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The electroretinographic diagnosis of the incomplete form of congenital stationary night blindness.

Fifteen patients with the incomplete form of congenital stationary night blindness (iCSNB) were reviewed to better characterize their electroretinographic (ERG) findings in view of differential diagnosis with other retinal conditions also presenting with negative bright-flash ERG responses. In all 15 patients, in dark-adapted conditions, the bright-flash ERG response had a normal a-wave followed by a subnormal b-wave. Oscillatory potentials (OPs) observed on the ascending limb of the b-wave, although delayed in implicit time, were of large amplitude. The response to a long-wavelength stimulus showed cone-related components and some well-delineated OPs. On the other hand, the photopically elicited cone responses were strongly abnormal, with a subnormal a-wave followed by a barely recordable b-wave. No OPs could be elicited under photopic conditions. The cone related components and the OP characteristics clearly distinguish iCSNB from the complete form of CSNB and other retinal conditions presenting with minimal fundus abnormalities but with negative bright-flash ERG responses, such as found in Duchenne muscular dystrophy and Aland Island eye disease. The severely abnormal post-synaptic components in the photopic recordings contrast with the well-differentiated cone activity evoked in scotopic conditions. We propose a cone system that does not respond optimally under the normal operating range (photopic) but rather under mesopic or scotopic conditions. In spite of the severe cone-ERG deficits, visual acuity was only slightly reduced. We propose that the defect, which interferes marginally with the neuronal flow of information, lies in the structures responsible for the building of the b-wave.

Adaptation, Ocular↗

Signal transmission from cones to amacrine cells in dark- and light-adapted tiger salamander retina.

Amacrine cells (ACs) are third-order interneurons in the retina that mediate antagonistic surround inputs to retinal ganglion cells and motion-related signals in the inner retina. Previous studies have revealed that rod-to-AC signals in dark-adapted retina are mediated by a nonlinear high-gain synaptic pathway. In this study, we investigated how cone signals are transmitted to ACs under dark- and light-adapted conditions. By using the spectral subtraction method, we found that the voltage gain of the cone-AC synaptic pathway in dark-adapted salamander retina (GD) is between 28 and 72, which is about one order of magnitude lower than the voltage gain of the rod-AC pathway. This suggests that, in darkness, rod signals are more efficiently transmitted to the ACs than cone signals. The voltage gain of the cone-AC synaptic pathway in the presence of 500 nm/-2.4 background light, GL, ranges between 28 and 56. Linear regression analysis indicates that GD and GL are strongly, positively, and linearly correlated. The average GL/GD ratio is 0.73, suggesting that, on average, GL in any given AC is about 73% of GD. This adaptation-induced change in cone-AC voltage gain exemplifies use-dependent modulations of synaptic transmission in the retina, and possible mechanisms underlying light-mediated alterations of retinal synaptic function are discussed.

Adaptation, Ocular↗

Arrestin migrates in photoreceptors in response to light: a study of arrestin localization using an arrestin-GFP fusion protein in transgenic frogs.

Subcellular translocation of phototransduction proteins in response to light has previously been detected by immunocytochemistry. This movement is consistent with the hypothesis that migration is part of a basic cellular mechanism regulating photoreceptor sensitivity. In order to monitor the putative migration of arrestin in response to light, we expressed a functional fusion between the signal transduction protein arrestin and green fluorescent protein (GFP) in rod photoreceptors of transgenic Xenopus laevis. In addition to confirming reports that arrestin is translocated, this alternative approach generated unique observations, raising new questions regarding the nature and time scale of migration. Confocal fluorescence microscopy was performed on fixed frozen retinal sections from tadpoles exposed to three different lighting conditions. A consistent pattern of localization emerged in each case. During early light exposure, arrestin-GFP levels diminished in the inner segments (ISs) and simultaneously increased in the outer segments (OSs), initially at the base and eventually at the distal tips as time progressed. Arrestin-GFP reached the distal tips of the photoreceptors by 45-75 min at which time the ratio of arrestin-GFP fluorescence in the OSs compared to the ISs was maximal. When dark-adaptation was initiated after 45 min of light exposure, arrestin-GFP rapidly re-localized to the ISs and axoneme within 30 min. Curiously, prolonged periods of light exposure also resulted in re-localization of arrestin-GFP. Between 150 and 240 min of light adaptation the arrestin-GFP in the ROS gradually declined until the pattern of arrestin-GFP localization was indistinguishable from that of dark-adapted photoreceptors. This distribution pattern was observed over a wide range of lighting intensity (25-2700 lux). Immunocytochemical analysis of arrestin in wild-type Xenopus retinas gave similar results.

Adaptation, Ocular↗

Absolute and spectral sensitivities in dark- and light-adapted Pagothenia borchgrevinki, an Antarctic nototheniid fish.

Functional properties of the retina of Pagothenia borchgrevinki, an Antarctic nototheniid fish that lives beneath the 2.5-3 m thick sea-ice in water of -1.8 degrees C temperature, were analyzed electrophysiologically at Scott Base (77 degrees 50'S; 166 degrees 45'E). The waveform of the ERG was monophasic in the dark-adapted state and showed an off-response of opposite polarity in the light-adapted condition. Responses of the light-adapted retina were smaller than those of the dark-adapted eye, although both photopic and scotopic components were observed. Spectral sensitivity measured by monochromatic photostimulation at 14 different wavelengths across the 400-700-nm range showed a single maximum at 490 nm. The spectral sensitivity curve is consistent with a rhodopsin photopigment. The dark-adapted retina exhibited a photon flux density threshold of approximately 2 x 10(9) photons cm-2 s-1) when monochromatic flashes of 500 nm wavelength and 250 ms duration were used. When the stimulus consisted of 1 s white light, a minimum energy flux density of approx. 2 x 10(-4) microW/cm2 was necessary to elicit a detectable response. It was concluded that the visual system of P. borchgrevinki was in tune with the dominant downwelling spectral irradiance and that, due to retinal thermal noise reduction in the cold environment, no great need for particular anatomical adaptations to further enhance sensitivity existed.

Acclimatization↗

Ontogeny of a photic response in the retina and suprachiasmatic nucleus in the mouse.

The ontogeny of photic responsiveness in the retina and the suprachiasmatic nucleus (SCN) of C57BL/6J mouse was studied using the enhanced expression of the immediate early gene c-fos as a marker of neuronal activation. c-fos expression was assessed by immunocytochemical localisation of its protein product. Light induction of Fos-like protein in the retina and SCN occurred first at postnatal day four (PD 4). At this stage of development, some cