; Popova, Evgenya Y; Barnstable, Colin J

2017-09-12

In vitro differentiation of mouse embryonic stem cells (ESCs) into retinal fates can be used to study the roles of exogenous factors acting through multiple signaling pathways during retina development. Application of activin A during a specific time frame that corresponds to early embryonic retinogenesis caused increased generation of CRX + photoreceptor precursors and decreased PAX6 + retinal progenitor cells (RPCs). Following activin A treatment, SMAD2/3 was activated in RPCs and bound to promoter regions of key RPC and photoreceptor genes. The effect of activin on CRX expression was repressed by pharmacological inhibition of SMAD2/3 phosphorylation. Activin signaling through SMAD2/3 in RPCs regulates expression of transcription factors involved in cell type determination and promotes photoreceptor lineage specification. Our findings can contribute to the production of photoreceptors for cell replacement therapy. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Maximum/minimum asymmetric rod detection

International Nuclear Information System (INIS)

Huston, J.T.

1990-01-01

This patent describes a system for determining the relative position of each control rod within a control rod group in a nuclear reactor. The control rod group having at least three control rods therein. It comprises: means for producing a signal representative of a position of each control rod within the control rod group in the nuclear reactor; means for establishing a signal representative of the highest position of a control rod in the control rod group in the nuclear reactor; means for establishing a signal representative of the lowest position of a control rod in the control rod group in the nuclear reactor; means for determining a difference between the signal representative of the position of the highest control rod and the signal representative of the position of the lowest control rod; means for establishing a predetermined limit for the difference between the signal representative of the position of the highest control rod and the signal representative of the position of the lowest control rod; and means for comparing the difference between the signals with the predetermined limit. The comparing means producing an output signal when the difference between the signals exceeds the predetermined limit

Simple explant culture of the embryonic chicken retina with long-term preservation of photoreceptors.

Science.gov (United States)

Thangaraj, Gopenath; Greif, Alexander; Layer, Paul G

2011-10-01

Structurally stable in vitro-model systems are indispensible to analyse neural development during embryogenesis, follow cellular differentiation and evaluate neurotoxicological or growth factor effects. Here we describe a three-dimensional, long-term in vitro-culture system of the embryonic chick retina which supports photoreceptor development. Retinal tissue was isolated from E6 chick eye, and cultured as explants by continuous orbital rotation to allow free floatation without any supporting materials. Young stage (E6) immature retinas were cultured for various time periods in order to follow the differentiation of cell types and plexiform layers by immunocytochemical methods. These explants could be cultured for at least 2-3 weeks with remarkable retention of retinal architecture. Interestingly, photoreceptors developed in the absence of pigment epithelium. Electron microscopic studies revealed formation of structures resembling photoreceptor outer segments, a feature not reported previously. Thus, the verification of photoreceptors, Müller cells, inner retinal cells and the inner plexiform layer described in our study establishes this explant culture as a valuable in vivo-like model system. Crown Copyright © 2011. Published by Elsevier Ltd. All rights reserved.

Melanopsin-expressing amphioxus photoreceptors transduce light via a phospholipase C signaling cascade.

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Juan Manuel Angueyra

Full Text Available Melanopsin, the receptor molecule that underlies light sensitivity in mammalian 'circadian' receptors, is homologous to invertebrate rhodopsins and has been proposed to operate via a similar signaling pathway. Its downstream effectors, however, remain elusive. Melanopsin also expresses in two distinct light-sensitive cell types in the neural tube of amphioxus. This organism is the most basal extant chordate and can help outline the evolutionary history of different photoreceptor lineages and their transduction mechanisms; moreover, isolated amphioxus photoreceptors offer unique advantages, because they are unambiguously identifiable and amenable to single-cell physiological assays. In the present study whole-cell patch clamp recording, pharmacological manipulations, and immunodetection were utilized to investigate light transduction in amphioxus photoreceptors. A G(q was identified and selectively localized to the photosensitive microvillar membrane, while the pivotal role of phospholipase C was established pharmacologically. The photocurrent was profoundly depressed by IP₃ receptor antagonists, highlighting the importance of IP₃ receptors in light signaling. By contrast, surrogates of diacylglycerol (DAG, as well as poly-unsaturated fatty acids failed to activate a membrane conductance or to alter the light response. The results strengthen the notion that calcium released from the ER via IP₃-sensitive channels may fulfill a key role in conveying--directly or indirectly--the melanopsin-initiated light signal to the photoconductance; moreover, they challenge the dogma that microvillar photoreceptors and phoshoinositide-based light transduction are a prerogative of invertebrate eyes.

Control rod drives

International Nuclear Information System (INIS)

Hayakawa, Hiroyasu; Kawamura, Atsuo.

1979-01-01

Purpose: To reduce pellet-clad mechanical interactions, as well as improve the fuel safety. Constitution: In the rod drive of a bwr type reactor, an electric motor operated upon intermittent input such as of pulse signals is connected to a control rod. A resolver for converting the rotational angle of the motor to electric signals is connected to the rotational shaft of the motor and the phase difference between the output signal from the resolver and a reference signal is adapted to detect by a comparator. Based on the detection result, the controller is actuated to control a motor for control rod drive so that fine control for the movement of the control rod is made possible. This can reduce the moving distance of the control rod, decrease the thermal stress applied to the control rod and decrease the pellet clad mechanical interaction failures due to thermal expansion between the cladding tube and the pellets caused by abrupt changes in the generated power. (Furukawa, Y.)

OFD1, as a Ciliary Protein, Exhibits Neuroprotective Function in Photoreceptor Degeneration Models.

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Juan Wang

Full Text Available Ofd1 is a newly identified causative gene for Retinitis pigmentosa (RP, a photoreceptor degenerative disease. This study aimed to examine Ofd1 localization in retina and further to investigate its function in photoreceptor degeneration models. Ofd1 localization in rat retina was examined using immunofluorescence. N-methyl-N-nitrosourea (MNU-induced rats and Royal College of Surgeons (RCS rats were used as photoreceptor degeneration models. The expression pattern of Ofd1, other ciliary associated genes and Wnt signaling pathway genes were examined in rat models. Furthermore, pEGFP-Ofd1-CDS and pSUPER-Ofd1-shRNA were constructed to overexpress and knockdown the expression level in 661W and R28 cells. MNU was also used to induce cell death. Cilia formation was observed using immunocytochemistry (ICC. Reactive oxygen species (ROS were detected using the 2', 7'-Dichlorofluorescin diacetate (DCFH-DA assay. Apoptosis genes expression was examined using qRT-PCR, Western blotting and fluorescence-activated cell sorting (FACS. Ofd1 localized to outer segments of rat retina photoreceptors. Ofd1 and other ciliary proteins expression levels increased from the 1st and 4th postnatal weeks and decreased until the 6th week in the RCS rats, while their expression consistently decreased from the 1st and 7th day in the MNU rats. Moreover, Wnt signaling pathway proteins expression was significantly up-regulated in both rat models. Knockdown of Ofd1 expression resulted in a smaller population, shorter length of cell cilia, and lower cell viability. Ofd1 overexpression partially attenuated MNU toxic effects by reducing ROS levels and mitigating apoptosis. To the best of our knowledge, this is the first study demonstrating Ofd1 localization and its function in rat retina and in retinal degeneration rat models. Ofd1 plays a role in controlling photoreceptor cilium length and number. Importantly, it demonstrates a neuroprotective function by protecting the photoreceptor

The role of carcinine in signaling at the Drosophila photoreceptor synapse.

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Brendan A Gavin

2007-12-01

Full Text Available The Drosophila melanogaster photoreceptor cell has long served as a model system for researchers focusing on how animal sensory neurons receive information from their surroundings and translate this information into chemical and electrical messages. Electroretinograph (ERG analysis of Drosophila mutants has helped to elucidate some of the genes involved in the visual transduction pathway downstream of the photoreceptor cell, and it is now clear that photoreceptor cell signaling is dependent upon the proper release and recycling of the neurotransmitter histamine. While the neurotransmitter transporters responsible for clearing histamine, and its metabolite carcinine, from the synaptic cleft have remained unknown, a strong candidate for a transporter of either substrate is the uncharacterized inebriated protein. The inebriated gene (ine encodes a putative neurotransmitter transporter that has been localized to photoreceptor cells in Drosophila and mutations in ine result in an abnormal ERG phenotype in Drosophila. Loss-of-function mutations in ebony, a gene required for the synthesis of carcinine in Drosophila, suppress components of the mutant ine ERG phenotype, while loss-of-function mutations in tan, a gene necessary for the hydrolysis of carcinine in Drosophila, have no effect on the ERG phenotype in ine mutants. We also show that by feeding wild-type flies carcinine, we can duplicate components of mutant ine ERGs. Finally, we demonstrate that treatment with H(3 receptor agonists or inverse agonists rescue several components of the mutant ine ERG phenotype. Here, we provide pharmacological and genetic epistatic evidence that ine encodes a carcinine neurotransmitter transporter. We also speculate that the oscillations observed in mutant ine ERG traces are the result of the aberrant activity of a putative H(3 receptor.

The Role of Carcinine in Signaling at the Drosophila Photoreceptor Synapse

Science.gov (United States)

Gavin, Brendan A; Arruda, Susan E; Dolph, Patrick J

2007-01-01

The Drosophila melanogaster photoreceptor cell has long served as a model system for researchers focusing on how animal sensory neurons receive information from their surroundings and translate this information into chemical and electrical messages. Electroretinograph (ERG) analysis of Drosophila mutants has helped to elucidate some of the genes involved in the visual transduction pathway downstream of the photoreceptor cell, and it is now clear that photoreceptor cell signaling is dependent upon the proper release and recycling of the neurotransmitter histamine. While the neurotransmitter transporters responsible for clearing histamine, and its metabolite carcinine, from the synaptic cleft have remained unknown, a strong candidate for a transporter of either substrate is the uncharacterized inebriated protein. The inebriated gene (ine) encodes a putative neurotransmitter transporter that has been localized to photoreceptor cells in Drosophila and mutations in ine result in an abnormal ERG phenotype in Drosophila. Loss-of-function mutations in ebony, a gene required for the synthesis of carcinine in Drosophila, suppress components of the mutant ine ERG phenotype, while loss-of-function mutations in tan, a gene necessary for the hydrolysis of carcinine in Drosophila, have no effect on the ERG phenotype in ine mutants. We also show that by feeding wild-type flies carcinine, we can duplicate components of mutant ine ERGs. Finally, we demonstrate that treatment with H3 receptor agonists or inverse agonists rescue several components of the mutant ine ERG phenotype. Here, we provide pharmacological and genetic epistatic evidence that ine encodes a carcinine neurotransmitter transporter. We also speculate that the oscillations observed in mutant ine ERG traces are the result of the aberrant activity of a putative H3 receptor. PMID:18069895

Genesis and Evolution of Interfaces in Product Architecture

DEFF Research Database (Denmark)

Donmez, Mehmet; Hsuan, Juliana

Interfaces are elements of the product architecture that facilitates innovation and enables an organization to leverage the trade-off between cost and performance of its products. Despite the importance of interfaces for organizations, little is known about their genesis and evolution. In this st......Interfaces are elements of the product architecture that facilitates innovation and enables an organization to leverage the trade-off between cost and performance of its products. Despite the importance of interfaces for organizations, little is known about their genesis and evolution...

Atypical retinal pigment epithelial defects with retained photoreceptor layers

DEFF Research Database (Denmark)

Giannakaki-Zimmermann, Helena; Querques, Giuseppe; Munch, Inger Christine

2017-01-01

BACKGROUND: To report patients with age-related macular degeneration and atypical central retinal pigment epithelium (RPE) defects not attributable to geographic atrophy (GA) or RPE-tears with overlying preserved photoreceptor layers. METHODS: Multimodal imaging case-series evaluating the course...

Role of fractalkine/CX3CR1 interaction in light-induced photoreceptor degeneration through regulating retinal microglial activation and migration.

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Meng Zhang

Full Text Available BACKGROUND: Excessive exposure to light enhances the progression and severity of some human retinal degenerative diseases. While retinal microglia are likely to be important in neuron damage associated with these diseases, the relationship between photoreceptor damage and microglial activation remains poorly understood. Some recent studies have indicated that the chemokine fractalkine is involved in the pathogenesis of many neurodegenerative diseases. The present study was performed to investigate the cross-talk between injured photoreceptors and activated retinal microglia, focusing on the role of fractalkine and its receptor CX3CR1 in light-induced photoreceptor degeneration. METHODOLOGY/PRINCIPAL FINDINGS: Both in vivo and in vitro experiments were involved in the research. In vivo, Sprague-Dawley rats were exposed to blue light for 24 hours. In vitro, the co-culture of primary retinal microglia and a photoreceptor cell line (661W cell was exposed to blue light for five hours. Some cultures were pretreated by the addition of anti-CX3CR1 neutralizing antibody or recombinant fractalkine. Expression of fractalkine/CX3CR1 and inflammatory cytokines was detected by immunofluorescence, real-time PCR, Western immunoblot analysis, and ELISA assay. TUNEL method was used to detect cell apoptosis. In addition, chemotaxis assay was performed to evaluate the impact of soluble fractalkine on microglial migration. Our results showed that the expression of fractalkine that was significantly upregulated after exposure to light, located mainly at the photoreceptors. The extent of photoreceptor degeneration and microglial migration paralleled the increased level of fractalkine/CX3CR1. Compared with the control, the expression of inflammatory cytokines was significantly downregulated in the anti-CX3CR1 neutralizing antibody-treated group, and the number of photoreceptors was also well preserved. The addition of recombinant full-length fractalkine or soluble

Concentric retinitis pigmentosa: clinicopathologic correlations.

Science.gov (United States)

Milam, A H; De Castro, E B; Smith, J E; Tang, W X; John, S K; Gorin, M B; Stone, E M; Aguirre, G D; Jacobson, S G

2001-10-01

Progressive concentric (centripetal) loss of vision is one pattern of visual field loss in retinitis pigmentosa. This study provides the first clinicopathologic correlations for this form of retinitis pigmentosa. A family with autosomal dominant concentric retinitis pigmentosa was examined clinically and with visual function tests. A post-mortem eye of an affected 94 year old family member was processed for histopathology and immunocytochemistry with retinal cell specific antibodies. Unrelated simplex/multiplex patients with concentric retinitis pigmentosa were also examined. Affected family members of the eye donor and patients from the other families had prominent peripheral pigmentary retinopathy with more normal appearing central retina, good visual acuity, concentric field loss, normal or near normal rod and cone sensitivity within the preserved visual field, and reduced rod and cone electroretinograms. The eye donor, at age 90, had good acuity and function in a central island. Grossly, the central region of the donor retina appeared thinned but otherwise normal, while the far periphery contained heavy bone spicule pigment. Microscopically the central retina showed photoreceptor outer segment shortening and some photoreceptor cell loss. The mid periphery had a sharp line of demarcation where more central photoreceptors were near normal except for very short outer segments and peripheral photoreceptors were absent. Rods and cones showed abrupt loss of outer segments and cell death at this interface. It is concluded that concentric retinitis pigmentosa is a rare but recognizable phenotype with slowly progressive photoreceptor death from the far periphery toward the central retina. The disease is retina-wide but shows regional variation in severity of degeneration; photoreceptor death is severe in the peripheral retina with an abrupt edge between viable and degenerate photoreceptors. Peripheral to central gradients of unknown retinal molecule(s) may be defective

Status of rod consolidation

International Nuclear Information System (INIS)

Bailey, W.J.

1985-04-01

Two of the factors that need to be taken into account with rod consolidation are (1) the effects on rods from their removal from the fuel assembly and (2) the effects on rods as a result of the consolidation process. Potential components of both factors are described in the report. Discussed under (1) are scratches on the fuel rod surfaces, rod breakage, crud, extended burnup, and possible cladding embrittlement due to hydrogen injection at BWRs. Discussed under (2) are the increased water temperature (less than 10 0 C) because of closer packing of the rods, formation of crevices between rods in the close-packed mode, contact with dissimilar metals, and the potential for rapid heating of fuel rods following the loss of water from a spent fuel storage pool. Another factor that plays an important role in rod consolidation is the cost of disposal of the nonfuel-bearing components of the fuel assembly. Also, the dose rate from the components - especially Inconel spacer grids - can affect the handling procedures. Several licensing issues that exist are described. A list of recommendations is provided. 98 refs., 5 figs., 5 tabs

Xbp1-Independent Ire1 Signaling Is Required for Photoreceptor Differentiation and Rhabdomere Morphogenesis in Drosophila

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Dina S. Coelho

2013-11-01

Full Text Available The unfolded protein response (UPR is composed by homeostatic signaling pathways that are activated by excessive protein misfolding in the endoplasmic reticulum. Ire1 signaling is an important mediator of the UPR, leading to the activation of the transcription factor Xbp1. Here, we show that Drosophila Ire1 mutant photoreceptors have defects in the delivery of rhodopsin-1 to the rhabdomere and in the secretion of Spacemaker/Eyes Shut into the interrhabdomeral space. However, these defects are not observed in Xbp1 mutant photoreceptors. Ire1 mutant retinas have higher mRNA levels for targets of regulated Ire1-dependent decay (RIDD, including for the fatty acid transport protein (fatp. Importantly, the downregulation of fatp by RNAi rescues the rhodopsin-1 delivery defects observed in Ire1 mutant photoreceptors. Our results show that the role of Ire1 during photoreceptor differentiation is independent of Xbp1 function and demonstrate the physiological relevance of the RIDD mechanism in this specific paradigm.

An Unexpected Diversity of Photoreceptor Classes in the Longfin Squid, Doryteuthis pealeii.

Science.gov (United States)

Kingston, Alexandra C N; Wardill, Trevor J; Hanlon, Roger T; Cronin, Thomas W

2015-01-01

Cephalopods are famous for their ability to change color and pattern rapidly for signaling and camouflage. They have keen eyes and remarkable vision, made possible by photoreceptors in their retinas. External to the eyes, photoreceptors also exist in parolfactory vesicles and some light organs, where they function using a rhodopsin protein that is identical to that expressed in the retina. Furthermore, dermal chromatophore organs contain rhodopsin and other components of phototransduction (including retinochrome, a photoisomerase first found in the retina), suggesting that they are photoreceptive. In this study, we used a modified whole-mount immunohistochemical technique to explore rhodopsin and retinochrome expression in a number of tissues and organs in the longfin squid, Doryteuthis pealeii. We found that fin central muscles, hair cells (epithelial primary sensory neurons), arm axial ganglia, and sucker peduncle nerves all express rhodopsin and retinochrome proteins. Our findings indicate that these animals possess an unexpected diversity of extraocular photoreceptors and suggest that extraocular photoreception using visual opsins and visual phototransduction machinery is far more widespread throughout cephalopod tissues than previously recognized.

An Unexpected Diversity of Photoreceptor Classes in the Longfin Squid, Doryteuthis pealeii.

Directory of Open Access Journals (Sweden)

Alexandra C N Kingston

Full Text Available Cephalopods are famous for their ability to change color and pattern rapidly for signaling and camouflage. They have keen eyes and remarkable vision, made possible by photoreceptors in their retinas. External to the eyes, photoreceptors also exist in parolfactory vesicles and some light organs, where they function using a rhodopsin protein that is identical to that expressed in the retina. Furthermore, dermal chromatophore organs contain rhodopsin and other components of phototransduction (including retinochrome, a photoisomerase first found in the retina, suggesting that they are photoreceptive. In this study, we used a modified whole-mount immunohistochemical technique to explore rhodopsin and retinochrome expression in a number of tissues and organs in the longfin squid, Doryteuthis pealeii. We found that fin central muscles, hair cells (epithelial primary sensory neurons, arm axial ganglia, and sucker peduncle nerves all express rhodopsin and retinochrome proteins. Our findings indicate that these animals possess an unexpected diversity of extraocular photoreceptors and suggest that extraocular photoreception using visual opsins and visual phototransduction machinery is far more widespread throughout cephalopod tissues than previously recognized.

A Region-Based GeneSIS Segmentation Algorithm for the Classification of Remotely Sensed Images

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Stelios K. Mylonas

2015-03-01

Full Text Available This paper proposes an object-based segmentation/classification scheme for remotely sensed images, based on a novel variant of the recently proposed Genetic Sequential Image Segmentation (GeneSIS algorithm. GeneSIS segments the image in an iterative manner, whereby at each iteration a single object is extracted via a genetic-based object extraction algorithm. Contrary to the previous pixel-based GeneSIS where the candidate objects to be extracted were evaluated through the fuzzy content of their included pixels, in the newly developed region-based GeneSIS algorithm, a watershed-driven fine segmentation map is initially obtained from the original image, which serves as the basis for the forthcoming GeneSIS segmentation. Furthermore, in order to enhance the spatial search capabilities, we introduce a more descriptive encoding scheme in the object extraction algorithm, where the structural search modules are represented by polygonal shapes. Our objectives in the new framework are posed as follows: enhance the flexibility of the algorithm in extracting more flexible object shapes, assure high level classification accuracies, and reduce the execution time of the segmentation, while at the same time preserving all the inherent attributes of the GeneSIS approach. Finally, exploiting the inherent attribute of GeneSIS to produce multiple segmentations, we also propose two segmentation fusion schemes that operate on the ensemble of segmentations generated by GeneSIS. Our approaches are tested on an urban and two agricultural images. The results show that region-based GeneSIS has considerably lower computational demands compared to the pixel-based one. Furthermore, the suggested methods achieve higher classification accuracies and good segmentation maps compared to a series of existing algorithms.

Control rod drive

International Nuclear Information System (INIS)

Okutani, Tetsuro.

1988-01-01

Purpose: To provide a simple and economical control rod drive using a control circuit requiring no pulse circuit. Constitution: Control rods in a BWR type reactor are driven by hydraulic pressure and inserted or withdrawn in the direction of applying the hydraulic pressure. The direction of the hydraulic pressure is controlled by a direction control valve. Since the driving for the control rod is extremely important in view of the operation, a self diagnosis function is disposed for rapid inspection of possible abnormality. In the present invention, two driving contacts are disposed each by one between the both ends of a solenoid valve of the direction control valve for driving the control rod and the driving power source, and diagnosis is conducted by alternately operating them. Therefore, since it is only necessary that the control circuit issues a driving instruction only to one of the two driving contacts, the pulse circuit is no more required. Further, since the control rod driving is conducted upon alignment of the two driving instructions, the reliability of the control rod drive can be improved. (Horiuchi, T.)

N-retinylidene-phosphatidylethanolamine is the preferred retinoid substrate for the photoreceptor-specific ABC transporter ABCA4 (ABCR).

Science.gov (United States)

Beharry, Seelochan; Zhong, Ming; Molday, Robert S

2004-12-24

ABCA4, a member of the family of ATP binding cassette (ABC) proteins found in rod and cone photoreceptors, has been implicated in the transport of retinoid compounds across the outer segment disk membrane following the photoactivation of rhodopsin. Mutations in the ABCA4 gene are responsible for Stargardt macular dystrophy and related retinal degenerative diseases that cause a loss in vision. To identify the retinoid substrate that interacts with ABCA4, we have isolated ABCA4 from rod outer segment disk membranes on an immunoaffinity matrix and analyzed retinoid compounds that bind to ABCA4 using high performance liquid chromatography and radiolabeling methods. When all-trans-retinal was added to ABCA4 in the presence of phosphatidylethanolamine, approximately 0.9 mol of N-retinylidene-phosphatidylethanolamine and 0.3 mol of all-trans-retinal were bound per mol of ABCA4 with an apparent K(d) of 2-5 microm. ATP and GTP released these retinoids from ABCA4, whereas ADP, GDP, and nonhydrolyzable derivatives, adenosine 5'-(beta,gamma-imido)triphosphate and guanosine 5'-(beta,gamma-imido)triphosphate, were ineffective. One mole of N-retinyl-phosphatidylethanolamine, the reduced form of N-retinylidene-phosphatidylethanolamine, bound per mol of ABCA4, whereas 0.3 mol of all-trans-retinal were bound in the absence of phosphatidylethanolamine. No binding of all-trans-retinol to ABCA4 was observed. Our results indicate that ABCA4 preferentially binds N-retinylidene-phosphatidylethanolamine with high affinity in the absence of ATP. Our studies further suggest that ATP binding and hydrolysis induces a protein conformational change that causes N-retinylidene-phosphatidylethanolamine to dissociate from ABCA4.

Derivation of human differential photoreceptor-like cells from the iris by defined combinations of CRX, RX and NEUROD.

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Yuko Seko

Full Text Available Examples of direct differentiation by defined transcription factors have been provided for beta-cells, cardiomyocytes and neurons. In the human visual system, there are four kinds of photoreceptors in the retina. Neural retina and iris-pigmented epithelium (IPE share a common developmental origin, leading us to test whether human iris cells could differentiate to retinal neurons. We here define the transcription factor combinations that can determine human photoreceptor cell fate. Expression of rhodopsin, blue opsin and green/red opsin in induced photoreceptor cells were dependent on combinations of transcription factors: A combination of CRX and NEUROD induced rhodopsin and blue opsin, but did not induce green opsin; a combination of CRX and RX induced blue opsin and green/red opsin, but did not induce rhodopsin. Phototransduction-related genes as well as opsin genes were up-regulated in those cells. Functional analysis; i.e. patch clamp recordings, clearly revealed that generated photoreceptor cells, induced by CRX, RX and NEUROD, responded to light. The response was an inward current instead of the typical outward current. These data suggest that photosensitive photoreceptor cells can be generated by combinations of transcription factors. The combination of CRX and RX generate immature photoreceptors: and additional NEUROD promotes maturation. These findings contribute substantially to a major advance toward eventual cell-based therapy for retinal degenerative diseases.

Control rod withdrawal monitoring device

International Nuclear Information System (INIS)

Ebisuya, Mitsuo.

1984-01-01

Purpose: To prevent the power ramp even if a plurality of control rods are subjected to withdrawal operation at a time, by reducing the reactivity applied to the reactor. Constitution: The control rod withdrawal monitoring device is adapted to monitor and control the withdrawal of the control rods depending on the reactor power and the monitoring region thereof is divided into a control rod group monitoring region a transition region and a control group monitoring not interfere region. In a case if the distance between a plurality of control rods for which the withdrawal positions are selected is less than a limiting value, the coordinate for the control rods, distance between the control rods and that the control rod distance is shorter are displayed on a display panel, and the withdrawal for the control rods are blocked. Accordingly, even if a plurality of control rods are subjected successively to the withdrawal operation contrary to the control rod withdrawal sequence upon high power operation of the reactor, the power ramp can be prevented. (Kawakami, Y.)

Subretinally transplanted embryonic stem cells rescue photoreceptor cells from degeneration in the RCS rats.

Science.gov (United States)

Schraermeyer, U; Thumann, G; Luther, T; Kociok, N; Armhold, S; Kruttwig, K; Andressen, C; Addicks, K; Bartz-Schmidt, K U

2001-01-01

The Royal College of Surgeons (RCS) rat is an animal model for retinal degeneration such as the age-related macular degeneration. The RCS rat undergoes a progressive retinal degeneration during the early postnatal period. A potential treatment to prevent this retinal degeneration is the transplantation into the subretinal space of cells that would replace functions of the degenerating retinal pigment epithelium (RPE) cells or may form neurotrophic factors. In this study we have investigated the potential of subretinally transplanted embryonic stem cells to prevent the genetically determined photoreceptor cell degeneration in the RCS rat. Embryonic stem cells from the inner cell mass of the mouse blastocyst were allowed to differentiate to neural precursor cells in vitro and were then transplanted into the subretinal space of 20-day-old RCS rats. Transplanted and sham-operated rats were sacrificed 2 months following cell transplantation. The eyes were enucleated and photoreceptor degeneration was quantified by analyzing and determining the thickness of the outer nuclear layer by light and electron microscopy. In the eyes transplanted with embryonic cells up to 8 rows of photoreceptor cell nuclei were observed, whereas in nontreated control eyes the outer nuclear layer had degenerated completely. Transplantation of embryonic stem cells appears to delay photoreceptor cell degeneration in RCS rats.

Thermal behavior simulation of a nuclear fuel rod through an eletrically heated rod

International Nuclear Information System (INIS)

Lima, R. de C.F. de.

1984-01-01

In thermalhydraulic loops the nuclear industry often uses electrically heated rods to simulate power transients, which occur in nuclear fuel rods. The development and design of a electrically heated rod, by supplying the dimensions and materials which should be used in order to yeld the same temperature and heat flux at the surfaces of the nuclear rod and the electrically heated rod are presented. To a given nuclear transient this equality was obtained by fitting the linear power through the lumped parameters technique. (Author) [pt

Müller cells separate between wavelengths to improve day vision with minimal effect upon night vision.

Science.gov (United States)

Labin, Amichai M; Safuri, Shadi K; Ribak, Erez N; Perlman, Ido

2014-07-08

Vision starts with the absorption of light by the retinal photoreceptors-cones and rods. However, due to the 'inverted' structure of the retina, the incident light must propagate through reflecting and scattering cellular layers before reaching the photoreceptors. It has been recently suggested that Müller cells function as optical fibres in the retina, transferring light illuminating the retinal surface onto the cone photoreceptors. Here we show that Müller cells are wavelength-dependent wave-guides, concentrating the green-red part of the visible spectrum onto cones and allowing the blue-purple part to leak onto nearby rods. This phenomenon is observed in the isolated retina and explained by a computational model, for the guinea pig and the human parafoveal retina. Therefore, light propagation by Müller cells through the retina can be considered as an integral part of the first step in the visual process, increasing photon absorption by cones while minimally affecting rod-mediated vision.

Negative regulation of ciliary length by ciliary male germ cell-associated kinase (Mak) is required for retinal photoreceptor survival.

Science.gov (United States)

Omori, Yoshihiro; Chaya, Taro; Katoh, Kimiko; Kajimura, Naoko; Sato, Shigeru; Muraoka, Koichiro; Ueno, Shinji; Koyasu, Toshiyuki; Kondo, Mineo; Furukawa, Takahisa

2010-12-28

Cilia function as cell sensors in many organs, and their disorders are referred to as "ciliopathies." Although ciliary components and transport machinery have been well studied, regulatory mechanisms of ciliary formation and maintenance are poorly understood. Here we show that male germ cell-associated kinase (Mak) regulates retinal photoreceptor ciliary length and subcompartmentalization. Mak was localized both in the connecting cilia and outer-segment axonemes of photoreceptor cells. In the Mak-null retina, photoreceptors exhibit elongated cilia and progressive degeneration. We observed accumulation of intraflagellar transport 88 (IFT88) and IFT57, expansion of kinesin family member 3A (Kif3a), and acetylated α-tubulin signals in the Mak-null photoreceptor cilia. We found abnormal rhodopsin accumulation in the Mak-null photoreceptor cell bodies at postnatal day 14. In addition, overexpression of retinitis pigmentosa 1 (RP1), a microtubule-associated protein localized in outer-segment axonemes, induced ciliary elongation, and Mak coexpression rescued excessive ciliary elongation by RP1. The RP1 N-terminal portion induces ciliary elongation and increased intensity of acetylated α-tubulin labeling in the cells and is phosphorylated by Mak. These results suggest that Mak is essential for the regulation of ciliary length and is required for the long-term survival of photoreceptors.

Nuclear fuel rod loading apparatus

International Nuclear Information System (INIS)

King, H.B.; Macivergan, R.; Mckenzie, G.W.

1980-01-01

An apparatus incorporating a microprocessor control is provided for automatically loading nuclear fuel pellets into fuel rods commonly used in nuclear reactor cores. The apparatus comprises a split ''v'' trough for assembling segments of fuel pellets in rows and a shuttle to receive the fuel pellets from the split ''v'' trough when the two sides of the split ''v'' trough are opened. The pellets are weighed while in the shuttle, and the shuttle then moves the pellets into alignment with a fuel rod. A guide bushing is provided to assist the transfer of the pellets into the fuel rod. A rod carousel which holds a plurality of fuel rods presents the proper rod to the guide bushing at the appropriate stage in the loading sequence. The bushing advances to engage the fuel rod, and the shuttle advances to engage the guide bushing. The pellets are then loaded into the fuel rod by a motor operated push rod. The guide bushing includes a photocell utilized in conjunction with the push rod to measure the length of the row of fuel pellets inserted in the fuel rod

Control rod shutdown system

International Nuclear Information System (INIS)

Miyamoto, Yoshiyuki; Higashigawa, Yuichi.

1996-01-01

The present invention provides a control rod terminating system in a BWR type nuclear power plant, which stops an induction electric motor as rapidly as possible to terminate the control rods. Namely, the control rod stopping system controls reactor power by inserting/withdrawing control rods into a reactor by driving them by the induction electric motor. The system is provided with a control device for controlling the control rods and a control device for controlling the braking device. The control device outputs a braking operation signal for actuating the braking device during operation of the control rods to stop the operation of the control rods. Further, the braking device has at least two kinds of breaks, namely, a first and a second brakes. The two kinds of brakes are actuated by receiving the brake operation signals at different timings. The brake device is used also for keeping the control rods after the stopping. Even if a stopping torque of each of the breaks is small, different two kinds of brakes are operated at different timings thereby capable of obtaining a large stopping torque as a total. (I.S.)

RPGR: Its role in photoreceptor physiology, human disease, and future therapies.

Science.gov (United States)

Megaw, Roly D; Soares, Dinesh C; Wright, Alan F

2015-09-01

Mammalian photoreceptors contain specialised connecting cilia that connect the inner (IS) to the outer segments (OS). Dysfunction of the connecting cilia due to mutations in ciliary proteins are a common cause of the inherited retinal dystrophy retinitis pigmentosa (RP). Mutations affecting the Retinitis Pigmentosa GTPase Regulator (RPGR) protein is one such cause, affecting 10-20% of all people with RP and the majority of those with X-linked RP. RPGR is located in photoreceptor connecting cilia. It interacts with a wide variety of ciliary proteins, but its exact function is unknown. Recently, there have been important advances both in our understanding of RPGR function and towards the development of a therapy. This review summarises the existing literature on human RPGR function and dysfunction, and suggests that RPGR plays a role in the function of the ciliary gate, which controls access of both membrane and soluble proteins to the photoreceptor outer segment. We discuss key models used to investigate and treat RPGR disease and suggest that gene augmentation therapy offers a realistic therapeutic approach, although important questions still remain to be answered, while cell replacement therapy based on retinal progenitor cells represents a more distant prospect. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

Fuel rod leak detector

International Nuclear Information System (INIS)

Womack, R.E.

1978-01-01

A typical embodiment of the invention detects leaking fuel rods by means of a radiation detector that measures the concentration of xenon-133 ( 133 Xe) within each individual rod. A collimated detector that provides signals related to the energy of incident radiation is aligned with one of the ends of a fuel rod. A statistically significant sample of the gamma radiation (γ-rays) that characterize 133 Xe is accumulated through the detector. The data so accumulated indicates the presence of a concentration of 133 Xe appropriate to a sound fuel rod, or a significantly different concentration that reflects a leaking fuel rod

Fuel rods

International Nuclear Information System (INIS)

Hattori, Shinji; Kajiwara, Koichi.

1980-01-01

Purpose: To ensure the safety for the fuel rod failures by adapting plenum springs to function when small forces such as during transportation of fuel rods is exerted and not to function the resilient force when a relatively great force is exerted. Constitution: Between an upper end plug and a plenum spring in a fuel rod, is disposed an insertion member to the lower portion of which is mounted a pin. This pin is kept upright and causes the plenum spring to function resiliently to the pellets against the loads due to accelerations and mechanical vibrations exerted during transportation of the fuel rods. While on the other hand, if a compression force of a relatively high level is exerted to the plenum spring during reactor operation, the pin of the insertion member is buckled and the insertion member is inserted to the inside of the plenum spring, whereby the pellets are allowed to expand freely and the failures in the fuel elements can be prevented. (Moriyama, K.)

Adaptive optics fundus images of cone photoreceptors in the macula of patients with retinitis pigmentosa

Directory of Open Access Journals (Sweden)

Tojo N

2013-01-01

Full Text Available Naoki Tojo, Tomoko Nakamura, Chiharu Fuchizawa, Toshihiko Oiwake, Atsushi HayashiDepartment of Ophthalmology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, JapanBackground: The purpose of this study was to examine cone photoreceptors in the macula of patients with retinitis pigmentosa using an adaptive optics fundus camera and to investigate any correlations between cone photoreceptor density and findings on optical coherence tomography and fundus autofluorescence.Methods: We examined two patients with typical retinitis pigmentosa who underwent ophthalmological examination, including measurement of visual acuity, and gathering of electroretinographic, optical coherence tomographic, fundus autofluorescent, and adaptive optics fundus images. The cone photoreceptors in the adaptive optics images of the two patients with retinitis pigmentosa and five healthy subjects were analyzed.Results: An abnormal parafoveal ring of high-density fundus autofluorescence was observed in the macula in both patients. The border of the ring corresponded to the border of the external limiting membrane and the inner segment and outer segment line in the optical coherence tomographic images. Cone photoreceptors at the abnormal parafoveal ring were blurred and decreased in the adaptive optics images. The blurred area corresponded to the abnormal parafoveal ring in the fundus autofluorescence images. Cone densities were low at the blurred areas and at the nasal and temporal retina along a line from the fovea compared with those of healthy controls. The results for cone spacing and Voronoi domains in the macula corresponded with those for the cone densities.Conclusion: Cone densities were heavily decreased in the macula, especially at the parafoveal ring on high-density fundus autofluorescence in both patients with retinitis pigmentosa. Adaptive optics images enabled us to observe in vivo changes in the cone photoreceptors of

Lean Management Genesis

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Parkes Aneta

2015-12-01

Full Text Available Lean Management is a philosophy and management concept, based on reduction of the waste and resources used in the process of producing goods and providing services. Lean Management genesis dates back to scientific management in America (for example concepts of H. Ford and F.W. Taylor and quality management, including development of TQM concept. Japanese Toyota Production System has been inspired by chosen elements of these concepts, and then it evolved towards global concept called Toyota Way (which connects production rules with values and work attitude.

Lectures on Genesis 1:26-2:3 and 2:21-25

DEFF Research Database (Denmark)

Stjerna, Kirsi; Pedersen, Else Marie Wiberg

2017-01-01

This introduction to parts of Luther's lectures on Genesis (1535-45), namely his lectures on Genesis 1:26-2:3 and 2:21-25, discusses the reformer's grappling with the text in its historical and theological context. Not least is the question of gender taken under scrutiny, as the two authors, Kirsi...

Multiple fuel rod gripper

International Nuclear Information System (INIS)

Shields, E.P.

1987-01-01

An apparatus is described for gripping an array of rods comprising: (a) gripping members grippingly engageable with the rods, each of which has a hollow portion terminating in an open end for receiving the end of one of the rods; (b) a closing means for causing the hollow portion of each of the gripping members to apply substantially the same gripping force onto the end of its respective rod, including (i) a locking plate having a plurality of tapered holes registrable with the array of rods, wherein the exterior of each of the gripping members is tapered and nested within one of the tapered holes, (ii) a withdrawing means having a hydraulic plunger operatively connected to each of the gripping members for applying a substantially identical withdrawing force on each of the gripping members, whereby the hollow portion of each of the gripping members applies substantially the same gripping force on its respective rod, and (c) means for detecting whether each of the gripping members has grippingly engaged its respective rod

High-yield production of hydrophobins RodA and RodB from Aspergillus fumigatus in Pichia pastoris

DEFF Research Database (Denmark)

Pedersen, Mona Højgaard; Borodina, Irina; Moresco, Jacob Lange

2011-01-01

A as well as rRodB were able to convert a glass surface from hydrophilic to hydrophobic similar to native RodA, but only rRodB was able to decrease the hydrophobicity of a Teflon-like surface to the same extent as native RodA, while rRodA showed this ability to a lesser extent. Recombinant RodA and native...

UV-sensitive photoreceptor protein OPN5 in humans and mice.

Science.gov (United States)

Kojima, Daisuke; Mori, Suguru; Torii, Masaki; Wada, Akimori; Morishita, Rika; Fukada, Yoshitaka

2011-01-01

A variety of animal species utilize the ultraviolet (UV) component of sunlight as their environmental cues, whereas physiological roles of UV photoreception in mammals, especially in human beings, remain open questions. Here we report that mouse neuropsin (OPN5) encoded by the Opn5 gene exhibited an absorption maximum (λmax) at 380 nm when reconstituted with 11-cis-retinal. Upon UV-light illumination, OPN5 was converted to a blue-absorbing photoproduct (λmax 470 nm), which was stable in the dark and reverted to the UV-absorbing state by the subsequent orange light illumination, indicating its bistable nature. Human OPN5 also had an absorption maximum at 380 nm with spectral properties similar to mouse OPN5, revealing that OPN5 is the first and hitherto unknown human opsin with peak sensitivity in the UV region. OPN5 was capable of activating heterotrimeric G protein Gi in a UV-dependent manner. Immuno-blotting analyses of mouse tissue extracts identified the retina, the brain and, unexpectedly, the outer ears as the major sites of OPN5 expression. In the tissue sections of mice, OPN5 immuno-reactivities were detected in a subset of non-rod/non-cone retinal neurons as well as in the epidermal and muscle cells of the outer ears. Most of these OPN5-immuno-reactivities in mice were co-localized with positive signals for the alpha-subunit of Gi. These results demonstrate the first example of UV photoreceptor in human beings and strongly suggest that OPN5 triggers a UV-sensitive Gi-mediated signaling pathway in the mammalian tissues.

Overexpression of retinal degeneration slow (RDS protein adversely affects rods in the rd7 model of enhanced S-cone syndrome.

Directory of Open Access Journals (Sweden)

Dibyendu Chakraborty

Full Text Available The nuclear receptor NR2E3 promotes expression of rod photoreceptor genes while repressing cone genes. Mice lacking NR2E3 (Nr2e3(rd7/rd7 referred to here as rd7 are a model for enhanced S-cone syndrome, a disease associated with increased sensitivity to blue light and night blindness. Rd7 retinas have reduced levels of the outer segment (OS structural protein retinal degeneration slow (RDS. We test the hypothesis that increasing RDS levels would improve the Rd7 phenotype. Transgenic mice over-expressing normal mouse peripherin/RDS (NMP in rods and cones were crossed onto the rd7 background. Disease phenotypes were assessed in NMP/rd7 eyes and compared to wild-type (WT and rd7 eyes at postnatal day 30. NMP/rd7 retinas expressed total RDS (transgenic and endogenous message at WT levels, and NMP protein was correctly localized to the OS. NMP/rd7 retinas have shorter OSs compared to rd7 and WT and significantly reduced number of rosettes. NMP/rd7 mice also exhibited significant deficits in scotopic ERG amplitudes compared to rd7 while photopic amplitudes remained unaffected. Protein levels of rhodopsin, RDS, and the RDS homologue ROM-1 were significantly reduced in the NMP/rd7 retinas compared to rd7. We show that correcting the levels of RDS gene expression does not improve the phenotype of the rd7 suggesting that RDS deficiency is not responsible for the defect in this model. We suggest that the specific rod defect in the NMP/rd7 is likely associated with ongoing problems in the rd7 that are related to the expression of cone genes in rod cells, a characteristic of the model.

Adaptive optics fundus images of cone photoreceptors in the macula of patients with retinitis pigmentosa.

Science.gov (United States)

Tojo, Naoki; Nakamura, Tomoko; Fuchizawa, Chiharu; Oiwake, Toshihiko; Hayashi, Atsushi

2013-01-01

The purpose of this study was to examine cone photoreceptors in the macula of patients with retinitis pigmentosa using an adaptive optics fundus camera and to investigate any correlations between cone photoreceptor density and findings on optical coherence tomography and fundus autofluorescence. We examined two patients with typical retinitis pigmentosa who underwent ophthalmological examination, including measurement of visual acuity, and gathering of electroretinographic, optical coherence tomographic, fundus autofluorescent, and adaptive optics fundus images. The cone photoreceptors in the adaptive optics images of the two patients with retinitis pigmentosa and five healthy subjects were analyzed. An abnormal parafoveal ring of high-density fundus autofluorescence was observed in the macula in both patients. The border of the ring corresponded to the border of the external limiting membrane and the inner segment and outer segment line in the optical coherence tomographic images. Cone photoreceptors at the abnormal parafoveal ring were blurred and decreased in the adaptive optics images. The blurred area corresponded to the abnormal parafoveal ring in the fundus autofluorescence images. Cone densities were low at the blurred areas and at the nasal and temporal retina along a line from the fovea compared with those of healthy controls. The results for cone spacing and Voronoi domains in the macula corresponded with those for the cone densities. Cone densities were heavily decreased in the macula, especially at the parafoveal ring on high-density fundus autofluorescence in both patients with retinitis pigmentosa. Adaptive optics images enabled us to observe in vivo changes in the cone photoreceptors of patients with retinitis pigmentosa, which corresponded to changes in the optical coherence tomographic and fundus autofluorescence images.

On the genesis of the uraniferous deposits I

International Nuclear Information System (INIS)

Mingarro, E.

1964-01-01

The main problems of the genesis of uranium deposits as hydro thermals are objectively considered here under three aspects: uranium source transport and deposition. The transport of uranium can be effected under a tetravalent form, or as complex ions of hexavalent uranium: as uranyl ion (UO 2 ) 2 + or under complex carbonic or sulfuric forms, such as UO 2 (XO n ) 2 2 - or UO 2 (XO n ) 3 4 -. These three ways of transport correspond to the three basic geochemical para genesis of uranium: uranium-titanium, uranium-cobalt, uranium. Deposition is currently made by reduction and in some way is no dependent of mineralogical association. (Author) 61 refs

Pupillometer-based objective chromatic perimetry in normal eyes and patients with retinal photoreceptor dystrophies.

Science.gov (United States)

Skaat, Alon; Sher, Ifat; Kolker, Andrew; Elyasiv, Sivan; Rosenfeld, Elkana; Mhajna, Mohamad; Melamed, Shlomo; Belkin, Michael; Rotenstreich, Ygal

2013-04-17

To evaluate a novel objective perimetry using multifocal chromatic pupil light reflex in normal participants and patients with photoreceptor dysfunction, and to relate this new technique with subjective dark-adapted chromatic Goldmann perimetry. Thirty-two eyes of 17 retinitis pigmentosa (RP) or cone-rod dystrophy patients and 20 eyes of 12 healthy individuals were tested. A computerized infrared video pupillometer was used to record changes in pupil diameter in response to short- and long-wavelength stimuli (peak 485 and 640 nm, respectively; light intensity 40 cd/m(2)) at 13 different points of the 30° visual field (VF), under background illumination of 2.7 cd/m(2). The pupillary response (PR) of patients was compared with PR obtained from normal control participants. In 11 patients, the pupillary responses were also compared with their findings on dark-adapted chromatic Goldmann perimetry. Significantly reduced pupillary responses were obtained in RP patients in response to the short-wavelength stimulus in nearly all perimetric locations (P chromatic Goldmann perimetry. In all patients that were tested by the chromatic Goldmann, minimal PR was recorded in areas that were nondetected in the chromatic Goldmann perimetry. This study demonstrates the potential feasibility of using pupillometer-based chromatic perimetry for objectively assessing VF defects and retinal function in patients with retinal dystrophies. (ClinicalTrials.gov number, NCT01021982.).

Control rod drive for vertical movement

International Nuclear Information System (INIS)

Suskov, I.I.; Gorjunov, V.S.; Zajcev, B.I.; Derevjankin, N.E.; Petrov, V.A.; Istomin, S.D.; Kovalencik, D.I.; Archipov, E.A.; Serebrjakov, V.I.; Kacalin, V.S.

1982-01-01

The control of the rod repositioning gear unit and the control unit of the profile grab of the control rod drive for the alkali metal-cooled fast breeder reactor is achieved by an electromotor being arranged outside the hermetic drive casing. The guide tube is directly repositioned by the rod repositioning gear unit. Coupling control of the drive with the control rod is done in the lower operative position of the control rod and that because of the interaction of the tie rod arranged on the spring-mounted control rod with the induction transmitter for the lower position of the control rod. In the transfer position the rod is fixed within the guide tube. (orig.)

Safety rod driving device

International Nuclear Information System (INIS)

Murakami, Kiyonobu; Kurosaki, Akira.

1988-01-01

Purpose: To rapidly insert safety rods for a criticality experiment device into a reactor core container to stop the criticality reaction thereby prevent reactivity accidents. Constitution: A cylinder device having a safety rod as a cylinder rod attached with a piston at one end is constituted. The piston is elevated by pressurized air and attracted and fixed by an electromagnet which is a stationary device disposed at the upper portion of the cylinder. If the current supply to the electromagnet is disconnected, the safety rod constituting the cylinder rod is fallen together with the piston to the lower portion of the cylinder. Since the cylinder rod driving device has neither electrical motor nor driving screw as in the conventional device, necessary space can be reduced and the weight is decreased. In addition, since the inside of the nuclear reactor can easily be shielded completely from the external atmosphere, leakage of radioactive materials can be prevented. (Horiuchi, T.)

Scotopic vision in the monkey is modulated by the G protein-coupled receptor 55

DEFF Research Database (Denmark)

Bouskila, Joseph; Harrar, Vanessa; Javadi, Pasha

2016-01-01

The endogenous cannabinoid system plays important roles in the retina of mice and monkeys via their classic CB1 and CB2 receptors. We have previously reported that the G protein-coupled receptor 55 (GPR55), a putative cannabinoid receptor, is exclusively expressed in rod photoreceptors in the mon......The endogenous cannabinoid system plays important roles in the retina of mice and monkeys via their classic CB1 and CB2 receptors. We have previously reported that the G protein-coupled receptor 55 (GPR55), a putative cannabinoid receptor, is exclusively expressed in rod photoreceptors...

Defects in the outer limiting membrane are associated with rosette development in the Nrl-/- retina.

Directory of Open Access Journals (Sweden)

Michael W Stuck

Full Text Available The neural retinal leucine zipper (Nrl knockout mouse is a widely used model to study cone photoreceptor development, physiology, and molecular biology in the absence of rods. In the Nrl(-/- retina, rods are converted into functional cone-like cells. The Nrl(-/- retina is characterized by large undulations of the outer nuclear layer (ONL commonly known as rosettes. Here we explore the mechanism of rosette development in the Nrl(-/- retina. We report that rosettes first appear at postnatal day (P8, and that the structure of nascent rosettes is morphologically distinct from what is seen in the adult retina. The lumen of these nascent rosettes contains a population of aberrant cells protruding into the subretinal space that induce infolding of the ONL. Morphologically adult rosettes do not contain any cell bodies and are first detected at P15. The cells found in nascent rosettes are photoreceptors in origin but lack inner and outer segments. We show that the adherens junctions between photoreceptors and Müller glia which comprise the retinal outer limiting membrane (OLM are not uniformly formed in the Nrl(-/- retina and thus allow protrusion of a population of developing photoreceptors into the subretinal space where their maturation becomes delayed. These data suggest that the rosettes of the Nrl(-/- retina arise due to defects in the OLM and delayed maturation of a subset of photoreceptors, and that rods may play an important role in the proper formation of the OLM.

Python as a federation tool for GENESIS 3.0.

Directory of Open Access Journals (Sweden)

Hugo Cornelis

Full Text Available The GENESIS simulation platform was one of the first broad-scale modeling systems in computational biology to encourage modelers to develop and share model features and components. Supported by a large developer community, it participated in innovative simulator technologies such as benchmarking, parallelization, and declarative model specification and was the first neural simulator to define bindings for the Python scripting language. An important feature of the latest version of GENESIS is that it decomposes into self-contained software components complying with the Computational Biology Initiative federated software architecture. This architecture allows separate scripting bindings to be defined for different necessary components of the simulator, e.g., the mathematical solvers and graphical user interface. Python is a scripting language that provides rich sets of freely available open source libraries. With clean dynamic object-oriented designs, they produce highly readable code and are widely employed in specialized areas of software component integration. We employ a simplified wrapper and interface generator to examine an application programming interface and make it available to a given scripting language. This allows independent software components to be 'glued' together and connected to external libraries and applications from user-defined Python or Perl scripts. We illustrate our approach with three examples of Python scripting. (1 Generate and run a simple single-compartment model neuron connected to a stand-alone mathematical solver. (2 Interface a mathematical solver with GENESIS 3.0 to explore a neuron morphology from either an interactive command-line or graphical user interface. (3 Apply scripting bindings to connect the GENESIS 3.0 simulator to external graphical libraries and an open source three dimensional content creation suite that supports visualization of models based on electron microscopy and their conversion to

Python as a federation tool for GENESIS 3.0.

Science.gov (United States)

Cornelis, Hugo; Rodriguez, Armando L; Coop, Allan D; Bower, James M

2012-01-01

The GENESIS simulation platform was one of the first broad-scale modeling systems in computational biology to encourage modelers to develop and share model features and components. Supported by a large developer community, it participated in innovative simulator technologies such as benchmarking, parallelization, and declarative model specification and was the first neural simulator to define bindings for the Python scripting language. An important feature of the latest version of GENESIS is that it decomposes into self-contained software components complying with the Computational Biology Initiative federated software architecture. This architecture allows separate scripting bindings to be defined for different necessary components of the simulator, e.g., the mathematical solvers and graphical user interface. Python is a scripting language that provides rich sets of freely available open source libraries. With clean dynamic object-oriented designs, they produce highly readable code and are widely employed in specialized areas of software component integration. We employ a simplified wrapper and interface generator to examine an application programming interface and make it available to a given scripting language. This allows independent software components to be 'glued' together and connected to external libraries and applications from user-defined Python or Perl scripts. We illustrate our approach with three examples of Python scripting. (1) Generate and run a simple single-compartment model neuron connected to a stand-alone mathematical solver. (2) Interface a mathematical solver with GENESIS 3.0 to explore a neuron morphology from either an interactive command-line or graphical user interface. (3) Apply scripting bindings to connect the GENESIS 3.0 simulator to external graphical libraries and an open source three dimensional content creation suite that supports visualization of models based on electron microscopy and their conversion to computational models

Micro-calibration of space and motion by photoreceptors synchronized in parallel with cortical oscillations: A unified theory of visual perception.

Science.gov (United States)

Jerath, Ravinder; Cearley, Shannon M; Barnes, Vernon A; Jensen, Mike

2018-01-01

A fundamental function of the visual system is detecting motion, yet visual perception is poorly understood. Current research has determined that the retina and ganglion cells elicit responses for motion detection; however, the underlying mechanism for this is incompletely understood. Previously we proposed that retinogeniculo-cortical oscillations and photoreceptors work in parallel to process vision. Here we propose that motion could also be processed within the retina, and not in the brain as current theory suggests. In this paper, we discuss: 1) internal neural space formation; 2) primary, secondary, and tertiary roles of vision; 3) gamma as the secondary role; and 4) synchronization and coherence. Movement within the external field is instantly detected by primary processing within the space formed by the retina, providing a unified view of the world from an internal point of view. Our new theory begins to answer questions about: 1) perception of space, erect images, and motion, 2) purpose of lateral inhibition, 3) speed of visual perception, and 4) how peripheral color vision occurs without a large population of cones located peripherally in the retina. We explain that strong oscillatory activity influences on brain activity and is necessary for: 1) visual processing, and 2) formation of the internal visuospatial area necessary for visual consciousness, which could allow rods to receive precise visual and visuospatial information, while retinal waves could link the lateral geniculate body with the cortex to form a neural space formed by membrane potential-based oscillations and photoreceptors. We propose that vision is tripartite, with three components that allow a person to make sense of the world, terming them "primary, secondary, and tertiary roles" of vision. Finally, we propose that Gamma waves that are higher in strength and volume allow communication among the retina, thalamus, and various areas of the cortex, and synchronization brings cortical

Testing device for control rod drives

International Nuclear Information System (INIS)

Hayakawa, Toshifumi.

1992-01-01

A testing device for control rod drives comprises a logic measuring means for measuring an output signal from a control rod drive logic generation circuit, a control means for judging the operation state of a control rod and a man machine interface means for outputting the result of the judgement. A driving instruction outputted from the control rod operation device is always monitored by the control means, and if the operation instruction is stopped, a testing signal is outputted to the control rod control device to simulate a control rod operation. In this case, the output signal of the control rod drive logic generation circuit is held in a control rod drive memory means and intaken into a logic analysis means for measurement and an abnormality is judged by the control means. The stopping of the control rod drive instruction is monitored and the operation abnormality of the control rod is judged, to mitigate the burden of an operator. Further, the operation of the control rod drive logic generation circuit can be confirmed even during a nuclear plant operation by holding the control rod drive instruction thereby enabling to improve maintenance efficiency. (N.H.)

Cell Fate of Müller Cells During Photoreceptor Regeneration in an N-Methyl-N-nitrosourea-Induced Retinal Degeneration Model of Zebrafish.

Science.gov (United States)

Ogai, Kazuhiro; Hisano, Suguru; Sugitani, Kayo; Koriyama, Yoshiki; Kato, Satoru

2016-01-01

Zebrafish can regenerate several organs such as the tail fin, heart, central nervous system, and photoreceptors. Very recently, a study has demonstrated the photoreceptor regeneration in the alkylating agent N-methyl-N-nitrosourea (MNU)-induced retinal degeneration (RD) zebrafish model, in which whole photoreceptors are lost within a week after MNU treatment and then regenerated within a month. The research has also shown massive proliferation of Müller cells within a week. To address the question of whether proliferating Müller cells are the source of regenerating photoreceptors, which remains unknown in the MNU-induced zebrafish RD model, we employed a BrdU pulse-chase technique to label the proliferating cells within a week after MNU treatment. As a result of the BrdU pulse-chase technique, a number of BrdU(+) cells were observed in the outer nuclear layer as well as the inner nuclear layer. This implies that regenerating photoreceptors are derived from proliferating Müller cells in the zebrafish MNU-induced RD model.

Failed fuel rod detector

Energy Technology Data Exchange (ETDEWEB)

Uchida, Katsuya; Matsuda, Yasuhiko

1984-05-02

The purpose of the project is to enable failed fuel rod detection simply with no requirement for dismantling the fuel assembly. A gamma-ray detection section is arranged so as to attend on the optional fuel rods in the fuel assembly. The fuel assembly is adapted such that a gamma-ray shielding plate is detachably inserted into optional gaps of the fuel rods or, alternatively, the fuel assembly can detachably be inserted to the gamma-ray shielding plate. In this way, amount of gaseous fission products accumulated in all of the plenum portions in the fuel rods as the object of the measurement can be determined without dismantling the fuel assembly. Accordingly, by comparing the amounts of the gaseous fission products, the failed fuel rod can be detected.

RodPilotR - The Innovative and Cost-Effective Digital Control Rod Drive Control System for PWRs

International Nuclear Information System (INIS)

Baron, Clemens

2008-01-01

With RodPilot, AREVA NP offers an innovative and cost-effective system for controlling control rods in Pressurized Water Reactors. RodPilot controls the three operating coils of the control rod drive mechanism (lift, moveable gripper and stationary gripper coil). The rods are inserted into or withdrawn from the core as required by the Reactor Control System. The system combines modern components, state-of-the-art logic and a proven electronic control rod drive control principle to provide enhanced reliability and lower maintenance costs. (author)

Photoreceptor inner segment ellipsoid band integrity on spectral domain optical coherence tomography

Directory of Open Access Journals (Sweden)

Saxena S

2014-12-01

Full Text Available Sandeep Saxena,1 Khushboo Srivastav,1 Chui M Cheung,2 Joanne YW Ng,3 Timothy YY Lai3 1Retina Service, Department of Ophthalmology, King George’s Medical University Lucknow, India; 2Singapore National Eye Centre, Singapore; 3Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Kowloon, Hong Kong Abstract: Spectral domain optical coherence tomography cross-sectional imaging of the macula has conventionally been resolved into four bands. However, some doubts were raised regarding authentication of the existence of these bands. Recently, a number of studies have suggested that the second band appeared to originate from the inner segment ellipsoids of the foveal cone photoreceptors, and therefore the previously called inner segment-outer segment junction is now referred to as inner segment ellipsoidband. Photoreceptor dysfunction may be a significant predictor of visual acuity in a spectrum of surgical and medical retinal diseases. This review aims to provide an overview and summarizes the role of the photoreceptor inner segment ellipsoid band in the management and prognostication of various vitreoretinal diseases. Keywords: spectral domain optical coherence tomography, inner segment-outer segment junction, external limiting membrane, macular hole, diabetic macular edema, age relate macular degeneration

Integrated control rod monitoring device

International Nuclear Information System (INIS)

Saito, Katsuhiro

1997-01-01

The present invention provides a device in which an entire control rod driving time measuring device and a control rod position support device in a reactor building and a central control chamber are integrated systematically to save hardwares such as a signal input/output device and signal cables between boards. Namely, (1) functions of the entire control rod driving time measuring device for monitoring control rods which control the reactor power and a control rod position indication device are integrated into one identical system. Then, the entire devices can be made compact by the integration of the functions. (2) The functions of the entire control rod driving time measuring device and the control rod position indication device are integrated in a central operation board and a board in the site. Then, the place for the installation of them can be used in common in any of the cases. (3) The functions of the entire control rod driving time measuring device and the control rod position indication device are integrated to one identical system to save hardware to be used. Then, signal input/output devices and drift branching panel boards in the site and the central operation board can be saved, and cables for connecting both of the boards is no more necessary. (I.S.)

Unidirectional photoreceptor-to-Müller glia coupling and unique K+ channel expression in Caiman retina.

Directory of Open Access Journals (Sweden)

Astrid Zayas-Santiago

Full Text Available Müller cells, the principal glial cells of the vertebrate retina, are fundamental for the maintenance and function of neuronal cells. In most vertebrates, including humans, Müller cells abundantly express Kir4.1 inwardly rectifying potassium channels responsible for hyperpolarized membrane potential and for various vital functions such as potassium buffering and glutamate clearance; inter-species differences in Kir4.1 expression were, however, observed. Localization and function of potassium channels in Müller cells from the retina of crocodiles remain, hitherto, unknown.We studied retinae of the Spectacled caiman (Caiman crocodilus fuscus, endowed with both diurnal and nocturnal vision, by (i immunohistochemistry, (ii whole-cell voltage-clamp, and (iii fluorescent dye tracing to investigate K+ channel distribution and glia-to-neuron communications.Immunohistochemistry revealed that caiman Müller cells, similarly to other vertebrates, express vimentin, GFAP, S100β, and glutamine synthetase. In contrast, Kir4.1 channel protein was not found in Müller cells but was localized in photoreceptor cells. Instead, 2P-domain TASK-1 channels were expressed in Müller cells. Electrophysiological properties of enzymatically dissociated Müller cells without photoreceptors and isolated Müller cells with adhering photoreceptors were significantly different. This suggests ion coupling between Müller cells and photoreceptors in the caiman retina. Sulforhodamine-B injected into cones permeated to adhering Müller cells thus revealing a uni-directional dye coupling.Our data indicate that caiman Müller glial cells are unique among vertebrates studied so far by predominantly expressing TASK-1 rather than Kir4.1 K+ channels and by bi-directional ion and uni-directional dye coupling to photoreceptor cells. This coupling may play an important role in specific glia-neuron signaling pathways and in a new type of K+ buffering.

Rod drive and latching mechanism

International Nuclear Information System (INIS)

Veronesi, L.; Sherwood, D.G.

1982-01-01

Hydraulic drive and latching mechanisms for driving reactivity control mechanisms in nuclear reactors are described. Preferably, the pressurized reactor coolant is utilized to raise the drive rod into contact with and to pivot the latching mechanism so as to allow the drive rod to pass the latching mechanism. The pressure in the housing may then be equalized which allows the drive rod to move downwardly into contact with the latching mechanism but to hold the shaft in a raised position with respect to the reactor core. Once again, the reactor coolant pressure may be utilized to raise the drive rod and thus pivot the latching mechanism so that the drive rod passes above the latching mechanism. Again, the mechanism pressure can be equalized which allows the drive rod to fall and pass by the latching mechanism so that the drive rod approaches the reactor core. (author)

Control rods

International Nuclear Information System (INIS)

Koga, Isao; Masuoka, Ryuzo.

1979-01-01

Purpose: To prevent fuel element failures during power conditioning by removing liquid absorbents in poison tubes of control rods in a fast power up step and extracting control rods to slightly increase power in a medium power up step. Constitution: A plurality of poison tubes are disposed in a coaxial or plate-like arrangement and divided into a region capable of compensating the reactivity from the initial state at low temperature to 40% power operation and a region capable of compensating the reactivity in the power up above 40% power operation. Soluble poisons are used as absorbers in the poison tubes corresponding to above 40% power operation and they are adapted to be removed independently from the driving of control rods. The poison tubes filled with the soluble absorbers are responsible for the changes in the reactivity from the initial state at low temperature to the medium power region and the reactivity control is conducted by the elimination of liquid absorbers from the poison tubes. In the succeeding slight power up region above the medium power, power up is proceeding by extracting the control rods having remaining poison tubes filled with solid or liquid absorbers. (Seki, T.)

Simulation of nuclear fuel rods by using process computer-controlled power for indirect electrically heated rods

International Nuclear Information System (INIS)

Malang, S.

1975-11-01

An investigation was carried out to determine how the simulation of nuclear fuel rods with indirect electrically heated rods could be improved by use of a computer to control the electrical power during a loss-of-coolant accident (LOCA). To aid in the experiment, a new version of the HETRAP code was developed which simulates a LOCA with heater rod power controlled by a computer that adjusts rod power during a blowdown to minimize the difference in heat flux of the fuel and heater rods. Results show that without computer control of heater rod power, only the part of a blowdown up to the time when the heat transfer mode changes from nucleate boiling to transition or film boiling can be simulated well and then only for short times. With computer control, the surface heat flux and temperature of an electrically heated rod can be made nearly identical to that of a reactor fuel rod with the same cooling conditions during much of the LOCA. A small process control computer can be used to achieve close simulation of a nuclear fuel rod with an indirect electrically heated rod

Fuel rod technology

International Nuclear Information System (INIS)

Bezold, H.; Romeiser, H.J.

1979-07-01

By extensive mechanization and automation of the fuel rod production, also at increasing production numbers, an efficient production shall be secured, simultaneously corresponding to the high quality standard of the fuel rods. The works done up to now concentrated on the lay out of a rough concept for a mechanized production course. Detail-studies were made for the problems of fuel rod humidity, filling and resistance welding. Further promotion of this project and thus further report will be stopped, since the main point of these works is the production technique. (orig.) [de

Digital control rod blocking monitor

International Nuclear Information System (INIS)

Funayama, Yoshio.

1996-01-01

The present invention system is used for monitoring of a power region of a reactor, and used for monitoring of simultaneous withdrawal of a plurality of control rods without increasing the size or complicating the system. Namely, the system processes signals from a neutron flux detectors at the periphery of control rods controlled for withdrawal. As a result of the processing, the digital monitoring system generates an alarm when the reactor power at the periphery of the control rods fluctuates exceeding an allowable range. In the system, a control rod information forming means prepares frame data comprising front data, positions of the control rods to be withdrawn, frame numbers and completion data. A serial data transmitting means transmits the frame data successively as repeating frame data rows. A control rod information receiving means takes up the frame data of each of control rods to be withdrawn from the transmitted frame data rows. Since the system of the present invention can monitor the withdrawal of a plurality of control rods simultaneously without increasing the size or complicating the system, cost can be saved and the maintenance can be improved. (I.S.)

The xanthopsins : a new family of eubacterial blue-light photoreceptors

NARCIS (Netherlands)

Kort, R; Hoff, W.D.; West, M.E.; Kroon, A R; Hoffer, S.M.; Vlieg, K H; Crielaand, W; van Beeumen, J.; Hellingwerf, K J

1996-01-01

Photoactive yellow protein (PYP) is a photoreceptor that has been isolated from three halophilic phototrophic purple bacteria. The PYP from Ectothiorhodospira halophila BN9626 is the only member for which the sequence has been reported at the DNA level. Here we describe the cloning and sequencing of

The Xanthopsins: a new family of eubacterial blue-light photoreceptors

NARCIS (Netherlands)

Kort, R.; Hoff, W.D.; van West, W.S.; Kroon, A.R.; Hoffer, S.M.; Vlieg, K.H.; Crielaard, W.; van Beeumen, J.J.; Hellingwerf, K.J.

1996-01-01

Photoactive yellow protein (PYP) is a photoreceptor that has been isolated from three halophilic phototrophic purple bacteria. The PYP from Ectothiorhodospira halophila BN9626 is the only member for which the sequence has been reported at the DNA level. Here we describe the cloning and sequencing of

Cubozoan genome illuminates functional diversification of opsins and photoreceptor evolution

Czech Academy of Sciences Publication Activity Database

Liegertová, Michaela; Pergner, Jiří; Kozmiková, Iryna; Fabian, Peter; Pombinho, António R.; Strnad, Hynek; Pačes, Jan; Vlček, Čestmír; Bartůněk, Petr; Kozmik, Zbyněk

2015-01-01

Roč. 5, Jul 8 (2015) ISSN 2045-2322 R&D Projects: GA ČR GAP305/10/2141; GA MŠk LO1220 Institutional support: RVO:68378050 Keywords : Cubozoan genome * opsins * photoreceptor * evolution Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 5.228, year: 2015

No evidence for a genetic blueprint: The case of the "complex" mammalian photoreceptor

Directory of Open Access Journals (Sweden)

G Kumaramanickavel

2015-01-01

Full Text Available Despite the intensity of the search for genes causing inherited retinal degenerations over the past 3 decades, of the approximately 200 disease genes identified to date, all appear to be ordinary housekeeping genes specifying proteins playing basic structural and functional roles in the mature photoreceptor cells. No genes or genetic elements have been identified which can be construed as having a specific morphogenic role, directing the development of the cytoarchitecture of any particular retinal cell. The evidence suggests that the cytoarchitecture of the retinal photoreceptors, although enormously complex, arises from the self-organization of the cells constituents without any regulation or direction from an external genetic blueprint.

The BWR Hybrid 4 control rod

International Nuclear Information System (INIS)

Gross, H.; Fuchs, H.P.; Lippert, H.J.; Dambietz, W.

1988-01-01

The service life of BWR control rods designed in the past has been unsatisfactory. The main reason was irradiation assisted stress corrosion cracking of B 4 C rods caused by external swelling of the B 4 C powder. By this reason KWU developed an improved BWR control rod (Hybrid 4 control rod) with extended service life and increased control rod worth. It also allows the procedure for replacing and rearranging fuel assemblies to be considerably simplified. A complete set of Hydbrid 4 control rods is expected to last throughout the service life of a plant (assumption: ca. 40 years) if an appropriate control rod reshuffling management program is used. (orig.)

Burnable poison rod

International Nuclear Information System (INIS)

Natsume, Tomohiro.

1988-01-01

Purpose: To decrease the effect of water elimination and the effect of burn-up residue boron, thereby reduce the effect of burnable poison rods as the neutron poisons at the final stage of reactor core lifetime. Constitution: In a burnable poison rod according to the present invention, a hollow burnable poison material is filled in an external fuel can, an inner fuel can mounted with a carbon rod is inserted to the hollow portion of the burnable poison material and helium gases are charged in the outer fuel can. In such a burnable poison rod, the reactivity worths after the burning are reduced to one-half as compared with the conventional case. Accordingly, since the effect of the burnable poison as the neutron poisons is reduced at the final stage of the reactor core of lifetime, the excess reactivity of the reactor core is increased. (Horiuchi, T.)

Modeling retinal degeneration using patient-specific induced pluripotent stem cells.

Directory of Open Access Journals (Sweden)

Zi-Bing Jin

Full Text Available Retinitis pigmentosa (RP is the most common inherited human eye disease resulting in night blindness and visual defects. It is well known that the disease is caused by rod photoreceptor degeneration; however, it remains incurable, due to the unavailability of disease-specific human photoreceptor cells for use in mechanistic studies and drug screening. We obtained fibroblast cells from five RP patients with distinct mutations in the RP1, RP9, PRPH2 or RHO gene, and generated patient-specific induced pluripotent stem (iPS cells by ectopic expression of four key reprogramming factors. We differentiated the iPS cells into rod photoreceptor cells, which had been lost in the patients, and found that they exhibited suitable immunocytochemical features and electrophysiological properties. Interestingly, the number of the patient-derived rod cells with distinct mutations decreased in vitro; cells derived from patients with a specific mutation expressed markers for oxidation or endoplasmic reticulum stress, and exhibited different responses to vitamin E than had been observed in clinical trials. Overall, patient-derived rod cells recapitulated the disease phenotype and expressed markers of cellular stresses. Our results demonstrate that the use of patient-derived iPS cells will help to elucidate the pathogenic mechanisms caused by genetic mutations in RP.

Circulating Reactive Oxidant Causes Apoptosis of Retinal Pigment Epithelium and Cone Photoreceptors in the Mouse Central Retina

Directory of Open Access Journals (Sweden)

Wei Wang

2011-01-01

Full Text Available Reactive oxidants damage the retinal pigment epithelium (RPE, which is required for viability of overlying photoreceptors. Smoking which leads to chronic accumulation of reactive oxidants in the circulation is linked to age-related macular degeneration (AMD where RPE death is seen along with photoreceptor loss in the central macular region of the retina. It is unclear why this damage is concentrated in the central retina. We asked whether circulating oxidant might specifically target the central retina. Mice were administered the classic reactive oxidant iodate through tail vein injection, and visual acuity was followed by optokinetic response. Histology and apoptosis was examined by H&E and immunostaining. Iodate indeed selectively damaged the central retina, and this damage was highlighted by early apoptosis of RPE in the central retina followed by apoptosis of photoreceptors adjacent to the region of RPE loss–-cones were lost preferentially. The pattern and extent of this damage was independent of exposure to light. We then conclude that circulating oxidant is sufficient to selectively damage the central retina highlighted by sequential apoptosis of RPE and photoreceptors, with cones being the most sensitivity to this RPE loss.

Biomechanics of lumbar cortical screw-rod fixation versus pedicle screw-rod fixation with and without interbody support.

Science.gov (United States)

Perez-Orribo, Luis; Kalb, Samuel; Reyes, Phillip M; Chang, Steve W; Crawford, Neil R

2013-04-15

Seven different combinations of posterior screw fixation, with or without interbody support, were compared in vitro using nondestructive flexibility tests. To study the biomechanical behavior of a new cortical screw (CS) fixation construct relative to the traditional pedicle screw (PS) construct. The CS is an alternative to the PS for posterior fixation of the lumbar spine. The CS trajectory is more sagittally and cranially oriented than the PS, being anchored in the pars interarticularis. Like PS fixation, CS fixation uses interconnecting rods fastened with top-locking connectors. Stability after bilateral CS fixation was compared with stability after bilateral PS fixation in the setting of intact disc and with direct lateral interbody fixation (DLIF) or transforaminal lateral interbody fixation (TLIF) support. Standard nondestructive flexibility tests were performed in cadaveric lumbar specimens, allowing non-paired comparisons of specific conditions from 28 specimens (4 groups of 7) within a larger experiment of multiple hardware configurations. Condition tested and group from which results originated were as follows: (1) intact (all groups); (2) with L3-L4 bilateral PS-rods (group 1); (3) with bilateral CS-rods (group 2); (4) with DLIF (group 3); (5) with DLIF + CS-rods (group 4); (6) with DLIF + PS-rods (group 3); (7) with TLIF + CS-rods (group 2), and (8) with TLIF + PS-rods (group 2). To assess spinal stability, the mean range of motion, lax zone, and stiff zone at L3-L4 were compared during flexion-extension, lateral bending, and axial rotation. With intact disc, stability was equivalent after PS-rod and CS-rod fixation, except that PS-rod fixation was stiffer during axial rotation. With DLIF support, there was no significant difference in stability between PS-rod and CS-rod fixation. With TLIF support, PS-rod fixation was stiffer than CS-rod fixation during lateral bending. Bilateral CS-rod fixation provided about the same stability in cadaveric specimens

Gene Correction Reverses Ciliopathy and Photoreceptor Loss in iPSC-Derived Retinal Organoids from Retinitis Pigmentosa Patients

Directory of Open Access Journals (Sweden)

Wen-Li Deng

2018-04-01

Full Text Available Summary: Retinitis pigmentosa (RP is an irreversible, inherited retinopathy in which early-onset nyctalopia is observed. Despite the genetic heterogeneity of RP, RPGR mutations are the most common causes of this disease. Here, we generated induced pluripotent stem cells (iPSCs from three RP patients with different frameshift mutations in the RPGR gene, which were then differentiated into retinal pigment epithelium (RPE cells and well-structured retinal organoids possessing electrophysiological properties. We observed significant defects in photoreceptor in terms of morphology, localization, transcriptional profiling, and electrophysiological activity. Furthermore, shorted cilium was found in patient iPSCs, RPE cells, and three-dimensional retinal organoids. CRISPR-Cas9-mediated correction of RPGR mutation rescued photoreceptor structure and electrophysiological property, reversed the observed ciliopathy, and restored gene expression to a level in accordance with that in the control using transcriptome-based analysis. This study recapitulated the pathogenesis of RPGR using patient-specific organoids and achieved targeted gene therapy of RPGR mutations in a dish as proof-of-concept evidence. : Jin and colleagues demonstrate that patient-specific iPSC-derived 3D retinae can recapitulate disease progress of retinitis pigmentosa through presenting defects in photoreceptor morphology, gene profile, and electrophysiology, as well as the defective ciliogenesis in iPSCs, iPSC-RPE, and 3D retinae. CRISPR/Cas9-mediated gene correction can rescue not only photoreceptor structure and electrophysiological property but also observed ciliopathy. Keywords: RPGR, photoreceptor, electrophysiology, retinitis pigmentosa, patient-derived iPSCs, retinal organoid, RPE cells, cilium, ciliopathy, disease modeling

Freely suspended rod fall dampener, especially for control rod of liquid-cooled nuclear reactor

International Nuclear Information System (INIS)

Becvar, J.; Saroch, V.

1977-01-01

A shock absorber is described whose advantage is that the space required for the movement of the shock absorber in the operating travel of the system suspension rod-control rod bundle may be reduced. The design allows the automatic disconnection of the system and the removal of the suspension rod from the reactor without dismantling. The braking force reaction is transmitted to the structure above the core. The system fall energy is absorbed on the side of the suspension rod which has a bigger mass. (J.B.)

RodPilot{sup R} - The Innovative and Cost-Effective Digital Control Rod Drive Control System for PWRs

Energy Technology Data Exchange (ETDEWEB)

Baron, Clemens [AREVA NP GmbH, NLEE-G, Postfach 1199, 91001 Erlangen (Germany)

2008-07-01

With RodPilot, AREVA NP offers an innovative and cost-effective system for controlling control rods in Pressurized Water Reactors. RodPilot controls the three operating coils of the control rod drive mechanism (lift, moveable gripper and stationary gripper coil). The rods are inserted into or withdrawn from the core as required by the Reactor Control System. The system combines modern components, state-of-the-art logic and a proven electronic control rod drive control principle to provide enhanced reliability and lower maintenance costs. (author)

Finding the Genesis for a Thesis

Science.gov (United States)

Caroll, Joyce Armstrong

2013-01-01

This article describes a prewriting heuristics strategy that can help students find the genesis of their thesis. The 3 functions of the heuristic procedure are that it aids in retrieving relevant information stored in the mind; draws attention to important information that can be further researched or accessed; and prepares the mind for the…

Photovoltaic technologies and the genesis project

International Nuclear Information System (INIS)

Kuwano, Y.

1994-01-01

In this paper, the author first presents briefly global environmental problems and energy problems, as well as recent trends in representative types of solar cell. Next, he gives examples of applications for these solar cells, and finally discusses future prospects for solar cells in the form of the GENESIS project. (TEC). 12 figs., 4 refs

Is the photoactive yellow protein a UV-B/blue light photoreceptor?

NARCIS (Netherlands)

Carroll, E. C.; Hospes, M.; Valladares, C.; Hellingwerf, K.J.; Larsen, D.S.

2011-01-01

UV light below 300 nm is shown to generate the first photocycle intermediate in the blue light photoreceptor Photoactive Yellow Protein. Fluorescence and ultrafast transient absorption measurements indicate two excitation pathways: UV-B absorption by the chromophore and Fluorescence Resonant Energy

Does Apoptosis Regulate the Function of Retinal Photoreceptors?

OpenAIRE

Halaby, Reginald

2012-01-01

Apoptosis, or programmed cell death, is an integral component of developmental biology, embryology, and anatomy. All eukaryotic cells possess the molecular machinery necessary to execute apoptosis. However, dysregulated apoptosis in the form of too much or too little cell death results in diseases such as Alzheimer’s disease, autoimmune disorders, and cancer. It is postulated that apoptosis of the photoreceptors in the retina plays a vital role in mediating vision, and evidence is presented h...

Visual cycle modulation in neurovascular retinopathy.

Science.gov (United States)

Akula, James D; Hansen, Ronald M; Tzekov, Radouil; Favazza, Tara L; Vyhovsky, Tanya C; Benador, Ilan Y; Mocko, Julie A; McGee, David; Kubota, Ryo; Fulton, Anne B

2010-08-01

Rats with oxygen-induced retinopathy (OIR) model the pediatric retinal disease retinopathy of prematurity (ROP). Recent findings in OIR rats imply a causal role for the rods in the ROP disease process, although only experimental manipulation of rod function can establish this role conclusively. Accordingly, a visual cycle modulator (VCM) - with no known direct effect on retinal vasculature - was administered to "50/10 model" OIR Sprague-Dawley rats to test the hypotheses that it would 1) alter rod function and 2) consequently alter vascular outcome. Four litters of pups (N=46) were studied. For two weeks, beginning on postnatal day (P) 7, the first and fourth litters were administered 6 mg kg(-1) N-retinylacetamide (the VCM) intraperitoneally; the second and third litters received vehicle (DMSO) alone. Following a longitudinal design, retinal function was assessed by electroretinography (ERG) and the status of the retinal vessels was monitored using computerized fundus photograph analysis. Rod photoreceptor and post-receptor response amplitudes were significantly higher in VCM-treated than in vehicle-treated rats; deactivation of phototransduction was also significantly more rapid. Notably, the arterioles of VCM-treated rats showed significantly greater recovery from OIR. Presuming that the VCM did not directly affect the retinal vessels, a causal role for the neural retina - particularly the rod photoreceptors - in OIR was confirmed. There was no evidence of negative alteration of photoreceptor function consequent to VCM treatment. This finding implicates the rods as a possible therapeutic target in neurovascular diseases such as ROP. Copyright 2010 Elsevier Ltd. All rights reserved.

Decrease of tropical cyclone genesis frequency in the western North Pacific since 1960s

Science.gov (United States)

Hu, Feng; Li, Tim; Liu, Jia; Bi, Mingyu; Peng, Melinda

2018-03-01

Tropical cyclone (TC) genesis frequency in the western North Pacific (WNP) during 1960-2014 shows a step-by-step decrease on interdecadal timescale, in accordance to the phase of the Interdecadal Pacific Oscillation (IPO). The environmental parameters responsible for the interdecadal change of TC genesis frequency were investigated. It was found that vertical wind shear especially the zonal wind shear plays a critical role, while other parameters such as sea surface temperature (SST), vertical velocity, divergence, humidity and maximum potential intensity cannot explain the step-by-step decrease of TC genesis frequency. A further diagnosis shows that the interdecadal change of vertical wind shear is caused by SST and associated rainfall pattern changes across the Indo-Pacific Ocean. A stronger warming in the Indian Ocean/western Pacific from 1960-1976 to 1977-1998 led to enhanced convection over the Maritime Continent and thus strengthened vertical shear over the key TC genesis region in the WNP. A La Nina-like SST pattern change from 1977-1998 to 1999-2014 led to a strengthened Walker circulation in the tropical Pacific, which further enhanced the vertical shear and decreased TC genesis frequency in the WNP.

Control-rod driving mechanism

International Nuclear Information System (INIS)

Jodoi, Takashi.

1976-01-01

Purpose: To prevent falling of control rods due to malfunction. Constitution: The device of the present invention has a scram function in particular, and uses principally a fluid pressure as a scram accelerating means. The control rod is held by upper and lower holding devices, which are connected by a connecting mechanism. This connecting mechanism is designed to be detachable only at the lower limit of driving stroke of the control rod so that there occurs no erroneous scram resulting from careless disconnection of the connecting mechanism. Further, scramming operation due to own weight of the scram operating portion such as control rod driving shaft may be effected to increase freedom. (Kamimura, M.)

REACTOR CONTROL ROD OPERATING SYSTEM

Science.gov (United States)

Miller, G.

1961-12-12

A nuclear reactor control rod mechanism is designed which mechanically moves the control rods into and out of the core under normal conditions but rapidly forces the control rods into the core by catapultic action in the event of an emergency. (AEC)

Excessive Myosin Activity in Mbs Mutants Causes Photoreceptor Movement Out of the Drosophila Eye Disc Epithelium

OpenAIRE

Lee, Arnold; Treisman, Jessica E.

2004-01-01

Neuronal cells must extend a motile growth cone while maintaining the cell body in its original position. In migrating cells, myosin contraction provides the driving force that pulls the rear of the cell toward the leading edge. We have characterized the function of myosin light chain phosphatase, which down-regulates myosin activity, in Drosophila photoreceptor neurons. Mutations in the gene encoding the myosin binding subunit of this enzyme cause photoreceptors to drop out of the eye disc e...

RNA interference gene therapy in dominant retinitis pigmentosa and cone-rod dystrophy mouse models caused by GCAP1 mutations

Directory of Open Access Journals (Sweden)

Li eJiang

2014-04-01

Full Text Available RNA interference (RNAi knockdown is an efficacious therapeutic strategy for silencing genes causative for dominant retinal dystrophies. To test this, we used self-complementary (sc AAV2/8 vector to develop an RNAi-based therapy in two dominant retinal degeneration mouse models. The allele-specific model expresses transgenic bovine GCAP1(Y99C establishing a rapid RP-like phenotype, whereas the nonallele-specific model expresses mouse GCAP1(L151F producing a slowly progressing cone/rod dystrophy (CORD. The late onset GCAP1(L151F-CORD mimics the dystrophy observed in human GCAP1-CORD patients. Subretinal injection of scAAV2/8 carrying shRNA expression cassettes specific for bovine or mouse GCAP1 showed strong expression at one week post-injection. In both allele-specific (GCAP1(Y99C-RP and nonallele-specific (GCAP1(L151F-CORD models of dominant retinal dystrophy, RNAi-mediated gene silencing enhanced photoreceptor survival, delayed onset of degeneration and improved visual function. Such results provide a proof of concept toward effective RNAi-based gene therapy mediated by scAAV2/8 for dominant retinal disease based on GCAP1 mutation. Further, nonallele-specific RNAi knockdown of GCAP1 may prove generally applicable toward the rescue of any human GCAP1-based dominant cone-rod dystrophy.

Turnover of rod photoreceptor outer segments. II. Membrane addition and loss in relationship to light

International Nuclear Information System (INIS)

Besharse, J.C.; Hollyfield, J.G.; Rayborn, M.E.

1977-01-01

The rate of disk addition to rod outer segments (ROS) varies widely in Xenopus laevis tadpoles kept in cyclic light (12L:12D). When measured as radioactive band ( 3 H-band) displacement during the 2nd day after injection of [ 3 H]leucine, 75% of the daily increment of displacement occurred during the first 8 h of light. During the same interval, the number of open disks at the ROS base increased more than threefold. During the last 8 h of darkness, 3 H-band displacement was undetectable and the number of open disks was reduced. These observations suggest the possibility that disk addition may occur discontinuously. During the 3rd and 4th days after injection of [ 3 H]leucine, maximal displacement of the 3 H-band occurred later in the day than on the 2nd day, its movement no longer corresponding to the increase in open disks. This delay in 3 H-band displacement may reflect a time delay as a result of propagation of compressive stress in an elastic ROS system. Maximal disk loss from ROS as reflected in counts of phagosomes in the pigment epithelium occurred within 1 h of light exposure, and phagosome counts remained high for 4 h before declining to a low level in darkness. Modified lighting regimes affected the daily rhythms of shedding and disk addition differently, suggesting that control mechanisms for the two processes are not directly coupled. During 3 days in darkness, disk addition was reduced 50% compared to controls (12L:12D), whereas shedding was reduced by about 40%. Although reduced in level, shedding occurred as a free-running circadian rhythm

Apigenin-7-diglucuronide protects retinas against bright light-induced photoreceptor degeneration through the inhibition of retinal oxidative stress and inflammation.

Science.gov (United States)

Bian, Minjuan; Zhang, Yong; Du, Xiaoye; Xu, Jing; Cui, Jingang; Gu, Jiangping; Zhu, Weiliang; Zhang, Teng; Chen, Yu

2017-05-15

Vision impairment in retinal degenerative diseases such as age-related macular degeneration is primarily associated with photoreceptor degeneration, in which oxidative stress and inflammatory responses are mechanistically involved as central players. Therapies with photoreceptor protective properties remain to be developed. Apigenin-7-diglucuronide (A7DG), a flavonoid glycoside, is present in an assortment of medicinal plants with anti-inflammatory or ant-oxidant activities. However, the pharmacological significance of A7DG remains unknown in vivo. The current study isolated A7DG from Glechoma longituba (Nakai) Kuprian and investigated the retinal protective effect A7DG in mice characterized by bright light-induced photoreceptor degeneration. The results showed that A7DG treatment led to remarkable photoreceptor protection in bright light-exposed BALB/c mice. Moreover, A7DG treatment alleviated photoreceptor apoptosis, mitigated oxidative stress, suppressed reactive gliosis and microglial activation and attenuated the expression of proinflammatory genes in bright light-exposed retinas. The results demonstrated for the first time remarkable photoreceptor protective activities of A7DG in vivo. Inhibition of bright light-induced retinal oxidative stress and retinal inflammatory responses was associated with the retinal protection conferred by A7DG. The work here warrants further evaluation of A7DG as a pharmacological candidate for the treatment of vision-threatening retinal degenerative disorders. Moreover, given the general implication of oxidative stress and inflammation in the pathogenesis of neurodegeneration, A7DG could be further tested for the treatment of other neurodegenerative disorders. Copyright © 2017 Elsevier B.V. All rights reserved.

Control rod velocity limiter

International Nuclear Information System (INIS)

Cearley, J.E.; Carruth, J.C.; Dixon, R.C.; Spencer, S.S.; Zuloaga, J.A. Jr.

1986-01-01

This patent describes a velocity control arrangement for a reciprocable, vertically oriented control rod for use in a nuclear reactor in a fluid medium, the control rod including a drive hub secured to and extending from one end therefrom. The control device comprises: a toroidally shaped control member spaced from and coaxially positioned around the hub and secured thereto by a plurality of spaced radial webs thereby providing an annular passage for fluid intermediate the hub and the toroidal member spaced therefrom in coaxial position. The side of the control member toward the control rod has a smooth generally conical surface. The side of the control member away from the control rod is formed with a concave surface constituting a single annular groove. The device also comprises inner and outer annular vanes radially spaced from one another and spaced from the side of the control member away from the control rod and positioned coaxially around and spaced from the hub and secured thereto by spaced radial webs thereby providing an annular passage for fluid intermediate the hub and the vanes. The vanes are angled toward the control member, the outer edge of the inner vane being closer to the control member and the inner edge of the outer vane being closer to the control member. When the control rod moves in the fluid in the direction toward the drive hub the vanes direct a flow of fluid turbulence which provides greater resistance to movement of the control rod in the direction toward the drive hub than in the other direction

Control rod position control device

International Nuclear Information System (INIS)

Ubukata, Shinji.

1997-01-01

The present invention provides a control rod position control device which stores data such as of position signals and driving control rod instruction before and after occurrence of abnormality in control for the control rod position for controlling reactor power and utilized the data effectively for investigating the cause of abnormality. Namely, a plurality of individual control devices have an operation mismatching detection circuit for outputting signals when difference is caused between a driving instruction given to the control rod position control device and the control rod driving means and signals from a detection means for detecting an actual moving amount. A general control device collectively controls the individual control devices. In addition, there is also disposed a position storing circuit for storing position signals at least before and after the occurrence of the control rod operation mismatching. With such procedures, the cause of the abnormality can be determined based on the position signals before and after the occurrence of control rod mismatching operation stored in the position storing circuit. Accordingly, the abnormality cause can be determined to conduct restoration in an early stage. (I.S.)

Augustine on redemption in Genesis 1�3

Directory of Open Access Journals (Sweden)

Edward L. Smither

2014-01-01

Full Text Available Many theologians, including those concerned with theology of mission, frame the dramaof God�s story and mission (missio Dei through the three major acts of creation, fall andredemption. Others add that the new creation ought to be regarded as a fourth act. Althoughthis framework describes the entire biblical narrative, creation, fall and the hope of redemptionare, of course, quite present in the first three chapters of Genesis. In this article, I endeavouredto engage with the commentaries of the African church father Augustine of Hippo (354�430 CE to grasp his thoughts on redemption in Genesis 1�3. In his Genesis works, Augustinewas primarily concerned with clarifying the doctrine of creation and, relatively speaking, hadfar less to say about redemption. That said, Augustine was, quite interested with Scripture�sstory of redemption in his magnum opus De Civitate Dei [City of God]. Thus, in this article, Iexplored two major questions: firstly, why did Augustine pay so little attention to redemptionin Genesis 1�3? Secondly, how did he articulate and relate redemption in these chapters? Itwas shown that whilst his primary focus was to articulate creation, his thoughts on redemptionwere probably limited some because of the insufficiency of his Old Latin Bible translation andperhaps because of other distractions in ministry. Furthermore, it was argued that Augustine�sdoctrine of redemption was a subset of his discussion on creation � specifically, that the secondAdam (Christ brought new life to God�s image bearers affected by the fall of the first Adam.Intradisciplinary and/or interdisciplinary implications: My aim was to establish Augustine�sthoughts on redemption as a point of dialogue for theologians of mission endeavouring toclarify a theology of mission. As most mission theologians do not consult Augustine in theirwork and as most early Christian scholars do not read Augustine missionally, this studyoffered fresh insights for

Age-related decrease in rod bipolar cell density of the human retina ...

Indian Academy of Sciences (India)

PRAKASH

Retinal bipolar cells situated in the inner nuclear layer are predominantly involved in mediating the transfer of signals from the photoreceptors to the ganglion cells. Their synapses are located both at the level of the outer and inner plexiform layers. In the former, their dendritic processes synapse with photoreceptors in the ...

Control rod selecting and driving device

International Nuclear Information System (INIS)

Isobe, Hideo.

1981-01-01

Purpose: To simultaneously drive a predetermined number of control rods in a predetermined mode by the control of addresses for predetermined number of control rods and read or write of driving codified data to and from the memory by way of a memory controller. Constitution: The system comprises a control rod information selection device for selecting predetermined control rods from a plurality of control rods disposed in a reactor and outputting information for driving them in a predetermined mode, a control rod information output device for codifying the information outputted from the above device and outputting the addresses to the predetermined control rods and driving mode coded data, and a driving device for driving said predetermined control rods in a predetermined mode in accordance with the codified data outputted from the above device, said control rod infromation output device comprising a memory device capable of storing a predetermined number of the codified data and a memory control device for storing the predetermined number of data into the above memory device at a predetermined timing while successively outputting the thus stored predetermined number of data at a predetermined timing. (Seki, T.)

NEUTRONIC REACTOR CONTROL ROD DRIVE APPARATUS

Science.gov (United States)

Oakes, L.C.; Walker, C.S.

1959-12-15

ABS>A suspension mechanism between a vertically movable nuclear reactor control rod and a rod extension, which also provides information for the operator or an automatic control signal, is described. A spring connects the rod extension to a drive shift. The extension of the spring indicates whether (1) the rod is at rest on the reactor, (2) the rod and extension are suspended, or (3) the extension alone is suspended, the spring controlling a 3-position electrical switch.

RODDRP - A FORTRAN program for use in control rod calibration by the rod drop method

International Nuclear Information System (INIS)

Wilson, W.E.

1972-01-01

The different methods to measure reactivity which are applicable to control rod calibration are discussed. They include: 1) the positive period method, 2) the rod drop method, 3) the source-jerk method, 4) the rod oscillation method, and 5) the pulsed neutron method. The instrument setup used at WSU for rod drop measurements is presented. To speed up the analysis of power fall-off trace, a FORTRAN IV program called RODDRP was written to simultaneously solve the in-hour equation and relative neutron flux. The procedure for calculating the worth of the rod that produced the power trace is given. The reactivity for each time relative flux point is obtained. Conclusions about the status of the equipment are made

Safety analysis of a pool Genesis II irradiator

International Nuclear Information System (INIS)

Rodrigues Junior, Ary de A.

2011-01-01

The Genesis II Irradiator manufactured by GRAT * STAR Inc. (USA) is a category III gamma irradiator in which the sealed source is contained in a water filled storage pool and is shielded permanently, i.e. the material has to move down to the source. Even though the pool is 5.6 m deep, what would happen if the water level lowered? There are a series of safety devices that will avoid this situation and calculations show that the water level has to be very low in order to deliver a significant dose; moreover, only in case a person remains at the border of the pool for a long time this would be risky. In conclusion, it is practically impossible for someone to be exposed to radiation from a Genesis II Irradiator source. (author)

Simulation of vibration modes of the fuel rod damaged due to the grid-to-rod fretting wear

International Nuclear Information System (INIS)

Kim, Kyu Tae; Kim, Kyeong Koo; Jang, Young Ki; Lee, Kyou Seok

1997-01-01

The flow-induced fuel fretting wear observed in some PWRs mainly proceeds in the grid-to-rod contact positions. The grid-to-rod fretting wear in the PWR fuel assembly depends on grid-to-rod gap size, its axial profile and flow-induced vibration. This paper describes the GRIDFORCE program which generates the axially dependent grid-to-rod gap size as a function of burnup. The axially dependent grid-to-rod gap profiles are employed to predict the fuel rod vibration mode shapes by the ANSYS code. With the help of the Paidousis empirical formula, this paper also calculates the fuel rod vibration amplitudes under various supporting conditions, which indicates that the increase of the number of unsupported mid-grids will increase the fuel rod vibration amplitude. On the other hand, the comparison of the predicted vibration mode shapes and the observed mid-grid fretting wear pattern indicates that the 1st and 6th vibration mode shapes under the supporting inactive condition at the mid-grids can simulate the observed mid-grid fretting wear profile. This paper also proposes design guidelines against the grid-to-rod fretting wear. (author). 3 refs., 8 figs

Automated nuclear fuel rod pattern loading system

International Nuclear Information System (INIS)

Lambert, D.V.; Nylund, T.W.; Byers, J.W.; Haley, D.E. Jr.; Cioffi, J.V.

1991-01-01

This patent describes a method for loading fuel rods in a desired pattern. It comprises providing a supply of fuel rods of known enrichments; providing a magazine defining a matrix of elongated slots open at their forward ends for receiving fuel rods; defining a fuel rod feed path; receiving successively one at a time along the feed path fuel rods selected from the supply thereof; verifying successively one at a time along the feed path the identity of the selected fuel rods, the verifying including blocking passage of each selected fuel rod along the feed path until the identity of each selected fuel rod is confirmed as correct; feeding to the magazine successively one at a time along the feed path the selective and verified fuel rods; and supporting and moving the magazine along X-Y axes to successively align one at a time selected ones of the slots with the feed path for loading in the magazine the successive fuel rods in a desired enrichment pattern

Slip rate and tremor genesis in Cascadia

Science.gov (United States)

Wech, Aaron G.; Bartlow, Noel M.

2014-01-01

At many plate boundaries, conditions in the transition zone between seismogenic and stable slip produce slow earthquakes. In the Cascadia subduction zone, these events are consistently observed as slow, aseismic slip on the plate interface accompanied by persistent tectonic tremor. However, not all slow slip at other plate boundaries coincides spatially and temporally with tremor, leaving the physics of tremor genesis poorly understood. Here we analyze seismic, geodetic, and strainmeter data in Cascadia to observe for the first time a large, tremor-generating slow earthquake change from tremor-genic to silent and back again. The tremor falls silent at reduced slip speeds when the migrating slip front pauses as it loads the stronger adjacent fault segment to failure. The finding suggests that rheology and slip-speed-regulated stressing rate control tremor genesis, and the same section of fault can slip both with and without detectable tremor, limiting tremor's use as a proxy for slip.

Control rod drives

International Nuclear Information System (INIS)

Ikakura, Hiroaki.

1986-01-01

Purpose: To enable to direct disconnection of control rods upon abnormal temperature rise in the reactor thereby improve the reliability for the disconnecting operation in control rod drives for FBR type reactors upon emergency. Constitution: A diaphragm is disposed to the upper opening of a sealing vessel inserted to the hollow portion of an electromagnet and a rod is secured to the central position of the upper surface. A spring contacts are attached by way of an insulator to the inner surface at the lower portion of an extension pipe and connected with cables for supplying electric power sources respectively to a magnet. If the temperature in the reactor abnormally rises, liquid metals in the sealing vessel are expanded tending to extend the bellows downwardly. However, since they are attracted by the electromagnet, the thermal expansion of the liquid metals exert on the diaphragm prior to the bellows. Thus, the switch between the spring contacts is made open to attain the deenergized state to thereby disconnect the control rod and shutdown the neclear reactor. (Horiuchi, T.)

Method for compacting spent nuclear reactor fuel rods

International Nuclear Information System (INIS)

Wachter, W.J.

1988-01-01

In a nuclear reactor system which requires periodic physical manipulation of spent fuel rods, the method of compacting fuel rods from a fuel rod assembly is described. The method consists of: (1) removing the top end from the fuel rod assembly; (2) passing each of multiple fuel rod pulling elements in sequence through a fuel rod container and thence through respective consolidating passages in a fuel rod directing chamber; (3) engaging one of the pulling elements to the top end of each of the fuel rods; (4) drawing each of the pulling elements axially to draw the respective engaged fuel rods in one axial direction through the respective the passages in the chamber to thereby consolidate the fuel rods into a compacted configuration of a cross-sectional area smaller than the cross-sectional area occupied thereby within the fuel rod assembly; and (5) drawing all of the engaged fuel rods concurrently and substantially parallel to one another in the one axial direction into the fuel rod container while maintaining the compacted configuration whereby the fuel rods are aligned within the container in a fuel rod density of the the fuel rod assembly

Gnaz couples the circadian and dopaminergic system to G protein-mediated signaling in mouse photoreceptors.

Directory of Open Access Journals (Sweden)

Patrick Vancura

Full Text Available The mammalian retina harbors a circadian clockwork that regulates vision and promotes healthiness of retinal neurons, mainly through directing the rhythmic release of the neurohormones dopamine-acting on dopamine D4 receptors-and melatonin-acting on MT1 and MT2 receptors. The gene Gnaz-a unique Gi/o subfamily member-was seen in the present study to be expressed in photoreceptors where its protein product Gαz shows a daily rhythm in its subcellular localization. Apart from subcellular localization, Gnaz displays a daily rhythm in expression-with peak values at night-in preparations of the whole retina, microdissected photoreceptors and photoreceptor-related pinealocytes. In retina, Gnaz rhythmicity was observed to persist under constant darkness and to be abolished in retina deficient for Clock or dopamine D4 receptors. Furthermore, circadian regulation of Gnaz was disturbed in the db/db mouse, a model of diabetic retinopathy. The data of the present study suggest that Gnaz links the circadian clockwork-via dopamine acting on D4 receptors-to G protein-mediated signaling in intact but not diabetic retina.

Control rod drives

International Nuclear Information System (INIS)

Asano, Hiromitsu.

1979-01-01

Purpose: To drive control rods at an optimum safety speed corresponding to the reactor core output. Constitution: The reactor power is detected by a neutron detector and the output signal is applied to a process computer. The process computer issues a signal representing the reactor core output, which is converted through a function generator into a signal representing the safety speed of control rods. The converted signal is further supplied to a V/F converter and converted into a pulse signal. The pulse signal is inputted to a step motor driving circuit, which actuates a step motor to operate the control rods always at a safety speed corresponding to the reactor core power. (Furukawa, Y.)

Individual nuclear fuel rod weighing system

International Nuclear Information System (INIS)

Fogg, J. L.; Howell, C. A.; Smith, J. H.; Vining, G. E.

1985-01-01

An individual nuclear fuel rod weighing system for rods carried on a tray which moves along a materials handling conveyor. At a first tray position on the conveyor, a lifting device raises the rods off the tray and places them on an overhead ramp. A loading mechanism conveys the rods singly from the overhead ramp onto an overhead scale for individual weighing. When the tray is at a second position on the conveyor, a transfer apparatus transports each weighed rod from the scale back onto the tray

Response Function of the Crayfish Caudal Photoreceptor to Hydrodynamic Stimuli

Science.gov (United States)

Breite, Sally; Bahar, Sonya; Neiman, Alexander; Moss, Frank

2002-03-01

In its abdominal 6th ganglion the crayfish houses 2 light-sensitive neurons (caudal photoreceptors, or CPRs). It is known that these neurons work in tandem with a mechanosensory system of tiny hairs spread across the tailfan, which make synaptic contact with the photoreceptors. A stochastic resonance effect has been shown in this system in which light enhances the transduction of a weak, periodic mechanosensory (hydrodynamic) stimulus. It is not known, however, whether an optimal response from the CPR is induced by a single sine wave cycle or some other waveform. We have experimentally investigated this favorable waveform by driving a tailfan preparation with mechanical 10 Hz correlated Ornstein-Uhlenbeck noise and calculating the response function from the spike-triggered average of the applied noise waveform. We will discuss differences in the shape of the optimal waveform under dark and light conditions, as well as what seems to be a noticeable difference in the magnitude of the animals' response to a noisy stimulus in comparison with a periodic stimulus.

Measuring device for control rod driving time

International Nuclear Information System (INIS)

Tanaka, Kazuhiko; Hanabusa, Masatoshi.

1993-01-01

The present invention concerns a measuring device for control driving time having a function capable of measuring a selected control rod driving time and measuring an entire control rod driving time simultaneously. A calculation means and a store means for the selected rod control rod driving time, and a calculation means and a store means for the entire control rod driving time are disposed individually. Each of them measures the driving time and stores the data independent of each other based on a selected control rod insert ion signal and an entire control rod insertion signal. Even if insertion of selected and entire control rods overlaps, each of the control rod driving times can be measured reliably to provide an advantageous effect capable of more accurately conducting safety evaluation for the nuclear reactor based on the result of the measurement. (N.H.)

Is the poleward migration of tropical cyclone maximum intensity associated with a poleward migration of tropical cyclone genesis?

Science.gov (United States)

Daloz, Anne Sophie; Camargo, Suzana J.

2018-01-01

A recent study showed that the global average latitude where tropical cyclones achieve their lifetime-maximum intensity has been migrating poleward at a rate of about one-half degree of latitude per decade over the last 30 years in each hemisphere. However, it does not answer a critical question: is the poleward migration of tropical cyclone lifetime-maximum intensity associated with a poleward migration of tropical cyclone genesis? In this study we will examine this question. First we analyze changes in the environmental variables associated with tropical cyclone genesis, namely entropy deficit, potential intensity, vertical wind shear, vorticity, skin temperature and specific humidity at 500 hPa in reanalysis datasets between 1980 and 2013. Then, a selection of these variables is combined into two tropical cyclone genesis indices that empirically relate tropical cyclone genesis to large-scale variables. We find a shift toward greater (smaller) average potential number of genesis at higher (lower) latitudes over most regions of the Pacific Ocean, which is consistent with a migration of tropical cyclone genesis towards higher latitudes. We then examine the global best track archive and find coherent and significant poleward shifts in mean genesis position over the Pacific Ocean basins.

Growth and Morphology of Rod Eutectics

Energy Technology Data Exchange (ETDEWEB)

Jing Teng; Shan Liu; R. Trivedi

2008-03-17

The formation of rod eutectic microstructure is investigated systematically in a succinonitrile-camphor alloy of eutectic composition by using the directional solidification technique. A new rod eutectic configuration is observed in which the rods form with elliptical cylindrical shape. Two different orientations of the ellipse are observed that differ by a 90{sup o} rotation such that the major and the minor axes are interchanged. Critical experiments in thin samples, where a single layer of rods forms, show that the spacing and orientation of the elliptic rods are governed by the growth rate and the sample thickness. In thicker samples, multi layers of rods form with circular cross-section and the scaling law between the spacing and velocity predicted by the Jackson and Hunt model is validated. A theoretical model is developed for a two-dimensional array of elliptical rods that are arranged in a hexagonal or a square array, and the results are shown to be consistent with the experimental observations. The model of elliptic rods is also shown to reduce to that for the circular rod eutectic when the lengths of the two axes are equal, and to the lamellar eutectic model when one of the axes is much larger than the other one.

Status of rod consolidation, 1988

International Nuclear Information System (INIS)

Bailey, W.J.

1989-01-01

It is estimated that the spent fuel storage pools at some domestic light-water reactors will run out of space before 2003, the year that the US Department of Energy currently predicts it will have a repository available. Of the methods being studied to alleviate the problem, rod consolidation is one of the leading candidates for achieving more efficient use of existing space in spent fuel storage pools. Rod consolidation involves mechanically removing all the fuel rods from the fuel assembly hardware (i.e., the structural components) and placing the fuel rods in a close-packed array in a canister without space grids. A typical goal of rod consolidation systems is to insert the fuel rods from two fuel assemblies into a canister that has the same exterior dimensions as one standard fuel assembly (i.e., to achieve a consolidation or compaction ratio of 2:1) and to compact the nonfuel-bearing structural components from those two fuel assemblies by a factor of 10 to 20. This report provides an overview of the current status of rod consolidation in the United States and a small amount of information on related activities in other countries. 85 refs., 36 figs., 5 tabs

Inspecting method for fuel rods

International Nuclear Information System (INIS)

Watanabe, Masaaki; Kogure, Sumio.

1976-01-01

Purpose: To precisely detect the response of flaw in clad tube and submerged fuel pellets from a relationship between the surface of fuel rod and internal signal. Constitution: Ultrasonic reflected waves from the surface of fuel rods and the interior are detected and either one of fuel rod or ultrasonic flaw detecting contact is rotated to thereby precisely detect the response of the flaw of clad tube and submerged fuel pellets from a relationship between said surface and the interior. It will be noted that the ultrasonic flaw detecting contact used is of the line-focus type, the incident angle of ultrasonic wave from the ultrasonic flaw detecting contact relative to the fuel rod is the angle of skew, that is, the ultrasonic flaw detecting contact is not perpendicular to a center axis of the fuel rod but is slightly displace. That is, the use of the aforesaid contact may facilitate discrimination between the surface flaw of the fuel rod and the response of submergence, and in addition, the employment of the aforesaid incident angle makes it hard to receive reflected waves from the surface of the fuel rod which is great in terms of energy to facilitate discrimination of waves responsive to submergence. (Kawakami, Y.)

Control rod position detection device

International Nuclear Information System (INIS)

Akita, Haruo; Ogiwara, Sakae.

1996-01-01

The device of the present invention is used in a back-up shut down system of an LMFBR type reactor which is easy for maintenance, has high reliability and can recognize the position of control rods accurately. Namely, a permanent magnet is disposed to a control rod extension tube connected to the lower portion of the control rod. The detector guide tube is disposed in the vicinity of the control rod extension tube. A detector having a detection coil is inserted into a detector tube. With such constitution, the control rod can be detected at one position using the following method. (1) the movement of the magnetic field of the permanent magnet is detected by the detection coil. (2) a plurality of grooves are formed on the control rod extension tube, and the movement of the grooves is detected. In addition, the detection coil is inserted into the detector guide tube, and the signals from the detection coil are inputted to a signal processing circuit disposed at the outside of the reactor vessel using an MI cable to enable the maintenance of the detector. Further, if the detector comprises a detection coil and an excitation coil, the position of a dropped control rod can be recognized at a plurality of points. (I.S.)

Rope wind-up type control rod

International Nuclear Information System (INIS)

Tsuji, Teruaki; Watanabe, Shigeru.

1979-01-01

Purpose: To hold a control rod at a certain position even if the sealed cover of the rod drive mechanism should fail. Constitution: A plurality of friction plates, engaging wheels and a threaded shaft are provided to the wind-up drum for winding up a rope which moves the control rod up and down. While the control rod is adapted to drop by its own weight upon insertion, it is adapted to stop at a predetermined position exactly with no shocks by gradually increasing braking force by the sliding friction caused from the friction plates or the like. A ratch mechanism is provided to the upper portion of the control rod so that the top of the ratch piece may automatically engage the guide passage wall of the control rod upon uncontrolled running of the control rod to prevent further uncontrolled running thereof. (Ikeda, J.)

Genesis nature of financial strategy

OpenAIRE

O.V. Pashchenko

2015-01-01

The article is devoted to the study of the origin and history of the interpretation of the genesis of «strategy», «financial strategy», its elements and composite types. Scientists studied different views on the concept of strategy. Several specific positions founders of different approaches to define the strategy of schools operating strategies involved in the formulation and implementation of the concept of «strategy». Evaluation of different schools of strategies that deepened understandin...

ABRAHAM IN GALATIANS AND IN GENESIS1

African Journals Online (AJOL)

In Genesis 12-25, attention is paid to the promise of land and on Abraham as a blessing to ... It is a truism to claim that Abraham serves as an important figure in both the ..... them rest (Jos 21:43), became the ground for hope for the people who.

GENESI-DR: Discovery, Access and on-Demand Processing in Federated Repositories

Science.gov (United States)

Cossu, Roberto; Pacini, Fabrizio; Parrini, Andrea; Santi, Eliana Li; Fusco, Luigi

2010-05-01

GENESI-DR (Ground European Network for Earth Science Interoperations - Digital Repositories) is a European Commission (EC)-funded project, kicked-off early 2008 lead by ESA; partners include Space Agencies (DLR, ASI, CNES), both space and no-space data providers such as ENEA (I), Infoterra (UK), K-SAT (N), NILU (N), JRC (EU) and industry as Elsag Datamat (I), CS (F) and TERRADUE (I). GENESI-DR intends to meet the challenge of facilitating "time to science" from different Earth Science disciplines in discovery, access and use (combining, integrating, processing, …) of historical and recent Earth-related data from space, airborne and in-situ sensors, which are archived in large distributed repositories. In fact, a common dedicated infrastructure such as the GENESI-DR one permits the Earth Science communities to derive objective information and to share knowledge in all environmental sensitive domains over a continuum of time and a variety of geographical scales so addressing urgent challenges such as Global Change. GENESI-DR federates data, information and knowledge for the management of our fragile planet in line with one of the major goals of the many international environmental programmes such as GMES, GEO/GEOSS. As of today, 12 different Digital Repositories hosting more than 60 heterogeneous dataset series are federated in GENESI-DR. Series include satellite data, in situ data, images acquired by airborne sensors, digital elevation models and model outputs. ESA has started providing access to: Category-1 data systematically available on Internet; level 3 data (e.g., GlobCover map, MERIS Global Vegetation Index); ASAR products available in ESA Virtual Archive and related to the Supersites initiatives. In all cases, existing data policies and security constraints are fully respected. GENESI-DR also gives access to Grid and Cloud computing resources allowing authorized users to run a number of different processing services on the available data. The GENESI

The Ste20 kinase misshapen regulates both photoreceptor axon targeting and dorsal closure, acting downstream of distinct signals.

Science.gov (United States)

Su, Y C; Maurel-Zaffran, C; Treisman, J E; Skolnik, E Y

2000-07-01

We have previously shown that the Ste20 kinase encoded by misshapen (msn) functions upstream of the c-Jun N-terminal kinase (JNK) mitogen-activated protein kinase module in Drosophila. msn is required to activate the Drosophila JNK, Basket (Bsk), to promote dorsal closure of the embryo. A mammalian homolog of Msn, Nck interacting kinase, interacts with the SH3 domains of the SH2-SH3 adapter protein Nck. We now show that Msn likewise interacts with Dreadlocks (Dock), the Drosophila homolog of Nck. dock is required for the correct targeting of photoreceptor axons. We have performed a structure-function analysis of Msn in vivo in Drosophila in order to elucidate the mechanism whereby Msn regulates JNK and to determine whether msn, like dock, is required for the correct targeting of photoreceptor axons. We show that Msn requires both a functional kinase and a C-terminal regulatory domain to activate JNK in vivo in Drosophila. A mutation in a PXXP motif on Msn that prevents it from binding to the SH3 domains of Dock does not affect its ability to rescue the dorsal closure defect in msn embryos, suggesting that Dock is not an upstream regulator of msn in dorsal closure. Larvae with only this mutated form of Msn show a marked disruption in photoreceptor axon targeting, implicating an SH3 domain protein in this process; however, an activated form of Msn is not sufficient to rescue the dock mutant phenotype. Mosaic analysis reveals that msn expression is required in photoreceptors in order for their axons to project correctly. The data presented here genetically link msn to two distinct biological events, dorsal closure and photoreceptor axon pathfinding, and thus provide the first evidence that Ste20 kinases of the germinal center kinase family play a role in axonal pathfinding. The ability of Msn to interact with distinct classes of adapter molecules in dorsal closure and photoreceptor axon pathfinding may provide the flexibility that allows it to link to distinct

ELECTROMAGNETIC APPARATUS FOR MOVING A ROD

Science.gov (United States)

Young, J.N.

1958-04-22

An electromagnetic apparatus for moving a rod-like member in small steps in either direction is described. The invention has particular application in the reactor field where the reactor control rods must be moved only a small distance and where the use of mechanical couplings is impractical due to the high- pressure seals required. A neutron-absorbing rod is mounted in a housing with gripping uaits that engage the rod, and coils for magnetizing the gripping units to make them grip, shift, and release the rod are located outside the housing.

Individual nuclear fuel rod weighing system

International Nuclear Information System (INIS)

Fogg, J.L.; Smith, J.H.; Vining, G.E.; Howell, C.A.

1985-01-01

An individual nuclear fuel rod weighing system for rods carried on a tray which moves along a materials handling conveyor is discussed. At a first tray position on the conveyor, a lifting device raises the rods off the tray and places them on an overhead ramp. A loading mechanism conveys the rods singly from the overhead ramp onto an overhead scale for individual weighing. When the tray is at a second position on the conveyor, a transfer apparatus transports each weighed rod from the scale back onto the tray

Control rods in LMFBRs: a physics assessment

International Nuclear Information System (INIS)

McFarlane, H.F.; Collins, P.J.

1982-08-01

This physics assessment is based on roughly 300 control rod worth measurements in ZPPR from 1972 to 1981. All ZPPR assemblies simulated mixed-oxide LMFBRs, representing sizes of 350, 700, and 900 MWe. Control rod worth measurements included single rods, various combinations of rods, and Ta and Eu rods. Additional measurements studied variations in B 4 C enrichment, rod interaction effects, variations in rod geometry, neutron streaming in sodium-filled channels, and axial worth profiles. Analyses were done with design-equivalent methods, using ENDF/B Version IV data. Some computations for the sensitivities to approximations in the methods have been included. Comparisons of these analyses with the experiments have allowed the status of control rod physics in the US to be clearly defined

Variation in LOV Photoreceptor Activation Dynamics Probed by Time Resolved Infrared Spectroscopy

KAUST Repository

Iuliano, James N.; Gil, Agnieszka A.; Laptenok, Sergey P.; Hall, Christopher R.; Tolentino Collado, Jinnette; Lukacs, Andras; Hag Ahmed, Safaa A; Abyad, Jenna; Daryaee, Taraneh; Greetham, Gregory M.; Sazanovich, Igor V.; Illarionov, Boris; Bacher, Adelbert; Fischer, Markus; Towrie, Michael; French, Jarrod B.; Meech, Stephen R.; Tonge, Peter J

2017-01-01

The light, oxygen, voltage (LOV) domain proteins are blue light photoreceptors that utilize a non-covalently bound flavin mononucleotide (FMN) cofactor as the chromophore. The modular nature of these proteins has led to their wide adoption in the emerging fields of optogenetics and optobiology, where the LOV domain has been fused to a variety of output domains leading to novel light-controlled applications. In the present work, we extend our studies of the sub-picosecond to several hundred microsecond transient infrared spectroscopy of the isolated LOV domain AsLOV2 to three full-length photoreceptors in which the LOV domain is fused to an output domain: the LOV-STAS protein, YtvA, the LOV-HTH transcription factor, EL222, and the LOV-histidine kinase, LovK. Despite differences in tertiary structure, the overall pathway leading to cysteine adduct formation from the FMN triplet state is highly conserved, although there are slight variations in rate. However significant differences are observed in the vibrational spectra and kinetics after adduct formation, which are directly linked to the specific output function of the LOV domain. While the rate of adduct formation varies by only 3.6-fold amongst the proteins, the subsequent large-scale structural changes in the full-length LOV photoreceptors occur over the micro- to sub-millisecond timescales and vary by orders of magnitude depending on the different output function of each LOV domain.

Variation in LOV Photoreceptor Activation Dynamics Probed by Time Resolved Infrared Spectroscopy

KAUST Repository

Iuliano, James N.

2017-12-14

The light, oxygen, voltage (LOV) domain proteins are blue light photoreceptors that utilize a non-covalently bound flavin mononucleotide (FMN) cofactor as the chromophore. The modular nature of these proteins has led to their wide adoption in the emerging fields of optogenetics and optobiology, where the LOV domain has been fused to a variety of output domains leading to novel light-controlled applications. In the present work, we extend our studies of the sub-picosecond to several hundred microsecond transient infrared spectroscopy of the isolated LOV domain AsLOV2 to three full-length photoreceptors in which the LOV domain is fused to an output domain: the LOV-STAS protein, YtvA, the LOV-HTH transcription factor, EL222, and the LOV-histidine kinase, LovK. Despite differences in tertiary structure, the overall pathway leading to cysteine adduct formation from the FMN triplet state is highly conserved, although there are slight variations in rate. However significant differences are observed in the vibrational spectra and kinetics after adduct formation, which are directly linked to the specific output function of the LOV domain. While the rate of adduct formation varies by only 3.6-fold amongst the proteins, the subsequent large-scale structural changes in the full-length LOV photoreceptors occur over the micro- to sub-millisecond timescales and vary by orders of magnitude depending on the different output function of each LOV domain.

Measurements of Burnout Conditions for Flow of Boiling Water in Vertical 3-Rod and 7-Rod Clusters

Energy Technology Data Exchange (ETDEWEB)

Becker, Kurt M; Hernborg, G; Flinta, J E

1964-08-15

The present report deals with measurements of burnout conditions for flow of boiling water in vertical 3-rod and 7-rod clusters. Data were obtained,in respect of heating the rods only, as well as for simultaneous uniform and non-uniform heating of the rods and the shroud. Totally, 520 runs were performed. In the case of equal heat fluxes on all surfaces of the channels, burnout always occurred on the rods, and the data were low by a factor of about 1.3 compared with round duct data. When only the rods were heated, the data showed very low burnout values in comparison with the results for total uniform heating and round ducts. This disagreement was explained by considering the climbing film flow model and the fact that only a fraction of the channel perimeter was heated. For simultaneous and non-uniform heating of the rods and the shroud it was found that the shroud could be overloaded up to 50 per cent without reducing the margin of safety in respect of burnout for the rod cluster. Finally, a correlation for predicting burnout conditions in round ducts, annuli and rod clusters has been presented. This correlation predicts the burnout heat fluxes for the present measurements and previously obtained annuli measurements within {+-} 5 per cent.

Tropical cyclone genesis in the Southern Hemisphere and its relationship with the ENSO

Energy Technology Data Exchange (ETDEWEB)

Kuleshov, Y.; Qi, L. [Australian Bureau of Meteorology, Melbourne, VIC (Australia). National Climate Centre; Chane Ming, F.; Chouaibou, I.; Hoareau, C. [UMR CNRS-Meteo-France-Univ. de la Reunion, La Reunion (France). Lab. de l' Atmosphere et des Cyclones; Roux, F. [Paul Sabatier Univ., CNRS, Toulouse (France). Lab. d' Aerologie

2009-07-01

Tropical cyclogenesis climatology over the South Indian and South Pacific Oceans has been developed using a new tropical cyclone (TC) archive for the Southern Hemisphere, and changes in geographical distribution of areas favourable for TC genesis related to changes in the El Nino-Southern Oscillation (ENSO) phases have been investigated. To explain these changes, large-scale environmental variables which influence TC genesis and development such as sea surface temperatures (SSTs), relative humidity in mid-troposphere, vertical wind shear and lower tropospheric vorticity have been examined. In the South Indian Ocean, reduction of TC genesis in the western part of the basin and its increase in the eastern part as well as displacement of the area favourable for TC genesis further away from the equator during La Nina events compared to El Nino events can be explained by changes in geographical distribution of relative humidity and vorticity across the basin as primary contributors; positive anomalies of SSTs observed during La Nina seasons in the eastern part of the basin additionally contribute to enhanced cyclogenesis near the Western Australia. In the South Pacific Ocean, changes in geographical distribution of relative humidity and vorticity appear to be the key large-scale environmental factors responsible for enhanced TC genesis in the eastern (western) part of the basin as well as for the northeast (southwest) shift of points of cyclogenesis during El Nino (La Nina) events, with vertical wind shear and SSTs as additional contributing large-scale environmental variables. (orig.)

Development of a control rod drive

International Nuclear Information System (INIS)

1991-01-01

In the period under review, the computer codes required for transients calculation have been completed, as well as the programs for modelling and testing the hot-gas temperature control by means of combined core rod and reflector rod operation. The specification of requirements to be fulfilled by the rod drive computer and the neutron flux measuring system has been done relying essentially on the data obtained by the transients calculations performed and the resulting informations on operating conditions. The work for optimization of the core rod drive with regard to rod driving speeds and the 'three-point switch' with hysteresis for controlled, automatic core rod operation has been concentrating on the case of specified, normal operation of the reactor. (orig./DG) [de

75 FR 69458 - Notice of Availability of the Record of Decision for the Genesis Solar Energy Project and...

Science.gov (United States)

2010-11-12

..., LVRWB09B2520] Notice of Availability of the Record of Decision for the Genesis Solar Energy Project [email protected] . SUPPLEMENTARY INFORMATION: Genesis Solar, LLC, a wholly owned subsidiary of NextEra Energy Resources, filed right-of-way (ROW) application CACA-048880 for the proposed Genesis Solar Energy...

Self-Assembly of Rod-Coil Block Copolymers

National Research Council Canada - National Science Library

Jenekhe, S

1999-01-01

... the self-assembly of new rod-coil diblock, rod- coil-rod triblock, and coil-rod-coil triblock copolymers from solution and the resulting discrete and periodic mesostmctares with sizes in the 100...

Ejected control rod and rods drop measurements during Mochovce startup physical tests

International Nuclear Information System (INIS)

Minarcin, Miroslav; Elko, Marek

1998-01-01

Paper deals with measurements of asymmetric reactivity insertion into the reactor core that were carried out during physical startup tests of Mochovce Unit 1 in June 1998. Control rods worth measurements with one and two rods s tucked in upper limit and worth measurement of one control rod from group 6 'ejected' from the reactor core are discussed. During the experiments neutron flux was measured by four ionisation chambers (three of them were placed symmetrically around the reactor core). Results of measurements and influence of asymmetric reactivity influence on ionisation chambers response are presented in the paper. (Authors)

Quivers For Special Fuel Rods-Disposal Of Special Fuel Rods In CASTOR V Casks

International Nuclear Information System (INIS)

Bannani, Amin; Cebula, Wojciech; Buchmuller, Olga; Huggenberg, Roland; Helmut Kuhl

2015-01-01

While GNS casks of the CASTOR family are a suitable means to transfer fuel assemblies (FA) from the NPP to an interim dry storage site, Germanys phase-out of nuclear energy has triggered the demand for an additional solution to dispose of special fuel rods (SFR), normally remaining in the fuel pond until the final shutdown of the NPP. SFR are fuel rods that had to be removed from fuel assemblies mainly due to their special condition, e. g. damages in the cladding of the fuel rods which may have occurred during reactor operations. SFR are usually stored in the spent fuel pond after they are removed from the FA. The quiver for special fuel rods features a robust yet simple design, with a high mechanical stability, a reliable leak-tightness and large safety margins for future requirements on safety analysis. The quiver for special fuel rods can be easily adapted to a large variety of different damaged fuel rods and tailored to the specific need of the customer. The quiver for special fuel rods is adaptable e.g. in length and diameter for use in other types of transport and storage casks and is applicable in other countries as well. The overall concept presented here is a first of its kind solution for the disposal of SFRs via Castor V-casks. This provides an important precondition in achieving the status 'free from nuclear fuel' of the shut down German NPPs

Quivers For Special Fuel Rods-Disposal Of Special Fuel Rods In CASTOR V Casks

Energy Technology Data Exchange (ETDEWEB)

Bannani, Amin; Cebula, Wojciech; Buchmuller, Olga; Huggenberg, Roland [GNS, Essen (Germany); Helmut Kuhl [WTI, Julich (Germany)

2015-05-15

While GNS casks of the CASTOR family are a suitable means to transfer fuel assemblies (FA) from the NPP to an interim dry storage site, Germanys phase-out of nuclear energy has triggered the demand for an additional solution to dispose of special fuel rods (SFR), normally remaining in the fuel pond until the final shutdown of the NPP. SFR are fuel rods that had to be removed from fuel assemblies mainly due to their special condition, e. g. damages in the cladding of the fuel rods which may have occurred during reactor operations. SFR are usually stored in the spent fuel pond after they are removed from the FA. The quiver for special fuel rods features a robust yet simple design, with a high mechanical stability, a reliable leak-tightness and large safety margins for future requirements on safety analysis. The quiver for special fuel rods can be easily adapted to a large variety of different damaged fuel rods and tailored to the specific need of the customer. The quiver for special fuel rods is adaptable e.g. in length and diameter for use in other types of transport and storage casks and is applicable in other countries as well. The overall concept presented here is a first of its kind solution for the disposal of SFRs via Castor V-casks. This provides an important precondition in achieving the status 'free from nuclear fuel' of the shut down German NPPs.

Monitoring device for withdrawing control rods

International Nuclear Information System (INIS)

Higashigawa, Yuichi.

1985-01-01

Purpose: To improve the sensitivity and the responsivity to an equivalent extent to those in the case where local power range monitors are densely arranged near each of the control rods, with no actual but pseudo increase of the number of local power range monitors. Constitution: The monitor arrangement is patterned by utilizing the symmetricity of the reactor core and stored in a monitor designating device. The symmetricity of control rods to be selected and withdrawn by an operator is judged by a control rod symmetry monitoring device, while the symmetricity of the withdrawn control rods is judged by a control rod withdrawal state monitoring device. Then, only when both of the devices judge the symmetricity, the control rods are subjected to gang driving by the control rod drive mechanisms. In this way, monitoring at a high sensitivity and responsivity is enabled with no increase for the number of monitors. (Yoshino, Y.)

Nondestructive assay of HTGR fuel rods

International Nuclear Information System (INIS)

Menlove, H.O.

1974-01-01

Performance characteristics of three different radioactive source NDA systems are compared for the assay of HTGR fuel rods and stacks of rods. These systems include the fast neutron Sb-Be assay system, the 252 Cf ''Shuffler,'' and the thermal neutron PAPAS assay system. Studies have been made to determinethe perturbation on the measurements from particle size, kernel Th/U ratio, thorium content, and hydrogen content. In addition to the total 235 U determination, the pellet-to-pellet or rod-to-rod uniformity of HTGR fuel rod stacks has been measured by counting the delayed gamma rays with a NaI through-hole in the PAPAS system. These measurements showed that rod substitutions can be detected easily in a fuel stack, and that detailed information is available on the loading variations in a uniform stack. Using a 1.0 mg 252 Cf source, assay rates of 2 to 4 rods/s are possible, thus facilitating measurement of 100 percent of a plant's throughput. (U.S.)

The genetics of some planthormones and photoreceptors in Arabidopsis thaliana (L.) Heynh.

NARCIS (Netherlands)

Koornneef, M.

1982-01-01

This thesis describes the isolation and characterization in Arabidopsis thaliana (L.) Heynh. of induced mutants, deficient for gibberellins (GA's), abscisic acid (ABA) and photoreceptors.

These compounds are known to regulate various facets of plant growth and

Inspection system for Zircaloy clad fuel rods

International Nuclear Information System (INIS)

Yancey, M.E.; Porter, E.H.; Hansen, H.R.

1975-10-01

A description is presented of the design, development, and performance of a remote scanning system for nondestructive examination of fuel rods. Characteristics that are examined include microcracking of fuel rod cladding, fuel-cladding interaction, cladding thickness, fuel rod diameter variation, and fuel rod bowing. Microcracking of both the inner and outer fuel rod surfaces and variations in wall thickness are detected by using a pulsed eddy current technique developed by Argonne National Laboratory (ANL). Fuel rod diameter variation and fuel rod bowing are detected by using two linear variable differential transformers (LVDTs) and a signal conditioning system. The system's mechanical features include variable scanning speeds, a precision indexing system, and a servomechanism to maintain proper probe alignment. Initial results indicate that the system is a very useful mechanism for characterizing irradiated fuel rods

Seismic scrammability of HTTR control rods

International Nuclear Information System (INIS)

Nishiguchi, I.; Iyoku, T.; Ito, N.; Watanabe, Y.; Araki, T.; Katagiri, S.

1990-01-01

Scrammability tests on HTTR (High-Temperature Engineering Test Reactor) control rods under seismic conditions have been carried out and seismic conditions influences on scram time as well as functional integrity were examined. A control rod drive located in a stand-pipe at the top of a reactor vessel, raises and lowers a pair of control rods by suspension cables. Each flexible control rod consists of 10 neutron absorber sections held together by a metal spine passing through the center. It falls into a hole in graphite blocks due to gravity at scram. In the tests, a full scale control rod drive and a pair of control rods were employed with a column of graphite blocks in which holes for rods were formed. Blocks misalignment and contact with the hole surface during earthquakes were considered as major causes of disturbance in scram time. Therefore, the following parameters were set up in the tests: excitation direction, combination or horizontal and vertical excitation, acceleration, frequency and block to block gaps. Main results obtained from tests are as follow. 1) Every scram time obtained under the design conditions was within 6 seconds. On the contrary, the scram times were 5.2 seconds when there were no vibration. Therefore, it was concluded that the seismic effects on scram time were not significant. 2) Scram time became longer with increase in both acceleration and horizontal excitation frequency, and control rods fell very smoothly without any jerkiness. This suggests that collision between control rods and hole surface is the main disturbing factor of falling motion. 3) Mechanical and functional integrity of control rod drive mechanism, control rods and graphite blocks was confirmed after 140 seismic scrammability tests. (author). 10 figs, 1 tab

The Neural Retina in Retinopathy of Prematurity

Science.gov (United States)

Hansen, Ronald M.; Moskowitz, Anne; Akula, James D.; Fulton, Anne B.

2016-01-01

Retinopathy of prematurity (ROP) is a neurovascular disease that affects prematurely born infants and is known to have significant long term effects on vision. We conducted the studies described herein not only to learn more about vision but also about the pathogenesis of ROP. The coincidence of ROP onset and rapid developmental elongation of the rod photoreceptor outer segments motivated us to consider the role of the rods in this disease. We used noninvasive electroretinographic (ERG), psychophysical, and retinal imaging procedures to study the function and structure of the neurosensory retina. Rod photoreceptor and post-receptor responses are significantly altered years after the preterm days during which ROP is an active disease. The alterations include persistent rod dysfunction, and evidence of compensatory remodeling of the post-receptor retina is found in ERG responses to full-field stimuli and in psychophysical thresholds that probe small retinal regions. In the central retina, both Mild and Severe ROP delay maturation of parafoveal scotopic thresholds and are associated with attenuation of cone mediated multifocal ERG responses, significant thickening of post-receptor retinal laminae, and dysmorphic cone photoreceptors. These results have implications for vision and control of eye growth and refractive development and suggest future research directions. These results also lead to a proposal for noninvasive management using light that may add to the currently invasive therapeutic armamentarium against ROP. PMID:27671171

Control rod testing apparatus

International Nuclear Information System (INIS)

Gaunt, R.R.; Ashman, C.M.

1987-01-01

A control rod testing apparatus is described comprising: a first guide means having a vertical cylindrical opening for grossly guiding a control rod; a second guide means having a vertical cylindrical opening for grossly guiding a control rod. The first and second guide means are supported at axially spaced locations with the openings coaxial; and a substantially cylindrical subassembly having a vertical cylindrical opening therethrough. The subassembly is trapped coaxial with and between the first and second guide means, and the subassembly radially floats with respect to the first and second guide means

Restoration of outer segments of foveal photoreceptors after resolution of central serous chorioretinopathy.

Science.gov (United States)

Ojima, Yumiko; Tsujikawa, Akitaka; Yamashiro, Kenji; Ooto, Sotaro; Tamura, Hiroshi; Yoshimura, Nagahisa

2010-01-01

To study morphologically and functionally the prognosis of damaged outer segments of the foveal photoreceptor layer in eyes with resolved central serous chorioretinopathy (CSC). We studied retrospectively the medical records of 70 patients (74 eyes) with resolved CSC. Optical coherence tomography was used to detect the junctions between inner and outer segments of the photoreceptors (IS/OS) as a hallmark of the integrity of the outer photoreceptor layer. In 53 eyes (71.6%), the IS/OS line was clearly detected beneath the fovea immediately after resolution of the retinal detachment, with good visual acuity (VA). In the remaining 21 eyes (28.4%), however, the foveal IS/OS line could not be detected shortly after resolution of CSC, and VA was variable, ranging from 0.1 to 1.5 (median, 0.9). Of these 21 eyes, 15 had a follow-up examination with OCT, and in four the foveal IS/OS line was not detected at the follow-up and vision in these eyes remained poor. However, nine eyes showed recovery of the foveal IS/OS line during follow-up, and these eyes had substantial visual recovery. Immediately after resolution of active CSC, although the IS/OS line cannot be detected beneath the fovea, it often shows restoration over time, with visual recovery, though in some eyes no restoration takes place and the prognosis remains poor.

Development of the automatic control rod operation system for JOYO. Verification of automatic control rod operation guide system

International Nuclear Information System (INIS)

Terakado, Tsuguo; Suzuki, Shinya; Kawai, Masashi; Aoki, Hiroshi; Ohkubo, Toshiyuki

1999-10-01

The automatic control rod operation system was developed to control the JOYO reactor power automatically in all operation modes(critical approach, cooling system heat up, power ascent, power descent), development began in 1989. Prior to applying the system, verification tests of the automatic control rod operation guide system was conducted during 32nd duty cycles of JOYO' from Dec. 1997 to Feb. 1998. The automatic control rod operation guide system consists of the control rod operation guide function and the plant operation guide function. The control rod operation guide function provides information on control rod movement and position, while the plant operation guide function provide guidance for plant operations corresponding to reactor power changes(power ascent or power descent). Control rod insertion or withdrawing are predicted by fuzzy algorithms. (J.P.N.)

Control rod drive shaft latch

International Nuclear Information System (INIS)

Thorp, A.G. II.

1976-01-01

A latch mechanism is operated by differential pressure on a piston to engage the drive shaft for a control rod in a nuclear reactor, thereby preventing the control rod from being ejected from the reactor in case of failure of the control rod drive mechanism housing which is subjected to the internal pressure in the reactor vessel. 6 claims, 4 drawing figures

Measurements of Burnout Conditions for Flow of Boiling Water in Vertical 3-Rod and 7-Rod Clusters

International Nuclear Information System (INIS)

Becker, Kurt M.; Hernborg, G.; Flinta, J.E.

1964-08-01

The present report deals with measurements of burnout conditions for flow of boiling water in vertical 3-rod and 7-rod clusters. Data were obtained,in respect of heating the rods only, as well as for simultaneous uniform and non-uniform heating of the rods and the shroud. Totally, 520 runs were performed. In the case of equal heat fluxes on all surfaces of the channels, burnout always occurred on the rods, and the data were low by a factor of about 1.3 compared with round duct data. When only the rods were heated, the data showed very low burnout values in comparison with the results for total uniform heating and round ducts. This disagreement was explained by considering the climbing film flow model and the fact that only a fraction of the channel perimeter was heated. For simultaneous and non-uniform heating of the rods and the shroud it was found that the shroud could be overloaded up to 50 per cent without reducing the margin of safety in respect of burnout for the rod cluster. Finally, a correlation for predicting burnout conditions in round ducts, annuli and rod clusters has been presented. This correlation predicts the burnout heat fluxes for the present measurements and previously obtained annuli measurements within ± 5 per cent

Cellulose Acetate Replica Cleaning Study of Genesis Non-Flight Sample 3CZ00327

Science.gov (United States)

Kuhlman, K. R.; Schmeling, M.; Gonzalez, C. P.; Allton, J. H.; Burnett, D. S.

2014-01-01

The Genesis mission collected solar wind and brought it back to Earth in order to provide precise knowledge of solar isotopic and elemental compositions. The ions in the solar wind were stopped in the collectors at depths on the order of 10 to a few hundred nanometers. This shallow implantation layer is critical for scientific analysis of the composition of the solar wind and must be preserved throughout sample handling, cleaning, processing, distribution, preparation and analysis. We are working interactively with the community of scientists analyzing Genesis samples, using our unique laboratory facilities -- and, where needed, our unique cleaning techniques -- to significantly enhance the science return from the Genesis mission. This work is motivated by the need to understand the submicron contamination on the collectors in the Genesis payload as recovered from the crash site in the Utah desert, and -- perhaps more importantly -- how to remove it. That is, we are evaluating the effectiveness of the wet-chemical "cleaning" steps used by various investigators, to enable them to design improved methods of stripping terrestrial contamination from surfaces while still leaving the solar-wind signal intact.

Reconstitutable control rod spider assembly

International Nuclear Information System (INIS)

Shallenberger, J.M.; Ferian, S.J.

1990-01-01

A reconstitutable control rod/spider assembly includes a hollow connecting finger of the spider having a pair of opposing flat segments formed on the interior thereof and engaging a pair of opposing flat sectors formed on the exterior of a stem extending form the upper end of control rod. The stem also has an externally-threaded portion engaging a nut and a pilot aligning portion for the nut. The nut has a radially flexible and expandable thread-defining element captured in its bore. The segments and sectors allow the rod to be removed and reattached after turning through 180 0 to allow more even wear on the rod. (author)

Estimation of irradiated control rod worth

International Nuclear Information System (INIS)

Varvayanni, M.; Catsaros, N.; Antonopoulos-Domis, M.

2009-01-01

When depleted control rods are planned to be used in new core configurations, their worth has to be accurately predicted in order to deduce key design and safety parameters such as the available shutdown margin. In this work a methodology is suggested for the derivation of the distributed absorbing capacity of a depleted rod, useful in the case that the level of detail that is known about the irradiation history of the control rod does not allow an accurate calculation of the absorber's burnup. The suggested methodology is based on measurements of the rod's worth carried out in the former core configuration and on corresponding calculations based on the original (before first irradiation) absorber concentration. The methodology is formulated for the general case of the multi-group theory; it is successfully tested for the one-group approximation, for a depleted control rod of the Greek Research Reactor, containing five neutron absorbers. The computations reproduce satisfactorily the irradiated rod worth measurements, practically eliminating the discrepancy of the total rod worth, compared to the computations based on the nominal absorber densities.

Rod cluster having improved vane configuration

International Nuclear Information System (INIS)

Shockling, L.A.; Francis, T.A.

1989-01-01

This patent describes a pressurized water reactor vessel, the vessel defining a predetermined axial direction of the flow of coolant therewithin and having plural spider assemblies supporting, for vertical movement within the vessel, respective clusters of rods in spaced, parallel axial relationship, parallel to the predetermined axial direction of coolant flow, and a rod guide for each spider assembly and respective cluster of rods. The rod guide having horizontally oriented support plates therewithin, each plate having an interior opening for accommodating axial movement therethrough of the spider assembly and respective cluster of rods. The opening defining plural radially extending channels and corresponding parallel interior wall surfaces of the support plate

Sucker rod motor

Energy Technology Data Exchange (ETDEWEB)

Radzalov, N N; Radzhabov, N A

1983-01-01

The motor consists of rollers mounted on the wellmouth and connected by a flexible rink. Reciprocating mechanism is in the form of a horizontal non-mobile single-side operation cylinder, inside which a plunger and rod are mounted. The working housing of the hydrocylinder is connected to a gas-hydr aulic batter, and when running is connected via plunger to the high pressure source; running in reverse it is connected with a safety valve and automatic control unit. The unit is equipped with a reducer and a mechanical transformer consisting of screw and nut, and which is shutoff with a single-side lining. The plunger rod consists of an auger-like unit. The high pressure source is provided by the injection line of the sucker rod that has been equipped with a reverse valve.

Duke Power Company's control rod wear program

International Nuclear Information System (INIS)

Culp, D.C.; Kitlan, M.S. Jr.

1990-01-01

Recent examinations performed at several foreign and domestic pressurized water reactors have identified significant control rod cladding wear, leading to the conclusion that previously believed control rod lifetimes are not attainable. To monitor control rod performance and reduce safety concerns associated with wear, Duke Power Company has developed a comprehensive control rod wear program for Ag-In-Cd and boron carbide (B 4 C) rods at the McGuire and Catawba nuclear stations. Duke Power currently uses the Westinghouse 17 x 17 Ag-In-Cd control rod design at McGuire Unit 1 and the Westinghouse 17 x 17 hybrid B 4 C control rod design with a Ag-In-Cd tip at McGuire Unit 2 and Catawba Units 1 and 2. The designs are similar, with the exception of the absorber material and clad thickness. There are 53 control rods per unit

Fuel rod pellet loading head

International Nuclear Information System (INIS)

Howell, T.E.

1975-01-01

An assembly for loading nuclear fuel pellets into a fuel rod comprising a loading head for feeding pellets into the open end of the rod is described. The pellets rest in a perforated substantially V-shaped seat through which air may be drawn for removal of chips and dust. The rod is held in place in an adjustable notched locator which permits alignment with the pellets

Tropical cyclone genesis in the Southern Hemisphere and its relationship with the ENSO

Directory of Open Access Journals (Sweden)

Y. Kuleshov

2009-06-01

Full Text Available Tropical cyclogenesis climatology over the South Indian and South Pacific Oceans has been developed using a new tropical cyclone (TC archive for the Southern Hemisphere, and changes in geographical distribution of areas favourable for TC genesis related to changes in the El Niño-Southern Oscillation (ENSO phases have been investigated. To explain these changes, large-scale environmental variables which influence TC genesis and development such as sea surface temperatures (SSTs, relative humidity in mid-troposphere, vertical wind shear and lower tropospheric vorticity have been examined. In the South Indian Ocean, reduction of TC genesis in the western part of the basin and its increase in the eastern part as well as displacement of the area favourable for TC genesis further away from the equator during La Niña events compared to El Niño events can be explained by changes in geographical distribution of relative humidity and vorticity across the basin as primary contributors; positive anomalies of SSTs observed during La Niña seasons in the eastern part of the basin additionally contribute to enhanced cyclogenesis near the Western Australia. In the South Pacific Ocean, changes in geographical distribution of relative humidity and vorticity appear to be the key large-scale environmental factors responsible for enhanced TC genesis in the eastern (western part of the basin as well as for the northeast (southwest shift of points of cyclogenesis during El Niño (La Niña events, with vertical wind shear and SSTs as additional contributing large-scale environmental variables.

Rod displacement measurements by x-ray CT and its impact on thermal-hydraulics in tight-lattice rod bundle (Joint research)

International Nuclear Information System (INIS)

Mitsutake, Toru; Misawa, Takeharu; Kureta, Masatoshi; Akimoto, Hajime

2005-06-01

In tight-lattice simulated rod bundles with about 1 mm gap between rods, a rod displacement might affect thermal-hydraulic characteristics since the displacement has a strong impact on the flow area change along the heated section. It should be important to estimate how large the rod position displacement could quantitatively affect critical power for the tight-lattice rod bundle from the point of improvement of prediction capability of subchannel analysis. In the present study, the inside-structure observation of the simulated seven-rod bundle of Reduced Moderation Water Reactor (RMWR) was made through the whole length of the test assembly. Based on the measured rod position data, the relation between the rod position displacement and the heat transfer characteristics was investigated experimentally and through the two kinds of subchannel analysis, the nominal rod position case and the measured rod position case, the effect on the predicted critical power was estimated. The high-energy X-ray computer tomograph (CT) of Fuels Monitoring Facilities (FMF) at the O-arai Engineering Center in Japan Nuclear Cycle Institute (JNC) was applied for the inside-structure observation of the test assembly. The CT view of the cross sections within the test assembly assured the hexagonal rod position arrangement was almost the same as expected by design. The measured data with the X-ray CT facility showed that all rod displacements were small, 0.5 millimeters at maximum and 0.2 millimeters in average. In the heat transfer experiments for the seven-rod bundle, the boiling transition (BT) position and the rod surface temperature behavior was measured. All thermocouples on the center rod downstream from the BT-onset axial height showed almost simultaneous temperature increase due to BT. And the thermocouples located on the same axial heights showed quite similar time-variation behaviors in the vapor cooling heat transfer regime. These results demonstrated the effect of the

Dynamic rod worth measurements (''Rod Insertion''). Final report for the period 01 December 1994 - 30 November 1996

International Nuclear Information System (INIS)

Bogdan, G.

1996-12-01

Reload startup physics tests are performed for pressurized water reactors (PWR power plant) following a refuelling or other significant core alteration for which nuclear design calculations are required. Part of the reload startup physics tests are control rod group worths measurements. for this purpose a new so-called method ''Rod-Insertion'' was developed. It can also be used as an additional measuring instrument on the research reactor for education purposes. The principle of the rod-insertion method is to start from a critical reactor operating at low power and to measure the time-dependent reactivity change while a control rod is inserted into the core. Unlike in the rod-drop method, the measured control rod is inserted with the drive mechanism at normal speed. By analyzing the flux trace using point-kinetics, not only the total rod worth but also the differential and the integral rod worth curves are obtained. A high-quality electrometer is required for monitoring the neutron flux. The analysis is performed by transferring the data to an IBM PC compatible with some additional standard electronic board and the associated software. The new reactivity meter has been validated on the TRIGA Mark II reactors in Ljubljana and Vienna and at the Krsko Nuclear Power Plant during physics startup tests after reload. The results proved the high performance of the reactivity meter in the standard applications according to the existing procedures, as well as in the new rod-insertion technique of measuring the control rod group worths. This method drastically differs from others such as absence of any chemical control of reactivity (like boron exchange method), and minimizing a testing time and waste coolant production

Applications of hydrogen deuterium exchange (HDX for the characterization of conformational dynamics in light-activated photoreceptors

Directory of Open Access Journals (Sweden)

Robert eLindner

2015-06-01

Full Text Available Rational design of optogenetic tools is inherently linked to the understanding of photoreceptor function. Structural analysis of elements involved in signal integration in individual sensor domains provides an initial idea of their mode of operation, but understanding how local structural rearrangements eventually affect signal transmission to output domains requires inclusion of the effector regions in the characterization. However, the dynamic nature of these assemblies renders their structural analysis challenging and therefore a combination of high- and low-resolution techniques is required to appreciate functional aspects of photoreceptors.This review focuses on the potential of Hydrogen-Deuterium exchange coupled to mass spectrometry (HDX-MS for complementing the structural characterization of photoreceptors. In this respect, the ability of HDX-MS to provide information on the conformational dynamics and the possibility to address multiple functionally relevant states in solution render this methodology ideally suitable. We highlight recent examples demonstrating the potential of HDX-MS and discuss how these results can help to improve existing optogenetic systems or guide the design of novel optogenetic tools.

Genesis Eco Systems, Inc. soil washing process

International Nuclear Information System (INIS)

Cena, R.J.

1994-01-01

The Genesis soil washing system is an integrated system of modular design allowing for maximum material handling capabilities, with optimized use of space for site mobility. The Surfactant Activated Bio-enhanced Remediation Equipment-Generation 1 (SABRE-1, Patent Applied For) modification was developed specifically for removing petroleum byproducts from contaminated soils. Scientifically formulated surfactants, introduced by high pressure spray nozzles, displace the contaminant from the surface of the soil particles into the process solution. Once the contaminant is dispersed into the liquid fraction of the process, it is either mechanically removed, chemically oxidized, or biologically oxidized. The contaminated process water is pumped through the Genesis Biosep (Patent Applied For) filtration system where the fines portion is flocculated, and the contaminant-rich liquid portion is combined with an activated mixture of nutrients and carefully selected bacteria to decompose the hydrocarbon fraction. The treated soil and dewatered fines are transferred to a bermed stockpile where bioremediation continues during drying. The process water is reclaimed, filtered, and recycled within the system

Control rod ejection analysis during a depressurization accident and the development of a rod-ejection-preventing device

International Nuclear Information System (INIS)

Mitake, S.; Itoh, K.; Fukushima, H.; Inoue, T.

1982-01-01

The control rods used for the experimental VHTR are suspended in the core by means of flexible steel cables and it is conceivable that an accidental rod ejection could occur due to a depressurization accident. The computer code AFLADE was developed in order to analyze the possibility of accidental rod ejection, and several studies were performed. The parametric study results showed that the adopted design condition for the VHTR core will not cause a rod ejection accident. In parallel with these accident analyses, a rod-ejection-preventing device was developed in preparation for a hypothetical accident, and its function was verified by the component tests

Avian cone photoreceptors tile the retina as five independent, self-organizing mosaics.

Directory of Open Access Journals (Sweden)

Yoseph A Kram

2010-02-01

Full Text Available The avian retina possesses one of the most sophisticated cone photoreceptor systems among vertebrates. Birds have five types of cones including four single cones, which support tetrachromatic color vision and a double cone, which is thought to mediate achromatic motion perception. Despite this richness, very little is known about the spatial organization of avian cones and its adaptive significance. Here we show that the five cone types of the chicken independently tile the retina as highly ordered mosaics with a characteristic spacing between cones of the same type. Measures of topological order indicate that double cones are more highly ordered than single cones, possibly reflecting their posited role in motion detection. Although cones show spacing interactions that are cell type-specific, all cone types use the same density-dependent yardstick to measure intercone distance. We propose a simple developmental model that can account for these observations. We also show that a single parameter, the global regularity index, defines the regularity of all five cone mosaics. Lastly, we demonstrate similar cone distributions in three additional avian species, suggesting that these patterning principles are universal among birds. Since regular photoreceptor spacing is critical for uniform sampling of visual space, the cone mosaics of the avian retina represent an elegant example of the emergence of adaptive global patterning secondary to simple local interactions between individual photoreceptors. Our results indicate that the evolutionary pressures that gave rise to the avian retina's various adaptations for enhanced color discrimination also acted to fine-tune its spatial sampling of color and luminance.

Light- and GTP-activated hydrolysis of phosphatidylinositol bisphosphate in squid photoreceptor membranes

International Nuclear Information System (INIS)

Baer, K.M.; Saibil, H.R.

1988-01-01

Light stimulates the hydrolysis of exogenous, [ 3 H]inositol-labeled phosphatidylinositol bisphosphate (PtdInsP2) added to squid photoreceptor membranes, releasing inositol trisphosphate (InsP3). At free calcium levels of 0.05 microM or greater, hydrolysis of the labeled lipid is stimulated up to 4-fold by GTP and light together, but not separately. This activity is the biochemical counterpart of observations on intact retina showing that a rhodopsin-activated GTP-binding protein is involved in visual transduction in invertebrates, and that InsP3 release is correlated with visual excitation and adaptation. Using an in vitro assay, we investigated the calcium and GTP dependence of the phospholipase activity. At calcium concentrations between 0.1 and 0.5 microM, some hydrolysis occurs independently of GTP and light, with a light- and GTP-activated component superimposed. At 1 microM calcium there is no background activity, and hydrolysis absolutely requires both GTP and light. Ion exchange chromatography on Dowex 1 (formate form) of the water-soluble products released at 1 microM calcium reveals that the product is almost entirely InsP3. Invertebrate rhodopsin is homologous in sequence and function to vertebrate visual pigment, which modulates the concentration of cyclic GMP through the mediation of the GTP-binding protein transducin. While there is some evidence that light also modulates PtdInsP2 content in vertebrate photoreceptors, the case for its involvement in phototransduction is stronger for the invertebrate systems. The results reported here support the scheme of rhodopsin----GTP-binding protein----phospholipase C activation in invertebrate photoreceptors

Investigation of control rod worth and nuclear end of life of BWR control rods

International Nuclear Information System (INIS)

Magnusson, Per

2008-01-01

This work has investigated the Control Rod Worth (CRW) and Nuclear End of Life (NEOL) values for BWR control rods. A study of how different parameters affect NEOL was performed with the transport code PHOENIX4. It was found that NEOL, expressed in terms of 10 B depletion, can be generalized beyond the conditions for which the rod is depleted, such as different power densities and void fractions, the corresponding variation in the NEOL will be about 0.2-0.4% 10 B. It was also found that NEOL results for different fuel types and different fuel enrichments have a variation of about 2-3% in 10 B depletion. A comparative study on NHOL and CRW was made between PHOENIX4 and the stochastic Monte Carlo code MCNP. It was found that there is a significant difference, both due to differences in the codes and to limitations in the geometrical modeling in PHOENIX4. Since MCNP is considered more physically correct, a methodology was developed to calculate the nuclear end of life of BWR control rods with MCNP. The advantages of the methodology are that it does not require other codes to perform the depletion of the absorber material, it can describe control rods of any design and it can deplete the control rod absorber material without burning the fuel. The disadvantage of the method is that is it time-consuming

Preliminary nuclear design for test MOX Fuel rods

Energy Technology Data Exchange (ETDEWEB)

Joo, Hyung Kook; Kim, Taek Kyum; Jeong, Hyung Guk; Noh, Jae Man; Cho, Jin Young; Kim, Young Il; Kim, Young Jin; Sohn, Dong Seong

1997-10-01

As a part of activity for future fuel development project, test MOX fuel rods are going to be loaded and irradiated in Halden reactor core as a KAERI`s joint international program with Paul Scherrer Institute (PSI). PSI will fabricate test MOX rods with attrition mill device which was developed by KAERI. The test fuel assembly rig contains three MOX rods and three inert matrix rods. One of three MOX rods will be fabricated by BNFL, the other two MOX fuel rods will be manufacturing jointly by KAERI and PSI. Three inert matrix fuel rods will be fabricated with Zr-Y-Er-Pu oxide. Neutronic evaluation was preliminarily performed for test fuel assembly suggested by PSI. The power distribution of test fuel rod in test fuel assembly was analyzed for various fuel rods position in assembly and the depletion characteristic curve for test fuel was also determined. The fuel rods position in test fuel assembly does not effect the rod power distribution, and the proposal for test fuel rods suggested by PSI is proved to be feasible. (author). 2 refs., 13 tabs., 16 figs.

Single-phase convective heat transfer in rod bundles

International Nuclear Information System (INIS)

Holloway, Mary V.; Beasley, Donald E.; Conner, Michael E.

2008-01-01

The convective heat transfer for turbulent flow through rod bundles representative of nuclear fuel rods used in pressurized water reactors is examined. The rod bundles consist of a square array of parallel rods that are held on a constant pitch by support grids spaced axially along the rod bundle. Split-vane pair support grids, which create swirling flow in the rod bundle, as well as disc and standard support grids are investigated. Single-phase convective heat transfer coefficients are measured for flow downstream of support grids in a rod bundle. The rods are heated using direct resistance heating, and a bulk axial flow of air is used to cool the rods in the rod bundle. Air is used as the working fluid instead of water to reduce the power required to heat the rod bundle. Results indicate heat transfer enhancement for up to 10 hydraulic diameters downstream of the support grids. A general correlation is developed to predict the heat transfer development downstream of support grids. In addition, circumferential variations in heat transfer coefficients result in hot streaks that develop on the rods downstream of split-vane pair support grids

Single-phase convective heat transfer in rod bundles

Energy Technology Data Exchange (ETDEWEB)

Holloway, Mary V. [Mechanical Engineering Department, United States Naval Academy, 590 Holloway Rd., Annapolis, MD 21402 (United States)], E-mail: holloway@usna.edu; Beasley, Donald E. [Mechanical Engineering Department, Clemson University, Clemson, SC 29634 (United States); Conner, Michael E. [Westinghouse Nuclear Fuel, 5801 Bluff Road, Columbia, SC 29250 (United States)

2008-04-15

The convective heat transfer for turbulent flow through rod bundles representative of nuclear fuel rods used in pressurized water reactors is examined. The rod bundles consist of a square array of parallel rods that are held on a constant pitch by support grids spaced axially along the rod bundle. Split-vane pair support grids, which create swirling flow in the rod bundle, as well as disc and standard support grids are investigated. Single-phase convective heat transfer coefficients are measured for flow downstream of support grids in a rod bundle. The rods are heated using direct resistance heating, and a bulk axial flow of air is used to cool the rods in the rod bundle. Air is used as the working fluid instead of water to reduce the power required to heat the rod bundle. Results indicate heat transfer enhancement for up to 10 hydraulic diameters downstream of the support grids. A general correlation is developed to predict the heat transfer development downstream of support grids. In addition, circumferential variations in heat transfer coefficients result in hot streaks that develop on the rods downstream of split-vane pair support grids.

Fabrication of internally instrumented reactor fuel rods

International Nuclear Information System (INIS)

Schmutz, J.D.; Meservey, R.H.

1975-01-01

Procedures are outlined for fabricating internally instrumented reactor fuel rods while maintaining the original quality assurance level of the rods. Instrumented fuel rods described contain fuel centerline thermocouples, ultrasonic thermometers, and pressure tubes for internal rod gas pressure measurements. Descriptions of the thermocouples and ultrasonic thermometers are also contained

Vortex Noise from Rotating Cylindrical Rods

Science.gov (United States)

Stowell, E Z; Deming, A F

1935-01-01

A series of round rods of the some diameter were rotated individually about the mid-point of each rod. Vortices are shed from the rods when in motion, giving rise to the emission of sound. With the rotating system placed in the open air, the distribution of sound in space, the acoustical power output, and the spectral distribution have been studied. The frequency of emission of vortices from any point on the rod is given by the formula von Karman. From the spectrum estimates are made of the distribution of acoustical power along the rod, the amount of air concerned in sound production, the "equivalent size" of the vortices, and the acoustical energy content for each vortex.

Random Photon Absorption Model Elucidates How Early Gain Control in Fly Photoreceptors Arises from Quantal Sampling

Science.gov (United States)

Song, Zhuoyi; Zhou, Yu; Juusola, Mikko

2016-01-01

Many diurnal photoreceptors encode vast real-world light changes effectively, but how this performance originates from photon sampling is unclear. A 4-module biophysically-realistic fly photoreceptor model, in which information capture is limited by the number of its sampling units (microvilli) and their photon-hit recovery time (refractoriness), can accurately simulate real recordings and their information content. However, sublinear summation in quantum bump production (quantum-gain-nonlinearity) may also cause adaptation by reducing the bump/photon gain when multiple photons hit the same microvillus simultaneously. Here, we use a Random Photon Absorption Model (RandPAM), which is the 1st module of the 4-module fly photoreceptor model, to quantify the contribution of quantum-gain-nonlinearity in light adaptation. We show how quantum-gain-nonlinearity already results from photon sampling alone. In the extreme case, when two or more simultaneous photon-hits reduce to a single sublinear value, quantum-gain-nonlinearity is preset before the phototransduction reactions adapt the quantum bump waveform. However, the contribution of quantum-gain-nonlinearity in light adaptation depends upon the likelihood of multi-photon-hits, which is strictly determined by the number of microvilli and light intensity. Specifically, its contribution to light-adaptation is marginal (≤ 1%) in fly photoreceptors with many thousands of microvilli, because the probability of simultaneous multi-photon-hits on any one microvillus is low even during daylight conditions. However, in cells with fewer sampling units, the impact of quantum-gain-nonlinearity increases with brightening light. PMID:27445779

Vibrational characteristics and wear of fuel rods

International Nuclear Information System (INIS)

Schmugar, K.L.

1977-01-01

Fuel rod wear, due to vibration, is a continuing concern in the design of liquid-cooled reactors. In my report, the methodology and models that are used to predict fuel rod vibrational response and vibratory wear, in a light water reactor environment, are discussed. This methodology is being followed at present in the design of Westinghouse Nuclear Fuel. Fuel rod vibrations are expressed as the normal bending modes, and sources of rod vibration are examined with special emphasis on flow-induced mechanisms in the stable flow region. In a typical Westinghouse PWR fuel assembly design, each fuel rod is supported at multiple locations along the rod axis by a square-shaped 'grid cell'. For a fuel rod /grid support system, the development of small oscillatory motions, due to fluid flow at the rod/grid interface, results in material wear. A theoretical wear mode is developed using the Archard Theory of Adhesive Wear as the basis. Without question certainty, fretting wear becomes a serious problem if it progresses to the stage where the fuel cladding is penetrated and fuel is exposed to the coolant. Westinghouse fuel is designed to minimize fretting wear by limiting the relative motion between the fuel rod and its supports. The wear producing motion between the fuel rod and its supports occurs when the vibration amplitude exceeds the slippage threshold amplitude

Drive-in device for long thin rods into narrow cavitations, especially for control-shutdown rods e.g. of nuclear reactors

International Nuclear Information System (INIS)

Flessner, H.; Paeserack, U.

1974-01-01

The auxiliary device serves as holder for long and thin rods, e.g. control rods, transported hanging in bundles, when these are lowered into narrow cavities. It is constructed as a rod grab vertically movable at the end of a guide tube. A comb-shaped trap in connection with a guide rod serves for lateral support of the lower ends of the rods hanging on the grab. This guide rod can be moved in vertical direction by means of two pairs of convex rollers resting on the inner guide tube. In addition, the guide rod has a prolongation carrying a traverse by means of an abutment on the lower end. With these auxiliaries amongst others, the trap can be brought into a horizontal position by turning around an axis with the control rods meshing with the teeth of the trap while the parallelism of the rods is kept up during transport. (DG) [de

The synthetic progestin norgestrel modulates Nrf2 signaling and acts as an antioxidant in a model of retinal degeneration

Directory of Open Access Journals (Sweden)

Ashleigh M. Byrne

2016-12-01

Full Text Available Retinitis pigmentosa (RP is one of the most common retinal degenerative conditions affecting people worldwide, and is currently incurable. It is characterized by the progressive loss of photoreceptors, in which the death of rod cells leads to the secondary death of cone cells; the cause of eventual blindness. As rod cells die, retinal-oxygen metabolism becomes perturbed, leading to increased levels of reactive oxygen species (ROS and thus oxidative stress; a key factor in the secondary death of cones. In this study, norgestrel, an FDA-approved synthetic analog of progesterone, was found to be a powerful neuroprotective antioxidant, preventing light-induced ROS in photoreceptor cells, and subsequent cell death. Norgestrel also prevented light-induced photoreceptor morphological changes that were associated with ROS production, and that are characteristic of RP. Further investigation showed that norgestrel acts via post-translational modulation of the major antioxidant transcription factor Nrf2; bringing about its phosphorylation, subsequent nuclear translocation, and increased levels of its effector protein superoxide dismutase 2 (SOD2. In summary, these results demonstrate significant protection of photoreceptor cells from oxidative stress, and underscore the potential of norgestrel as a therapeutic option for RP.

RBX2 maintains final retinal cell position in a DAB1-dependent and -independent fashion.

Science.gov (United States)

Fairchild, Corinne L; Hino, Keiko; Han, Jisoo S; Miltner, Adam M; Peinado Allina, Gabriel; Brown, Caileigh E; Burns, Marie E; La Torre, Anna; Simó, Sergi

2018-02-02

The laminated structure of the retina is fundamental for the organization of the synaptic circuitry that translates light input into patterns of action potentials. However, the molecular mechanisms underlying cell migration and layering of the retina are poorly understood. Here, we show that RBX2, a core component of the E3 ubiquitin ligase CRL5, is essential for retinal layering and function. RBX2 regulates the final cell position of rod bipolar cells, cone photoreceptors and Muller glia. Our data indicate that sustained RELN/DAB1 signaling, triggered by depletion of RBX2 or SOCS7 - a CRL5 substrate adaptor known to recruit DAB1 - causes rod bipolar cell misposition. Moreover, whereas SOCS7 also controls Muller glia cell lamination, it is not responsible for cone photoreceptor positioning, suggesting that RBX2, most likely through CRL5 activity, controls other signaling pathways required for proper cone localization. Furthermore, RBX2 depletion reduces the number of ribbon synapses and disrupts cone photoreceptor function. Together, these results uncover RBX2 as a crucial molecular regulator of retina morphogenesis and cone photoreceptor function. © 2018. Published by The Company of Biologists Ltd.

Control rod excess withdrawal prevention device

International Nuclear Information System (INIS)

Takayama, Yoshihito.

1992-01-01

Excess withdrawal of a control rod of a BWR type reactor is prevented. That is, the device comprises (1) a speed detector for detecting the driving speed of a control rod, (2) a judging circuit for outputting an abnormal signal if the driving speed is greater than a predetermined level and (3) a direction control valve compulsory closing circuit for controlling the driving direction of inserting and withdrawing a control rod based on an abnormal signal. With such a constitution, when the with drawing speed of a control rod is greater than a predetermined level, it is detected by the speed detector and the judging circuit. Then, all of the direction control valve are closed by way of the direction control valve compulsory closing circuit. As a result, the operation of the control rod is stopped compulsorily and the withdrawing speed of the control rod can be lowered to a speed corresponding to that upon gravitational withdrawal. Accordingly, excess withdrawal can be prevented. (I.S)

Automated detection of photoreceptor disruption in mild diabetic retinopathy on volumetric optical coherence tomography.

Science.gov (United States)

Wang, Zhuo; Camino, Acner; Zhang, Miao; Wang, Jie; Hwang, Thomas S; Wilson, David J; Huang, David; Li, Dengwang; Jia, Yali

2017-12-01

Diabetic retinopathy is a pathology where microvascular circulation abnormalities ultimately result in photoreceptor disruption and, consequently, permanent loss of vision. Here, we developed a method that automatically detects photoreceptor disruption in mild diabetic retinopathy by mapping ellipsoid zone reflectance abnormalities from en face optical coherence tomography images. The algorithm uses a fuzzy c-means scheme with a redefined membership function to assign a defect severity level on each pixel and generate a probability map of defect category affiliation. A novel scheme of unsupervised clustering optimization allows accurate detection of the affected area. The achieved accuracy, sensitivity and specificity were about 90% on a population of thirteen diseased subjects. This method shows potential for accurate and fast detection of early biomarkers in diabetic retinopathy evolution.

Hollow rods for the oil producing industry

Energy Technology Data Exchange (ETDEWEB)

Khalimova, L M; Elyasheva, M A

1970-01-01

Hollow sucker rods have several advantages over conventional ones. The hollow rods actuate the well pump and at the same time conduct produced fluids to surface. When paraffin deposition occurs, it can be minimized by injecting steam, hot oil or hot water into the hollow rod. Other chemicals, such as demulsifiers, scale inhibitors, corrosion inhibitors, etc., can also be placed in the well through the hollow rods. This reduces cost of preventive treatments, reduces number of workovers, increases oil production, and reduces cost of oil. Because the internal area of the rod is small, the passing liquids have a high velocity and thereby carry sand and dirt out of the well. This reduces pump wear between the piston and the plunger. Specifications of hollow rods, their operating characteristics, and results obtained with such rods under various circumstances are described.

Salamander blue-sensitive cones lost during metamorphosis.

NARCIS (Netherlands)

Chen, Y.; Znoiko, S.; Grip, W.J. de; Crouch, R.K.; Ma, J.X.

2008-01-01

The tiger salamander lives in shallow water with bright light in the aquatic phase, and in dim tunnels or caves in the terrestrial phase. In the aquatic phase, there are five types of photoreceptors--two types of rods and three types of cones. Our previous studies showed that the green rods and

ELECTRIC FIELD MEASUREMENT IN ROD-DISCONTINUED ...

African Journals Online (AJOL)

2014-06-30

Jun 30, 2014 ... the electrogeometrical model using a laboratory experimental rod-plane air gap arrangement with a lightning conductor (Franklin rod or horizontal conductor). The stepped leader could be represented by the rod electrode under a negative lightning impulse voltage having a level leading to breakdown with ...

Method of inspecting control rod drive mechanism

International Nuclear Information System (INIS)

Sato, Tomomi; Tatemichi, Shin-ichiro; Hasegawa, Hidenobu.

1988-01-01

Purpose: To conduct inspection for control rod drives and fuel handling operations in parallel without taking out the entire fuel, while maintaining the reactor in a subcritical state. Method: Control rod drives are inspected through the release of connection between control rods and control rod drives, detachment and dismantling of control rod drives, etc. In this case, structural materials having neutron absorbing power equal to or greater than the control rods are inserted into the gap after taking out fuels. Since the structural materials have neutron absorbing portion, subcriticality is maintained by the neutron absorbing effect. Accordingly, there is no requirement for taking out all of the fuels, thereby enabling to check the control rod drives and conduct handling for the fuels in parallel. As a result, the number of days required for the inspection can be shortened and it is possible to improve the working efficiency for the decomposition, inspection, etc. of the control rod drives and, thus, improve the operation efficiency of the nuclear power plant thereby attaining the predetermined purpose. (Kawakami, Y.)

Control rod

International Nuclear Information System (INIS)

Takeda, Toshikazu; Inoue, Kotaro.

1979-01-01

Purpose: To flatten the power distribution in the reactor core without impairing neutron economy by disposing pins containing elements of lower atomic number in the central region of a shroud and loading pins containing depleted uranium in the periphery region thereof. Constitution: The shroud has a layer of pins containing depleted uranium in the peripheral region and a layer of pins containing elements of lower atomic number such as beryllium in the central region. Heat removal from those pins containing depleted uranium and elements of lower atomic number (neutron moderator) is effected by sodium flow outside of the cladding material. The control rod operation is conducted by inserting or extracting the central portion (pins containing elements of lower atomic number such as beryllium) inside of the stainless pipe. Upon extraction of the control rod, the moderator in the central region is removed whereby high speed neutrons are no more deccelerated and the absorption rate to the depleted uranium is decreased. This can flatten the power distribution in the reactore core with the disposition of a plurality of control rods at a better neutron economy as compared with the use of neutron absorber such as boron. (Seki, T.)

Control rod

International Nuclear Information System (INIS)

Fukumoto, Takashi; Hirakawa, Hiromasa; Kawashima, Norio; Goto, Yasuyuki.

1994-01-01

Neutron absorbers are contained in a tubular member comprising, integrally a tubular portion and four corners disposed at the outer circumference of the tubular portion at every 90deg, to provide a neutron absorbing tube. A plurality of neutron absorbing tubes are arranged in parallel in the lateral direction, and adjacent corners are joined, into a blade to constitute a control rod. Such a control rod has a great structural strength, simple in the structure and relatively light in weight and can contain a great amount of neutron absorbers. Upon formation of the control rod by arranging the blades in a cross-like shape, at least a portion thereof is constituted with short neutron absorbing tubes shorter than the entire length of the blade, and gaps are formed at positions in adjacent in the axial direction. With such a constitution, there is no worry that a wing end of the blade collides against or be abraded with a fuel channel box or a fuel support. Even if fuel channels are vibrated upon scram of the reactor, such as occurrence of earthquakes, it can be inserted to the reactor easily. (N.H.)

Experimental determination of temperature fields in sodium-cooled rod bundles with hexagonal rod arrangement and grid spacers

International Nuclear Information System (INIS)

Moeller, R.; Tschoeke, H.; Kolodziej, M.

1977-01-01

Three-dimensional temperature fields in the claddings of sodium cooled rods were determined experimentally under representative nominal operating conditions for a SNR typical 19-rod bundle model provided with spark-eroded spacers. These experiments are required to verify thermohydraulic computer programs which will provide the output data for strength calculations of the high loaded cladding tubes. In this work the essentials are reported of the measured circumferential distributions of wall temperatures of peripheral rods. In addition the sub-channel temperatures measured over the bundle cross section are indicated, they are required to sustain codes for the global thermohydraulic design of core elements. The most important results are: 1) The whole fuel element is located within the thermal entrance length. 2) High azimuthal temperature differences were measured in the claddings of peripheral rods, which are strongly influenced by the distance between the rod and the shroud, especially for the corner rod. 3) With decreasing Pe-number ( [de

Control rod housing alignment

International Nuclear Information System (INIS)

Dixon, R.C.; Deaver, G.A.; Punches, J.R.; Singleton, G.E.; Erbes, J.G.; Offer, H.P.

1990-01-01

This patent describes a process for measuring the vertical alignment between a hole in a core plate and the top of a corresponding control rod drive housing within a boiling water reactor. It comprises: providing an alignment apparatus. The alignment apparatus including a lower end for fitting to the top of the control rod drive housing; an upper end for fitting to the aperture in the core plate, and a leveling means attached to the alignment apparatus to read out the difference in angularity with respect to gravity, and alignment pin registering means for registering to the alignment pin on the core plate; lowering the alignment device on a depending support through a lattice position in the top guide through the hole in the core plate down into registered contact with the top of the control rod drive housing; registering the upper end to the sides of the hole in the core plate; registering the alignment pin registering means to an alignment pin on the core plate to impart to the alignment device the required angularity; and reading out the angle of the control rod drive housing with respect to the hole in the core plate through the leveling devices whereby the angularity of the top of the control rod drive housing with respect to the hole in the core plate can be determined

The Third ATLAS ROD Workshop

CERN Multimedia

Poggioli, L.

A new-style Workshop After two successful ATLAS ROD Workshops dedicated to the ROD hardware and held at the Geneva University in 1998 and in 2000, a new style Workshop took place at LAPP in Annecy on November 14-15, 2002. This time the Workshop was fully dedicated to the ROD-TDAQ integration and software in view of the near future integration activities of the final RODs for the detector assembly and commissioning. More precisely, the aim of this workshop was to get from the sub-detectors the parameters needed for T-DAQ, as well as status and plans from ROD builders. On the other hand, what was decided and assumed had to be stated (like EB decisions and URDs), and also support plans. The Workshop gathered about 70 participants from all ATLAS sub-detectors and the T-DAQ community. The quite dense agenda allowed nevertheless for many lively discussions, and for a dinner in the old town of Annecy. The Sessions The Workshop was organized in five main sessions: Assumptions and recommendations Sub-de...

Temperature actuated automatic safety rod release

Science.gov (United States)

Hutter, E.; Pardini, J.A.; Walker, D.E.

1984-03-13

A temperature-actuated apparatus is disclosed for releasably supporting a safety rod in a nuclear reactor, comprising a safety rod upper adapter having a retention means, a drive shaft which houses the upper adapter, and a bimetallic means supported within the drive shaft and having at least one ledge which engages a retention means of the safety rod upper adapter. A pre-determined increase in temperature causes the bimetallic means to deform so that the ledge disengages from the retention means, whereby the bimetallic means releases the safety rod into the core of the reactor.

Shielding device for control rod in nuclear reactor

International Nuclear Information System (INIS)

Sakamaki, Kazuo; Tomatsu, Tsutomu.

1995-01-01

The device of the present invention shields radiation emitted from control rods to greatly reduce an operator's radiation exposure even if reactor water level is lowered and the upper portion of the control rod is exposed upon inspection of a BWR type reactor. Namely, a shield assembly has a structure comprising a set of four columnar shields in a two-row and two-column arrangement, which can be inserted into a control rod guide tube. Upon conducting inspection, the control rod is lowered into the control rod guide tube, and in this state, the columnar shields of the shield assembly are inserted to the control rod in the control rod guide tube. With such procedures, the upper portion of the control rod protruded from the control rod guide tube is covered with the shield assembly. As a result, radiation leaked from the control rod is shielded. Accordingly, irradiation in the reactor due to leaked radiation can be prevented thereby enabling to reduce an operator's radiation exposure. (I.S.)

Burnable poison rod

International Nuclear Information System (INIS)

Natsume, Tomohiro.

1988-01-01

Purpose: To increase the reactor core lifetime by decreasing the effect of neutron absorption of burnable poison rods by using material with less neutron absorbing effect. Constitution: Stainless steels used so far as the coating material for burnable poison rods have relatively great absorption in the thermal neutral region and are not preferred in view of the neutron economy. Burnable poison rods having fuel can made of zirconium alloy shows absorption the thermal neutron region lower by one digit than that of stainless steels but they shows absorption in the resonance region and the cost is higher. In view of the above, the fuel can of the burnable poison material is made of aluminum or aluminu alloy. This can reduce the neutron absorbing effect by stainless steel fuel can and effectively utilize neutrons that have been wastefully absorbed and consumed in stainless steels. (Takahashi, M.)

Optical coherence tomography of the photoreceptor layer in the healthy eye and in eyes with hereditary macular dystrophy

International Nuclear Information System (INIS)

Stur, M.; Hermann, B.; Drexler, W.; Unterhuber, A.; Sattmann, H.; Ergun, E.; Wirtitsch, M.

2007-01-01

Optical coherence tomography is primarily used for the evaluation of pronounced alterations of the retinal architecture, such as in macular holes, epiretinal gliosis, intra- and subretinal fluid accumulation as well as retinal atrophy. Ultrahigh resolution OCT devices also allow the assessment of discrete alterations of the photoreceptor layer and the retinal pigment epithelium. On the basis of cases from two different macular dystrophies, the importance of the evaluation of the photoreceptor layer and its correlation with visual acuity is demonstrated.(author) [de

In silico modeling of epigenetic-induced changes in photoreceptor cis-regulatory elements.

Science.gov (United States)

Hossain, Reafa A; Dunham, Nicholas R; Enke, Raymond A; Berndsen, Christopher E

2018-01-01

DNA methylation is a well-characterized epigenetic repressor of mRNA transcription in many plant and vertebrate systems. However, the mechanism of this repression is not fully understood. The process of transcription is controlled by proteins that regulate recruitment and activity of RNA polymerase by binding to specific cis-regulatory sequences. Cone-rod homeobox (CRX) is a well-characterized mammalian transcription factor that controls photoreceptor cell-specific gene expression. Although much is known about the functions and DNA binding specificity of CRX, little is known about how DNA methylation modulates CRX binding affinity to genomic cis-regulatory elements. We used bisulfite pyrosequencing of human ocular tissues to measure DNA methylation levels of the regulatory regions of RHO , PDE6B, PAX6 , and LINE1 retrotransposon repeats. To describe the molecular mechanism of repression, we used molecular modeling to illustrate the effect of DNA methylation on human RHO regulatory sequences. In this study, we demonstrate an inverse correlation between DNA methylation in regulatory regions adjacent to the human RHO and PDE6B genes and their subsequent transcription in human ocular tissues. Docking of CRX to the DNA models shows that CRX interacts with the grooves of these sequences, suggesting changes in groove structure could regulate binding. Molecular dynamics simulations of the RHO promoter and enhancer regions show changes in the flexibility and groove width upon epigenetic modification. Models also demonstrate changes in the local dynamics of CRX binding sites within RHO regulatory sequences which may account for the repression of CRX-dependent transcription. Collectively, these data demonstrate epigenetic regulation of CRX binding sites in human retinal tissue and provide insight into the mechanism of this mode of epigenetic regulation to be tested in future experiments.

Phenotypic diversity in autosomal-dominant cone-rod dystrophy elucidated by adaptive optics retinal imaging.

Science.gov (United States)

Song, Hongxin; Rossi, Ethan A; Stone, Edwin; Latchney, Lisa; Williams, David; Dubra, Alfredo; Chung, Mina

2018-01-01

Several genes causing autosomal-dominant cone-rod dystrophy (AD-CRD) have been identified. However, the mechanisms by which genetic mutations lead to cellular loss in human disease remain poorly understood. Here we combine genotyping with high-resolution adaptive optics retinal imaging to elucidate the retinal phenotype at a cellular level in patients with AD-CRD harbouring a defect in the GUCA1A gene. Nine affected members of a four-generation AD-CRD pedigree and three unaffected first-degree relatives underwent clinical examinations including visual acuity, fundus examination, Goldmann perimetry, spectral domain optical coherence tomography and electroretinography. Genome-wide scan followed by bidirectional sequencing was performed on all affected participants. High-resolution imaging using a custom adaptive optics scanning light ophthalmoscope (AOSLO) was performed for selected participants. Clinical evaluations showed a range of disease severity from normal fundus appearance in teenaged patients to pronounced macular atrophy in older patients. Molecular genetic testing showed a mutation in in GUCA1A segregating with disease. AOSLO imaging revealed that of the two teenage patients with mild disease, one had severe disruption of the photoreceptor mosaic while the other had a normal cone mosaic. AOSLO imaging demonstrated variability in the pattern of cone and rod cell loss between two teenage cousins with early AD-CRD, who had similar clinical features and had the identical disease-causing mutation in GUCA1A . This finding suggests that a mutation in GUCA1A does not lead to the same degree of AD-CRD in all patients. Modifying factors may mitigate or augment disease severity, leading to different retinal cellular phenotypes. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Simulation of leaking fuel rods

International Nuclear Information System (INIS)

Hozer, Z.

2006-01-01

The behaviour of failed fuel rods includes several complex phenomena. The cladding failure initiates the release of fission product from the fuel and in case of large defect even urania grains can be released into the coolant. In steady state conditions an equilibrium - diffusion type - release is expected. During transients the release is driven by a convective type leaching mechanism. There are very few experimental data on leaking WWER fuel rods. For this reason the activity measurements at the nuclear power plants provide very important information. The evaluation of measured data can help in the estimation of failed fuel rod characteristics and the prediction of transient release dynamics in power plant transients. The paper deals with the simulation of leaking fuel rods under steady state and transient conditions and describes the following new results: 1) A new algorithm has been developed for the simulation of leaking fuel rods under steady state conditions and the specific parameters of the model for the Paks NPP has been determined; 2) The steady state model has been applied to calculation of leaking fuel characteristics using iodine and noble gas activity measurement data; 3) A new computational method has been developed for the simulation of leaking fuel rods under transient conditions and the specific parameters for the Paks NPP has been determined; 4) The transient model has been applied to the simulation of shutdown process at the Paks NPP and for the prediction of the time and magnitude of 123 I activity peak; 5) Using Paks NPP data a conservative value has been determined for the upper limit of the 123 I release from failed fuel rods during transients

Control rod for HTGR type reactor

International Nuclear Information System (INIS)

Mogi, Haruyoshi; Saito, Yuji; Fukamichi, Kenjiro.

1990-01-01

Upon dropping control rod elements into the reactor core, impact shocks are applied to wire ropes or spines to possibly deteriorate the integrity of the control rods. In view of the above in the present invention, shock absorbers such as springs or bellows are disposed between a wire rope and a spine in a HTGR type reactor control rod comprising a plurality of control rod elements connected axially by means of a spine that penetrates the central portion thereof, and is suspended at the upper end thereof by a wire rope. Impact shocks of about 5 kg are applied to the wire rope and the spine and, since they can be reduced by the shock absorbers, the control rod integrity can be maintained and the reactor safety can be improved. (T.M.)

Effect of carotenoid supplementation on plasma carotenoids, inflammation and visual development in preterm infants.

Science.gov (United States)

Rubin, L P; Chan, G M; Barrett-Reis, B M; Fulton, A B; Hansen, R M; Ashmeade, T L; Oliver, J S; Mackey, A D; Dimmit, R A; Hartmann, E E; Adamkin, D H

2012-06-01

Dietary carotenoids (lutein, lycopene and β-carotene) may be important in preventing or ameliorating prematurity complications. Little is known about carotenoid status or effects of supplementation. This randomized controlled multicenter trial compared plasma carotenoid levels among preterm infants (n=203, lutein, lycopene and β-carotene with human milk (HM)-fed term infants. We assessed safety and health. Plasma carotenoid levels were higher in the supplemented group at all time points (Plutein levels correlated with the full field electroretinogram-saturated response amplitude in rod photoreceptors (r=0.361, P=0.05). The supplemented group also showed greater rod photoreceptor sensitivity (least squares means 6.1 vs 4.1; Plutein on preterm retina health and maturation.

Why Rods and Cocci

Indian Academy of Sciences (India)

Bacteria exhibit a wide variety of shapes but the commonly studied species of bacteria are generally either spherical in shape which are called cocci (singular coccus) or have a cylindrical shape and are called rods or bacilli (singular bacillus). In reality rods and cocci are the ends of a continuum. Sonle of the cocci are.

Spacers for fuel rod clusters

International Nuclear Information System (INIS)

Jabsen, F.S.

1978-01-01

The proposition deals with the fixing of nuclear fuel element rods in a grid which consists of a number of crossed Zy-plates which form cells. The rectangular cells have projections which serve as spacers for the fuel rods. According to the invention there are additional butt straps which can be moved in such a way that insertion and extraction of the fuel rods can be done without obstruction and they can be spring-loaded hold in their final position. (UWI) [de

Rod consolidation at the West Valley Demonstration Project

International Nuclear Information System (INIS)

Bailey, W.J.

1986-12-01

A rod consolidation demonstration with irradiated pressurized water reactor fuel was recently conducted by personnel from Nuclear Assurance Corporation and West Valley Nuclear Services Company at the West Valley Demonstration Project in West Valley, New York. The rod consolidation demonstration involved pulling all of the fuel rods from six fuel Assemblies. In general, the rod pulling proceeded smoothly. The highest compaction ratio attained was 1:8:1. Among the total of 1074 fuel rods were some known degraded rods (they had collapsed cladding, a result of in-reactor fuel densification), but no rods were broken or dropped during the demonstration. One aim was to gather information on the effect of rod consolidation operations on the integrity of the fuel rods during subsequent handling and storage. Another goal was to collect information on the condition and handling of intact, damaged, and failed fuel that has been in storage for an extended period. 9 refs., 8 figs., 1 tab

Towards a Statistical Model of Tropical Cyclone Genesis

Science.gov (United States)

Fernandez, A.; Kashinath, K.; McAuliffe, J.; Prabhat, M.; Stark, P. B.; Wehner, M. F.

2017-12-01

Tropical Cyclones (TCs) are important extreme weather phenomena that have a strong impact on humans. TC forecasts are largely based on global numerical models that produce TC-like features. Aspects of Tropical Cyclones such as their formation/genesis, evolution, intensification and dissipation over land are important and challenging problems in climate science. This study investigates the environmental conditions associated with Tropical Cyclone Genesis (TCG) by testing how accurately a statistical model can predict TCG in the CAM5.1 climate model. TCG events are defined using TECA software @inproceedings{Prabhat2015teca, title={TECA: Petascale Pattern Recognition for Climate Science}, author={Prabhat and Byna, Surendra and Vishwanath, Venkatram and Dart, Eli and Wehner, Michael and Collins, William D}, booktitle={Computer Analysis of Images and Patterns}, pages={426-436}, year={2015}, organization={Springer}} to extract TC trajectories from CAM5.1. L1-regularized logistic regression (L1LR) is applied to the CAM5.1 output. The predictions have nearly perfect accuracy for data not associated with TC tracks and high accuracy differentiating between high vorticity and low vorticity systems. The model's active variables largely correspond to current hypotheses about important factors for TCG, such as wind field patterns and local pressure minima, and suggests new routes for investigation. Furthermore, our model's predictions of TC activity are competitive with the output of an instantaneous version of Emanuel and Nolan's Genesis Potential Index (GPI) @inproceedings{eman04, title = "Tropical cyclone activity and the global climate system", author = "Kerry Emanuel and Nolan, {David S.}", year = "2004", pages = "240-241", booktitle = "26th Conference on Hurricanes and Tropical Meteorology"}.

21 CFR 876.4270 - Colostomy rod.

Science.gov (United States)

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Colostomy rod. 876.4270 Section 876.4270 Food and... GASTROENTEROLOGY-UROLOGY DEVICES Surgical Devices § 876.4270 Colostomy rod. (a) Identification. A colostomy rod is a device used during the loop colostomy procedure. A loop of colon is surgically brought out through...

Control rod for FBR type reactor

International Nuclear Information System (INIS)

Nakai, Koichi.

1993-01-01

In a control rod for an LMFBR type reactor, a thermal resistor is disposed between a temperature sensitive cylinder and a cam unit support rod. A thermal expansion difference due to the temperature difference is caused between the temperature sensitive cylinder and the cam unit support rod only upon abrupt temperature change of coolants. A control rod shaft extending mechanism of downwardly depressing an absorbent portion by amplifying the thermal expansion difference by an extension link mechanism and the cam unit is provided. The thermal resistor comprises inconel 625 or like other steel of small heat conductivity. If a certain abnormality should cause to the reactor system to elevate the coolant temperature in the reactor elevates abruptly and the reactor shutdown system does not actuate, since the control rod extension shaft extends to urge the absorbent and lower the reactor core reactivity, so that leading to serious accident can be prevented surely. Further, the control rod extension shaft does not extend upon moderate temperature elevation in the usual startup and causes no unnecessary reactivity change. (N.H.)

Design of active-neutron fuel rod scanner

International Nuclear Information System (INIS)

Griffith, G.W.; Menlove, H.O.

1996-01-01

An active-neutron fuel rod scanner has been designed for the assay of fissile materials in mixed oxide fuel rods. A 252 Cf source is located at the center of the scanner very near the through hole for the fuel rods. Spontaneous fission neutrons from the californium are moderated and induce fissions within the passing fuel rod. The rod continues past a combined gamma-ray and neutron shield where delayed gamma rays above 1 MeV are detected. We used the Monte Carlo code MCNP to design the scanner and review optimum materials and geometries. An inhomogeneous beryllium, graphite, and polyethylene moderator has been designed that uses source neutrons much more efficiently than assay systems using polyethylene moderators. Layers of borated polyethylene and tungsten are used to shield the detectors. Large NaI(Tl) detectors were selected to measure the delayed gamma rays. The enrichment zones of a thermal reactor fuel pin could be measured to within 1% counting statistics for practical rod speeds. Applications of the rod scanner include accountability of fissile material for safeguards applications, quality control of the fissile content in a fuel rod, and the verification of reactivity potential for mixed oxide fuels. (orig.)

Hydraulically centered control rod

International Nuclear Information System (INIS)

Horlacher, W.R.; Sampson, W.T.; Schukei, G.E.

1981-01-01

A control rod suspended to reciprocate in a guide tube of a nuclear fuel assembly has a hydraulic bearing formed at its lower tip. The bearing includes a plurality of discrete pockets on its outer surface into which a flow of liquid is continuously provided. In one embodiment the flow is induced by the pressure head in a downward facing chamber at the end of the bearing. In another embodiment the flow originates outside the guide tube. In both embodiments the flow into the pockets produces pressure differences across the bearing which counteract forces tending to drive the rod against the guide tube wall. Thus contact of the rod against the guide tube is avoided

OUTCOMES REVIEW OF TOTAL KNEE REPLACEMENT WITH GENESIS II TOTAL KNEE SYSTEM: AUSTRALIAN ORTHOPAEDIC ASSOCIATION NATIONAL JOINT REPLACEMENT REGISTRY DATA

Directory of Open Access Journals (Sweden)

N. L. Logvinov

2014-01-01

Full Text Available ABSTRACT. The goal of the article was to review Genesis II Total Knee System use according to AoA NJRR report. Genesis II has been widely used in Russian Federation since 1996. The AoA NJRR report was studied in order to learn connection between number of revision procedures and surgeon’s skills or type of the System, adequate choice among cemented, cementless and hybrid fixation, number of surgeries using Genesis II Total Knee System annually, average revision rate for Genesis II and early cumulative revision rate.CONCLUSION. The systematic study of AoA NJRR allows us to make a conclusion that cemented fixation of Genesis II Total Knee System is mostly effective, and this fact is confirmed by domestic experience. 

Kollig op Genesis 1-3: 'n verslag van verskuiwende denke en geloof

OpenAIRE

Spangenberg, Sakkie

2014-01-01

The author narrates how his views concerning Gen 1-3 have changed over the past forty years. He enrolled for theological training at the University of Stellenbosch during the seventies of the previous century and had to study the book Hoe lezen wij Genesis 2 en 3? ("How should we read Genesis 2 and 3?"). The book had been written by B. J. Oosterhoff, a Dutch scholar who argued a case for a symbolic reading of the text. Since then the author has become acquainted with the paradigm change in th...

Spider and burnable poison rod combinations

International Nuclear Information System (INIS)

Edwards, G.T.; Schluderberg, D.C.

1980-01-01

An improved design of burnable poison rods and associated spiders used in fuel assemblies of pressurized water power reactor cores, is described. The rods are joined to the spider arms in a manner which is proof against the reactor core environment and yet allows the removal of the rods from the spider simply, swiftly and delicately. (U.K.)

192 RE-INTERPRETING GENESIS 34 IN THE LIGHT OF ...

African Journals Online (AJOL)

User

notoriety as one of the prime security challenges confronting the country. Genesis .... thing has been done to them by Shechem. ... The use of the word חַטֶבּ which means security ..... students and internet writers; and the polylectic approach.

Declines in arrestin and rhodopsin in the macula with progression of age-related macular degeneration.

Science.gov (United States)

Ethen, Cheryl M; Feng, Xiao; Olsen, Timothy W; Ferrington, Deborah A

2005-03-01

Biochemical analysis of age-related macular degeneration (AMD) at distinct stages of the disease will help further understanding of the molecular events associated with disease progression. This study was conducted to determine the ability of a new grading system for eye bank eyes, the Minnesota Grading System (MGS), to discern distinct stages of AMD so that retinal region-specific changes in rod photoreceptor protein expression from donors could be determined. Donor eyes were assigned to a specific level of AMD by using the MGS. Expression of the rod photoreceptor proteins rhodopsin and arrestin was evaluated by Western immunoblot analysis in the macular and peripheral regions of the neurosensory retina from donors at different stages of AMD. A significant linear decline in both arrestin and rhodopsin content correlated with progressive MGS levels in the macula. In contrast, the peripheral region showed no significant correlation between MGS level and the content of either protein. The statistically significant relationship between decreasing macular rod photoreceptor proteins and progressive MGS levels of AMD demonstrates the utility of the clinically based MGS to correspond with specific protein changes found at known, progressive stages of degeneration. Future biochemical analysis of clinically characterized donor eyes will further understanding of the pathobiochemistry of AMD.

An event-related potential study on the interaction between lighting level and stimulus spatial location

Directory of Open Access Journals (Sweden)

Luis eCarretié

2015-11-01

Full Text Available Due to heterogeneous photoreceptor distribution, spatial location of stimulation is crucial to study visual brain activity in different light environments. This unexplored issue was studied through occipital event-related potentials (ERPs recorded from 40 participants in response to discrete visual stimuli presented at different locations and in two environmental light conditions, low mesopic (L, 0.03 lux and high mesopic (H, 6.5 lux, characterized by a differential photoreceptor activity balance: rod>cone and rodphotoreceptor activation and their retinal distribution, and confirm that ERPs discriminate between rod- and cone-originated visual processing.

Modeling and simulation performance of sucker rod beam pump

Energy Technology Data Exchange (ETDEWEB)

Aditsania, Annisa, E-mail: annisaaditsania@gmail.com [Department of Computational Sciences, Institut Teknologi Bandung (Indonesia); Rahmawati, Silvy Dewi, E-mail: silvyarahmawati@gmail.com; Sukarno, Pudjo, E-mail: psukarno@gmail.com [Department of Petroleum Engineering, Institut Teknologi Bandung (Indonesia); Soewono, Edy, E-mail: esoewono@math.itb.ac.id [Department of Mathematics, Institut Teknologi Bandung (Indonesia)

2015-09-30

Artificial lift is a mechanism to lift hydrocarbon, generally petroleum, from a well to surface. This is used in the case that the natural pressure from the reservoir has significantly decreased. Sucker rod beam pumping is a method of artificial lift. Sucker rod beam pump is modeled in this research as a function of geometry of the surface part, the size of sucker rod string, and fluid properties. Besides its length, sucker rod string also classified into tapered and un-tapered. At the beginning of this research, for easy modeling, the sucker rod string was assumed as un-tapered. The assumption proved non-realistic to use. Therefore, the tapered sucker rod string modeling needs building. The numerical solution of this sucker rod beam pump model is computed using finite difference method. The numerical result shows that the peak of polished rod load for sucker rod beam pump unit C-456-D-256-120, for non-tapered sucker rod string is 38504.2 lb, while for tapered rod string is 25723.3 lb. For that reason, to avoid the sucker rod string breaks due to the overload, the use of tapered sucker rod beam string is suggested in this research.

Modeling and simulation performance of sucker rod beam pump

International Nuclear Information System (INIS)

Aditsania, Annisa; Rahmawati, Silvy Dewi; Sukarno, Pudjo; Soewono, Edy

2015-01-01

Artificial lift is a mechanism to lift hydrocarbon, generally petroleum, from a well to surface. This is used in the case that the natural pressure from the reservoir has significantly decreased. Sucker rod beam pumping is a method of artificial lift. Sucker rod beam pump is modeled in this research as a function of geometry of the surface part, the size of sucker rod string, and fluid properties. Besides its length, sucker rod string also classified into tapered and un-tapered. At the beginning of this research, for easy modeling, the sucker rod string was assumed as un-tapered. The assumption proved non-realistic to use. Therefore, the tapered sucker rod string modeling needs building. The numerical solution of this sucker rod beam pump model is computed using finite difference method. The numerical result shows that the peak of polished rod load for sucker rod beam pump unit C-456-D-256-120, for non-tapered sucker rod string is 38504.2 lb, while for tapered rod string is 25723.3 lb. For that reason, to avoid the sucker rod string breaks due to the overload, the use of tapered sucker rod beam string is suggested in this research

Detection device for control rod interference

International Nuclear Information System (INIS)

Saito, Noboru.

1984-01-01

Purpose: To enable to detect the mechanical interference or friction between a control rod and a channel box automatically, simply and rapidly. Constitution: A signal from a gate circuit and a signal from a comparison mechanism are inputted into an AND circuit if a control rod has not been displaced by a predetermined distance within a prescribed time Δt after the output of an insertion or withdrawal signal for the control rod, by which a control-rod-interference signal is outputted from the AND circuit. Accordingly, the interference between the control rod and the channel box can be detected automatically, easily and rapidly. Furthermore, by properly adjusting the prescribed time Δt set by the gate circuit, the degree of the interference can also be detected, whereby the safety and the reliability of the reactor can be improved significantly. (Horiuchi, T.)

Radioactive lightning rods waste treatment

International Nuclear Information System (INIS)

Vicente, Roberto; Dellamano, Jose C.; Hiromoto, Goro

2008-01-01

Full text: In this paper, we present alternative processes that could be adopted for the management of radioactive waste that arises from the replacement of lightning rods with attached Americium-241 sources. Lightning protectors, with Americium-241 sources attached to the air terminals, were manufactured in Brazil until 1989, when the regulatory authority overthrew the license for fabrication, commerce, and installation of radioactive lightning rods. It is estimated that, during the license period, about 75,000 such devices were set up in public, commercial and industrial buildings, including houses and schools. However, the policy of CNEN in regard to the replacement of the installed radioactive rods, has been to leave the decision to municipal governments under local building regulations, requiring only that the replaced rods be sent immediately to one of its research institutes to be treated as radioactive waste. As a consequence, the program of replacement proceeds in a low pace and until now only about twenty thousand rods have reached the waste treatment facilities The process of management that was adopted is based primarily on the assumption that the Am-241 sources will be disposed of as radioactive sealed sources, probably in a deep borehole repository. The process can be described broadly by the following steps: a) Receive and put the lightning rods in initial storage; b) Disassemble the rods and pull out the sources; c) Decontaminate and release the metal parts to metal recycling; d) Store the sources in intermediate storage; e) Package the sources in final disposal packages; and f) Send the sources for final disposal. Up to now, the disassembled devices gave rise to about 90,000 sources which are kept in storage while the design of the final disposal package is in progress. (author)

Genesis of cluster associations of enterprises

Directory of Open Access Journals (Sweden)

Pulina Tetyana V.

2013-03-01

Full Text Available The goal of the article is the study of genesis of creation of cluster associations of enterprises. It considers genesis of cluster definitions. It shows and analyses components that define the “cluster” concept. Researchers from many countries offer a significant number of definitions of the “cluster” term specifically in the economic direction, but there is no single generally accepted definition as of today. This fact is the result of a significant diversity of cluster structures. The article conducts a comparative analysis of classifications of cluster associations of enterprises. It identifies advantages and shortcomings of the cluster approach both from the position of an enterprise and from the position of a regional economy administration. The article marks out specific features of the life cycle of cluster associations of enterprises, which consists of the preparatory stage and stage of commercialisation. Majority of studies consider the preparatory stage and the stage of commercialisation, which consists of the following stages: entering market with a common brand, growth, maturity and crisis – is, practically, not considered. Taking into account the fact that the main result of cluster activity is the synergetic effect from mutually beneficial co-operation and activity results facilitate ensuring competitiveness of cluster enterprises, regional and national economies, the author gives own definition of a cluster.

Quantitative analysis of cone photoreceptor distribution and its relationship with axial length, age, and early age-related macular degeneration.

Directory of Open Access Journals (Sweden)

Ryo Obata

Full Text Available PURPOSE: It has not been clarified whether early age-related macular degeneration (AMD is associated with cone photoreceptor distribution. We used adaptive optics fundus camera to examine cone photoreceptors in the macular area of aged patients and quantitatively analyzed its relationship between the presence of early AMD and cone distribution. METHODS: Sixty cases aged 50 or older were studied. The eyes were examined with funduscopy and spectral-domain optical coherence tomography to exclude the eyes with any abnormalities at two sites of measurement, 2° superior and 5° temporal to the fovea. High-resolution retinal images with cone photoreceptor mosaic were obtained with adaptive optics fundus camera (rtx1, Imagine Eyes, France. After adjusting for axial length, cone packing density was calculated and the relationship with age, axial length, or severity of early AMD based on the age-related eye disease study (AREDS classification was analyzed. RESULTS: Patient's age ranged from 50 to 77, and axial length from 21.7 to 27.5 mm. Mean density in metric units and that in angular units were 24,900 cells/mm2, 2,170 cells/deg2 at 2° superior, and 18,500 cells/mm2, 1,570 cels/deg2 at 5° temporal, respectively. Axial length was significantly correlated with the density calculated in metric units, but not with that in angular units. Age was significantly correlated with the density both in metric and angular units at 2° superior. There was no significant difference in the density in metric and angular units between the eyes with AREDS category one and those with categories two or three. CONCLUSION: Axial length and age were significantly correlated with parafoveal cone photoreceptor distribution. The results do not support that early AMD might influence cone photoreceptor density in the area without drusen or pigment abnormalities.

The genesis and evolution of the African Field Epidemiology Network

African Journals Online (AJOL)

The genesis and evolution of the African Field Epidemiology Network. David Mukanga, Mufuta Tshimanga, Frederick Wurapa, David Serwada, George Pariyo, Fred Wabwire-Mangen, Sheba Gitta, Stella Chungong, Murray Trostle, Peter Nsubuga ...

Snubber assembly for a control rod drive

International Nuclear Information System (INIS)

1976-01-01

A snubber cartridge assembly is described which is mounted to the nozzle of a control rod drive mechanism to insure that it will be located within the liquid filled section of a nuclear reactor vessel whenever the control rod drive is assembled thereto. The snubber assembly includes a piston-mounted proximate to the control rod connecting end of the control rod drive leadscrew to allow the piston to travel within the liquid filled snubber cartridge and controllable exhaust the liquid during a 'scram' condition. The snubber cartridge provides three separate areas of increasing resistance to piston travel to insure a speedy but safe 'scram' of the control rod into the reactor

Automated nuclear fuel rod pattern loading system

International Nuclear Information System (INIS)

Lambert, D.V.; Nyland, T.W.; Byers, J.W.; Haley, D.E. Jr.; Cioffi, J.V.

1990-01-01

This patent describes an apparatus for loading fuel rods in a desired pattern. It comprises: a carousel having a plurality of movable gondolas for stocking thereon fuel rods of known enrichments; an elongated magazine defining a matrix of elongated slots being open at their forward ends for receiving fuel rods; a workstation defining a fuel rod feed path; and a holder and indexing mechanism for movably supporting the magazine and being actuatable for moving the magazine along X-Y axes to successively align one at a time selected ones of the slots with the feed path for loading in the magazine the successive fuel rods in a desired enrichment pattern

Snubber assembly for a control rod drive

International Nuclear Information System (INIS)

Matthews, J.C.

1978-01-01

A snubber cartridge assembly is mounted to the nozzle of a control rod drive mechanism to insure that the snubber assembly will be located within the liquid filled section of a nuclear reactor vessel whenever the control rod drive is assembled thereto. The snubber assembly includes a piston mounted proximate to the control rod connecting end of the control rod drive leadscrew to allow the piston to travel within the liquid filled snubber cartridge and controllably exhaust liquid therefrom during a ''scram'' condition. The snubber cartridge provides three separate areas of increasing resistance to piston travel to insure a speedy but safe ''scram'' of the control rod into the reactor

Method and apparatus for inspection of nuclear fuel rods

International Nuclear Information System (INIS)

Wachter, W.J.

1977-01-01

A method and apparatus are provided for the inspection of nuclear fuel rods to detect defects or failures in such rods. Assemblies of fuel rods are immersed in water and means are provided for causing a change in the relative pressures in the water and within the fuel rod such that fluid is expelled from the rod through any defects that may exist. Means are also provided for thereafter vibrating the rods to cause additional internal fluid or other material that may be trapped in the rod to be expelled. Sensors are provided for detecting the emission of bubbles of fluid or other material from the rod and for locating the position of the defective rod in the assembly. 5 figures

Mechanical properties of bioresorbable self-reinforced posterior cervical rods.

Science.gov (United States)

Savage, Katherine; Sardar, Zeeshan M; Pohjonen, Timo; Sidhu, Gursukhman S; Eachus, Benjamin D; Vaccaro, Alexander

2014-04-01

A biomechanical study. To test the mechanical and physical properties of self-reinforced copolymer bioresorbable posterior cervical rods and compare their mechanical properties to commonly used Irene titanium alloy rods. Bioresorbable instrumentation is becoming increasingly common in surgical spine procedures. Compared with metallic implants, bioresorbable implants are gradually reabsorbed as the bone heals, transferring the load from the instrumentation to bone, eliminating the need for hardware removal. In addition, bioresorbable implants produce less stress shielding due to a more physiological modulus of elasticity. Three types of rods were used: (1) 5.5 mm copolymer rods and (2) 3.5 mm and (3) 5.5 mm titanium alloy rods. Four tests were used on each rod: (1) 3-point bending test, (2) 4-point bending test, (3) shear test, and (4) differential scanning calorimeter test. The outcomes were recorded: Young modulus (E), stiffness, maximum load, deflection at maximum load, load at 1.0% strain of the rod's outer surface, and maximum bending stress. The Young modulus (E) for the copolymer rods (mean range, 6.4-6.8 GPa) was significantly lower than the 3.5 mm titanium rods (106 GPa) and the 5.5 mm titanium rods (95 GPa). The stiffness of the copolymer rods (mean range, 16.6-21.4 N/mm) was also significantly lower than the 3.5 mm titanium alloy rods (43.6 N/mm) and the 5.5 mm titanium alloy rods (239.6 N/mm). The mean maximum shear load of the copolymer rods was 2735 N and they had significantly lower mean maximum loads than the titanium rods. Copolymer rods have adequate shear resistance, but less load resistance and stiffness compared with titanium rods. Their stiffness is closer to that of bone, causing less stress shielding and better gradual dynamic loading. Their use in semirigid posterior stabilization of the cervical spine may be considered.

Implementation of CTRLPOS, a VENTURE module for control rod position criticality searches, control rod worth curve calculations, and general criticality searches

Energy Technology Data Exchange (ETDEWEB)

Smith, L.A.; Renier, J.P.

1994-06-01

A module in the VENTURE reactor analysis code system, CTRLPOS, is developed to position control rods and perform control rod position criticality searches. The module is variably dimensioned so that calculations can be performed with any number of control rod banks each having any number of control rods. CTRLPOS can also calculate control rod worth curves for a single control rod or a bank of control rods. Control rod depletion can be calculated to provide radiation source terms. These radiation source terms can be used to predict radiation doses to personnel and estimate the shielding and long-term storage requirements for spent control rods. All of these operations are completely automated. The numerous features of the module are discussed in detail. The necessary input data for the CTRLPOS module is explained. Several sample problems are presented to show the flexibility of the module. The results presented with the sample problems show that the CTRLPOS module is a powerful tool which allows a wide variety of calculations to be easily performed.

Absorber rod drive for nuclear reactors

International Nuclear Information System (INIS)

Acher, H.

1985-01-01

The invention concerns a further addition to the invention of DE 33 42 830 A1. The free contact of the hollow piston with the nut due to hydraulic pressure is replaced by a hydraulic or spring attachment. The pressure system required to produce the hydraulic pressure is therefore omitted, and the electrical power required for driving the pump or the mass flow is also omitted. The absorber rod slotted along its longitudinal axis is replaced by an absorber rod, in the longitudinal axis of which a hollow piston is connected together with the absorber rod. This makes the absorber rod more stable, and assembly is simplified. (orig./HP) [de

Nuclear reactor fuel rod attachment system

International Nuclear Information System (INIS)

Christiansen, D.W.

1983-01-01

The invention involves a technique to quickly, inexpensively and rigidly attach a nuclear reactor fuel rod to a support member. The invention also allows for the repeated non-destructive removal and replacement of the fuel rod. The proposed fuel rod and support member attachment and removal system consists of a locking cap fastened to the fuel rod and a locking strip fastened to the support member or vice versa. The locking cap has two or more opposing fingers shaped to form a socket. The fingers spring back when moved apart and released. The locking strip has an extension shaped to rigidly attach to the socket's body portion

Integrating Topographic Measures to Explore the Protective Effects of Peonidin Against the N-Methyl-N-Nitrosourea Induced Photoreceptor Degeneration

Directory of Open Access Journals (Sweden)

Ye Tao

2016-02-01

Full Text Available Background/Aims: The pathphysiological properties of N-Methyl -N -nitrosourea (MNU induced photoreceptor degeneration are similar to the hereditary retinitis pigmentosa (RP. The present study sought to explore the beneficial effects of the peonidin, a common aglycone form of anthocyanin, on the MNU induced photoreceptor degeneration via topographic measurements. Methods: The MNU administrated mouse received peonidin or vehicle injections, and then they were examined by electroretinography (ERG, multi electrode array (MEA, histological and immunohistochemistry studies. Results: The protective effects of peonidin on the MNU administrated retinas were systematically verified and quantified by topographic measures. The peonidin treatment could protect the photoreceptor against the MNU toxicity both functionally and morphologicaly. The most sensitive zone to peonidin therapy was sorted out, indicating that different rescuing kinetics existed between the retinal hemispheres and retinal quadrants. Moreover, the hyperactive spontaneous firing response and the debilitated light induced response in MNU administrated retinas could be partially reversed by peonidin treatment. To our knowledge, this was the first study to explore the pharmacological effects of peonidin on the electrophysiological properties of inner visual signal pathways. Conclusion: The peonidin could ameliorate the MNU induced photoreceptors degeneration and rectify the abnormities in the inner visual signal pathways. Future refinements of the knowledge cast insights into the discovery of a novel treatment for human RP.

Control device for the withdrawal of control rod

International Nuclear Information System (INIS)

Ando, Masaki.

1985-01-01

Purpose: To significantly suppress the maximum value of the control-rod worth upon control rod withdrawal. Constitution: At first, a signal for designating the first class is sent from a class-control section to the group-control section. In the group-control section, the peripheral group among the first class is designated by which the withdrawal of the control rods other than the peripheral group is inhibited and the control-rods in the peripheral group are withdrawn one by one. When all of them have been withdrawn, the group-control section designates the central group of the first class. All the control rods of the central group have been withdrawn, then the group-control section designates the peripheral group of the second class. Thereafter, the central group in the second class is designated. The control rods are thus withdrawn in the same manner hereinafter. The maximum value for the control-rod worth can be decreased by such a withdrawing sequence for the control rods. (Horiuchi, T.)

Folk narratives structures in Genesis 2, 4 -3, 24

Directory of Open Access Journals (Sweden)

Magdalena Flores Ferres

2015-04-01

Full Text Available The objective of this research is to demonstrate that Genesis 2.4 to 3.24, can be considered, from the perspective of the structure, a traditional folk story, since it contains the morphologic elements of this type of literature. To that end, in this paper we focus on the study of the structure of the folk tale. The structure of the action sequence of such tales is interesting from differente disciplines, due to its remarkable stability. To that end, we will review the postulates proposed by Vladimir Propp in his book “Morphology of the Folktale” (1928. From these theoretical foundations, we conducted a contrastive analysis of the Genesis 2.4 to 3.24, in paralell to the russian folktale “The Cat, the Rooster and the Fox” (Afanasiev, 1981, after which it was found that both tales have the same narrative structure.

Control rod drive mechanism

International Nuclear Information System (INIS)

Mizuno, Katsuyuki.

1976-01-01

Object: To restrict the reduction in performance due to stress corrosion cracks by making use of condensate produced in a turbine steam condenser. Structure: Water produced in a turbine steam condenser is forced into a condensed water desalting unit by low pressure condensate pump. The condensate is purified and then forced by a high pressure condensate pump into a feedwater heater for heating before it is returned to the reactor by a feedwater pump. Part of the condensate issuing from the condensate desalting unit is branched from the remaining portion at a point upstream the pump and is withdrawn into a control rod drive water pump after passing through a motordriven bypass valve, an orifice and a condenser water level control valve, is pressurized in the control rod drive water desalting unit and supplied to a control rod drive water pressure system. The control rod is vertically moved by the valve operation of the water pressure system. Since water of high oxygen concentration does not enter during normal operation, it is possible to prevent the stress cracking of the stainless steel apparatus. (Nakamura, S.)

Temperature measurement in cans of fuel rods and fuel rod simulators

International Nuclear Information System (INIS)

Tschoeke, H.; Moeller, R.

1977-01-01

On the sodium-cooled 19-rod cluster model for the SNR 300 the can wall temperature distributions of the non-uniformly cooled rods were measured with thermocouples mounted in outer grooves in the peripheral zone, permitting, in connection with Ni solder, a practically undisturbed measurement. For a more exact determination of the local surface temperature a calibration method, the so-called double-wall method, was developed and applied. The description of this calibration method and the experimental results achieved until now are presented. (orig./RW) [de

Cryptochrome photoreceptors in green algae: Unexpected versatility of mechanisms and functions.

Science.gov (United States)

Kottke, Tilman; Oldemeyer, Sabine; Wenzel, Sandra; Zou, Yong; Mittag, Maria

2017-10-01

Green algae have a highly complex and diverse set of cryptochrome photoreceptor candidates including members of the following subfamilies: plant, plant-like, animal-like, DASH and cryptochrome photolyase family 1 (CPF1). While some green algae encode most or all of them, others lack certain members. Here we present an overview about functional analyses of so far investigated cryptochrome photoreceptors from the green algae Chlamydomonas reinhardtii (plant and animal-like cryptochromes) and Ostreococcus tauri (CPF1) with regard to their biological significance and spectroscopic properties. Cryptochromes of both algae have been demonstrated recently to be involved to various extents in circadian clock regulation and in Chlamydomonas additionally in life cycle control. Moreover, CPF1 even performs light-driven DNA repair. The plant cryptochrome and CPF1 are UVA/blue light receptors, whereas the animal-like cryptochrome responds to almost the whole visible spectrum including red light. Accordingly, plant cryptochrome, animal-like cryptochrome and CPF1 differ fundamentally in their structural response to light as revealed by their visible and infrared spectroscopic signatures, and in the role of the flavin neutral radical acting as dark form or signaling state. Copyright © 2017 Elsevier GmbH. All rights reserved.

Interaction of two photoreceptors in the regulation of bacterial photosynthesis genes.

Science.gov (United States)

Metz, Sebastian; Haberzettl, Kerstin; Frühwirth, Sebastian; Teich, Kristin; Hasewinkel, Christian; Klug, Gabriele

2012-07-01

The expression of photosynthesis genes in the facultatively photosynthetic bacterium Rhodobacter sphaeroides is controlled by the oxygen tension and by light quantity. Two photoreceptor proteins, AppA and CryB, have been identified in the past, which are involved in this regulation. AppA senses light by its N-terminal BLUF domain, its C-terminal part binds heme and is redox-responsive. Through its interaction to the transcriptional repressor PpsR the AppA photoreceptor controls expression of photosynthesis genes. The cryptochrome-like protein CryB was shown to affect regulation of photosynthesis genes, but the underlying signal chain remained unknown. Here we show that CryB interacts with the C-terminal domain of AppA and modulates the binding of AppA to the transcriptional repressor PpsR in a light-dependent manner. Consequently, binding of the transcription factor PpsR to its DNA target is affected by CryB. In agreement with this, all genes of the PpsR regulon showed altered expression levels in a CryB deletion strain after blue-light illumination. These results elucidate for the first time how a bacterial cryptochrome affects gene expression.

Development of cutting device for irradiated fuel rod

International Nuclear Information System (INIS)

Lee, E. P.; Jun, Y. B.; Hong, K. P.; Min, D. K.; Lee, H. K.; Su, H. S.; Kim, K. S.; Kwon, H. M.; Joo, Y. S.; Yoo, K. S.; Joo, J. S.; Kim, E. K.

2004-01-01

Post Irradiation Examination(PIE) on irradiated fuel rods is essential for the evaluation of integrity and irradiation performance of fuel rods of commercial reactor fuel. For PIE, fuel rods should be cut very precisely. The cutting positions selected from NDT data are very important for further destructive examination and analysis. A fuel rod cutting device was developed witch can cut fuel rods longitudinal very precisely and can also cut the fuels into the same length rod cuts repeatedly. It is also easy to remove the fuel cutting powder after cutting works and it can extend the life time of cutting device and lower the contamination level of hot cell

Fabrication Of Control Rod System Of The RSG-GAS

International Nuclear Information System (INIS)

Sudirdjo, Hari; Setyono; Prasetya, Hendra

2001-01-01

Eight units of control rod mechanical system of RSG-GAS has been fabricated. The control rod mechanical system of RSG-GAS consist of guide tube and lifting rod. Complete construction of the control rod mechanical system of RSG-GAS are guide tube, lifting rod, absorber, and absorber casing. The eight units of the control rod mechanical system of RSG-GAS has been fabricated according to the mechanical engineering design

Optical imaging of human cone photoreceptors directly following the capture of light.

Directory of Open Access Journals (Sweden)

Phillip Bedggood

Full Text Available Capture of light in the photoreceptor outer segment initiates a cascade of chemical events that inhibit neurotransmitter release, ultimately resulting in vision. The massed response of the photoreceptor population can be measured non-invasively by electrical recordings, but responses from individual cells cannot be measured without dissecting the retina. Here we used optical imaging to observe individual human cones in the living eye as they underwent bleaching of photopigment and associated phototransduction. The retina was simultaneously stimulated and observed with high intensity visible light at 1 kHz, using adaptive optics. There was marked variability between individual cones in both photosensitivity and pigment optical density, challenging the conventional assumption that photoreceptors act as identical subunits (coefficient of variation in rate of photoisomerization = 23%. There was also a pronounced inverse correlation between these two parameters (p<10(-7; the temporal evolution of image statistics revealed this to be a dynamic relationship, with cone waveguiding efficiency beginning a dramatic increase within 3 ms of light onset. Beginning as early as 2 ms after light onset and including half of cells by ∼7 ms, cone intensity showed reversals characteristic of interference phenomena, with greater delays in reversal corresponding to cones with more photopigment (p<10(-3. The timing of these changes is argued to best correspond with either the cessation of dark current, or to related events such as changes in intracellular cGMP. Cone intensity also showed fluctuations of high frequency (332±25 Hz and low amplitude (3.0±0.85%. Other groups have shown similar fluctuations that were directly evoked by light; if this corresponds to the same phenomenon, we propose that the amplitude of fluctuation may be increased by the use of a bright flash followed by a brief pause, to allow recovery of cone circulating current.

Microcomputer system for controlling fuel rod length

International Nuclear Information System (INIS)

Meyer, E.R.; Bouldin, D.W.; Bolfing, B.J.

1979-01-01

A system is being developed at the Oak Ridge National Laboratory (ORNL) to automatically measure and control the length of fuel rods for use in a high temperature gas-cooled reactor (HTGR). The system utilizes an LSI-11 microcomputer for monitoring fuel rod length and for adjusting the primary factor affecting length. Preliminary results indicate that the automated system can maintain fuel rod length within the specified limits of 1.940 +- 0.040 in. This system provides quality control documentation and eliminates the dependence of the current fuel rod molding process on manual length control. In addition, the microcomputer system is compatible with planned efforts to extend control to fuel rod fissile and fertile material contents

Chemical Excitation and Inactivation in Photoreceptors of the Fly Mutants trp and nss

NARCIS (Netherlands)

Suss, E.; Barash, S.; Stavenga, D.G.; Stieve, H.; Selinger, Z.; Minke, B.

1989-01-01

The Drosophila and Lucilia photoreceptor mutants, trp and nss, respond like wild-type flies to a short pulse of intense light or prolonged dim light; however, upon continuous intense illumination, the trp and nss mutants are unable to maintain persistent excitation. This defect manifests itself by a

Low-resolution characterization of the 3D structure of the Euglena gracilis photoreceptor

International Nuclear Information System (INIS)

Barsanti, Laura; Coltelli, Primo; Evangelista, Valtere; Passarelli, Vincenzo; Frassanito, Anna Maria; Vesentini, Nicoletta; Gualtieri, Paolo

2008-01-01

This paper deals with the first characterization of the structure of the photoreceptive organelle of the unicellular alga Euglena gracilis (Euglenophyta). This organelle has a three-dimensional organization consisting of up to 50 closely stacked membrane lamellae. Ionically induced unstacking of the photoreceptor lamellae revealed ordered arrays well suited to structural analysis by electron microscopy and image analysis, which ultimately yielded a low-resolution picture of the structure. Each lamella is formed by the photoreceptive membrane protein of the cell assembled within the membrane layer in a hexagonal lattice. The first order diffraction spots in the calculated Fourier transform reveals the presence of 6-fold symmetrized topography (better resolution about 90 A). The 2D and 3D structural data are very similar with those recently published on proteorodopsin, a membrane protein used by marine bacterio-plankton as light-driven proton pump. In our opinion these similarity indicate that a photoreceptive protein belonging to the same superfamily of proteorodopsin could form the Euglena photoreceptor

Functional photoreceptor loss revealed with adaptive optics: an alternate cause of color blindness.

Science.gov (United States)

Carroll, Joseph; Neitz, Maureen; Hofer, Heidi; Neitz, Jay; Williams, David R

2004-06-01

There is enormous variation in the X-linked L/M (long/middle wavelength sensitive) gene array underlying "normal" color vision in humans. This variability has been shown to underlie individual variation in color matching behavior. Recently, red-green color blindness has also been shown to be associated with distinctly different genotypes. This has opened the possibility that there may be important phenotypic differences within classically defined groups of color blind individuals. Here, adaptive optics retinal imaging has revealed a mechanism for producing dichromatic color vision in which the expression of a mutant cone photopigment gene leads to the loss of the entire corresponding class of cone photoreceptor cells. Previously, the theory that common forms of inherited color blindness could be caused by the loss of photoreceptor cells had been discounted. We confirm that remarkably, this loss of one-third of the cones does not impair any aspect of vision other than color.

Volumetric imaging of rod and cone photoreceptor structure with a combined adaptive optics-optical coherence tomography-scanning laser ophthalmoscope

Science.gov (United States)

Wells-Gray, Elaine M.; Choi, Stacey S.; Zawadzki, Robert J.; Finn, Susanna C.; Greiner, Cherry; Werner, John S.; Doble, Nathan

2018-03-01

We have designed and implemented a dual-mode adaptive optics (AO) imaging system that combines spectral domain optical coherence tomography (OCT) and scanning laser ophthalmoscopy (SLO) for in vivo imaging of the human retina. The system simultaneously acquires SLO frames and OCT B-scans at 60 Hz with an OCT volume acquisition time of 4.2 s. Transverse eye motion measured from the SLO is used to register the OCT B-scans to generate three-dimensional (3-D) volumes. Key optical design considerations include: minimizing system aberrations through the use of off-axis relay telescopes, conjugate pupil plane requirements, and the use of dichroic beam splitters to separate and recombine the OCT and SLO beams around the nonshared horizontal scanning mirrors. To demonstrate system performance, AO-OCT-SLO images and measurements are taken from three normal human subjects ranging in retinal eccentricity from the fovea out to 15-deg temporal and 20-deg superior. Also presented are en face OCT projections generated from the registered 3-D volumes. The ability to acquire high-resolution 3-D images of the human retina in the midperiphery and beyond has clinical importance in diseases, such as retinitis pigmentosa and cone-rod dystrophy.

Post-Disaster Image Processing for Damage Analysis Using GENESI-DR, WPS and Grid Computing

OpenAIRE

Bielski, Conrad; Gentilini, Simone; Pappalardo, Marco

2011-01-01

The goal of the two year Ground European Network for Earth Science Interoperations-Digital Repositories (GENESI-DR) project was to build an open and seamless access service to Earth science digital repositories for European and world-wide science users. In order to showcase GENESI-DR, one of the developed technology demonstrators focused on fast search, discovery, and access to remotely sensed imagery in the context of post-disaster building damage assessment. This paper descri...

Cleaning Genesis Mission Payload for Flight with Ultra-Pure Water and Assembly in ISO Class 4 Environment

Science.gov (United States)

Allton, Judith H.

2012-01-01

Genesis mission to capture and return to Earth solar wind samples had very stringent contamination control requirements in order to distinguish the solar atoms from terrestrial ones. Genesis mission goals were to measure solar composition for most of the periodic table, so great care was taken to avoid particulate contamination. Since the number 1 and 2 science goals were to determine the oxygen and nitrogen isotopic composition, organic contamination was minimized by tightly controlling offgassing. The total amount of solar material captured in two years is about 400 micrograms spread across one sq m. The contamination limit requirement for each of C, N, and O was <1015 atoms/sq cm. For carbon, this is equivalent to 10 ng/cm2. Extreme vigilance was used in pre-paring Genesis collectors and cleaning hardware for flight. Surface contamination on polished silicon wafers, measured in Genesis laboratory is approximately 10 ng/sq cm.

Control rod for a nuclear reactor

Science.gov (United States)

Roman, Walter G.; Sutton, Jr., Harry G.

1979-01-01

A control rod assembly for a nuclear reactor is disclosed having a remotely disengageable coupling between the control rod and the control rod drive shaft. The coupling is actuated by first lowering then raising the drive shaft. The described motion causes axial repositioning of a pin in a grooved rotatable cylinder, each being attached to different parts of the drive shaft which are axially movable relative to each other. In one embodiment, the relative axial motion of the parts of the drive shaft is used either to couple or to uncouple the connection by forcing resilient members attached to the drive shaft into or out of shouldered engagement, respectively, with an indentation formed in the control rod.

Control rod for a nuclear reactor

International Nuclear Information System (INIS)

Roman, W.G.; Sutton, H.G. Jr.

1976-01-01

A control rod assembly for a nuclear reactor is disclosed having a remotely disengageable coupling between the control rod and the control rod drive shaft. The coupling is actuated by first lowering then raising the drive shaft. The described motion causes axial repositioning of a pin in a grooved rotatable cylinder, each being attached to different parts of the drive shaft which are axially movable relative to each other. In one embodiment, the relative axial motion of the parts of the drive shaft is used either to couple or to uncouple the connection by forcing resilent members attached to the drive shaft into or out of shouldered engagement, respectively, with an indentation formed in the control rod

Control rod for a nuclear reactor

International Nuclear Information System (INIS)

Roman, W.G.; Sutton, H.G. Jr.

1979-01-01

A control rod assembly for a nuclear reactor is disclosed having a remotely disengageable coupling between the control rod and the control rod drive shaft. The coupling is actuated by first lowering then raising the drive shaft. The described motion causes axial repositioning of a pin in a grooved rotatable cylinder, each being attached to different parts of the drive shaft which are axially movable relative to each other. In one embodiment, the relative axial motion of the parts of the drive shaft is used either to couple or to uncouple the connection by forcing resilient members attached to the drive shaft into or out of shouldered engagement, respectively, with an indentation formed in the control rod

Synaptojanin 1 is required for endolysosomal trafficking of synaptic proteins in cone photoreceptor inner segments.

Directory of Open Access Journals (Sweden)

Ashley A George

Full Text Available Highly polarized cells such as photoreceptors require precise and efficient strategies for establishing and maintaining the proper subcellular distribution of proteins. The signals and molecular machinery that regulate trafficking and sorting of synaptic proteins within cone inner segments is mostly unknown. In this study, we show that the polyphosphoinositide phosphatase Synaptojanin 1 (SynJ1 is critical for this process. We used transgenic markers for trafficking pathways, electron microscopy, and immunocytochemistry to characterize trafficking defects in cones of the zebrafish mutant, nrc(a14 , which is deficient in phosphoinositide phosphatase, SynJ1. The outer segments and connecting cilia of nrc(a14 cone photoreceptors are normal, but RibeyeB and VAMP2/synaptobrevin, which normally localize to the synapse, accumulate in the nrc(a14 inner segment. The structure of the Endoplasmic Reticulum in nrc(a14 mutant cones is normal. Golgi develop normally, but later become disordered. Large vesicular structures accumulate within nrc(a14 cone photoreceptor inner segments, particularly after prolonged incubation in darkness. Cone inner segments of nrc (a14 mutants also have enlarged acidic vesicles, abnormal late endosomes, and a disruption in autophagy. This last pathway also appears exacerbated by darkness. Taken altogether, these findings show that SynJ1 is required in cones for normal endolysosomal trafficking of synaptic proteins.

ABWR-II Core Design with Spectral Shift Rods for Operation with All Control Rods Withdrawn

International Nuclear Information System (INIS)

Moriwaki, Masanao; Aoyama, Motoo; Anegawa, Takafumi; Okada, Hiroyuki; Sakurada, Koichi; Tanabe, Akira

2004-01-01

An innovative reactor core concept applying spectral shift rods (SSRs) is proposed to improve the plant economy and the operability of the 1700-MW(electric) Advanced Boiling Water Reactor II (ABWR-II). The SSR is a new type of water rod in which a water level is naturally developed during operation and changed according to the coolant flow rate through the channel. By taking advantage of the large size of the ABWR-II bundle, the enhanced spectral shift operation by eight SSRs allows operation of the ABWR-II with all control rods withdrawn. In addition, the uranium-saving factor of 6 to 7% relative to the reference ABWR-II core with conventional water rods can be expected due to the greater effect of spectral shift. The combination of these advantages means the ABWR-II with SSRs should be an attractive alternative for the next-generation nuclear reactor

Process and apparatus for controlling control rods

International Nuclear Information System (INIS)

Gebelin, B.; Couture, R.

1987-01-01

This process and apparatus is characterized by 2 methods, for examination of cluster of nuclear control rods. Foucault current analyzer which examines fraction by fraction all the control rods. This examination is made by rotation of the cluster. Doubtful rods are then analysed by ultrasonic probe [fr

Nursing perspective on the Genesis account of creation.

Science.gov (United States)

Geary, Heidi D; Cone, Pamela H

2012-01-01

Biblical accounts of creation tell the origin of the world and the beginning of God's relationship with humankind. Azusa Pacific University assigns graduate nursing students a faith integration project analyzing Creation accounts through the eyes of a nurse researcher. This report integrates eight students' investigations of Genesis 1 and 2, offering deep, rich insight into nursing practice.

Performance of the NRX shut-off rods

International Nuclear Information System (INIS)

Manson, R.E.

1965-08-01

A new type of shut-off rod of electromechanical design was developed by the American Machine and Foundry Company for use in the NRX reactor following the accident of 1952. The new rods were installed in May, 1956, as part of the control system conversion program which was completed in 1958. Some problems were encountered with limit switch adjustment but minor modifications in design led to much improved operation. he performance of the rods also improved as more experience was gained in the maintenance and adjustment of the various headgear components. Each headgear is now overhauled once a year on a routine basis. The present design of shut-off rod is considered to be very satisfactory. There has only been one occasion when a shut-off rod has failed to come fully down on a trip. Rods have failed to operate correctly on five other occasions but these occurred during shutdown periods or when the reactor was being shutdown manually. (author)

Control rod supporting device in reactor

International Nuclear Information System (INIS)

Seki, Osamu; Itooka, Satoshi; Harada, Kiyoshi; Jodoi, Takashi.

1990-01-01

Since coolants flowing from a reactor core hit against a control rod and a control rod connection pipe, a considerable amount of bending moment for separating an attracting surface between an electromagnet and an armature is formed. Then, a plurality of grooves are formed on a heat sensitive material to dispose a heat collecting fin, and each of upper and lower contact portions of a control rod supporting portion in which the flanged portion of T-like cross section does not slip out is made into a partial spheric surface and a portion between the electromagnet and the attracted member are engaged by the unevenness. With such a constitution, even if a bending moment is applied, the control rod only swings and the bending moment is not transmitted to the attracted member. Further, since the temperature of the heat sensitive material can be rapidly made closer to the peripheral temperature by using the heat collecting fin, the timing for separation is made accurate. Further, since the engaging portion is brought into contact at the spheric surface, the load distribution on the control rod is made uniform, and the positional relationship is made accurate, to support the control rod reliably and the separation depends only on the temperature of the coolants. (N.H.)

Molecular Contamination on Anodized Aluminum Components of the Genesis Science Canister

Science.gov (United States)

Burnett, D. S.; McNamara, K. M.; Jurewicz, A.; Woolum, D.

2005-01-01

Inspection of the interior of the Genesis science canister after recovery in Utah, and subsequently at JSC, revealed a darkening on the aluminum canister shield and other canister components. There has been no such observation of film contamination on the collector surfaces, and preliminary spectroscopic ellipsometry measurements support the theory that the films observed on the anodized aluminum components do not appear on the collectors to any significant extent. The Genesis Science Team has made an effort to characterize the thickness and composition of the brown stain and to determine if it is associated with molecular outgassing.Detailed examination of the surfaces within the Genesis science canister reveals that the brown contamination is observed to varying degrees, but only on surfaces exposed in space to the Sun and solar wind hydrogen. In addition, the materials affected are primarily composed of anodized aluminum. A sharp line separating the sun and shaded portion of the thermal closeout panel is shown. This piece was removed from a location near the gold foil collector within the canister. Future plans include a reassembly of the canister components to look for large-scale patterns of contamination within the canister to aid in revealing the root cause.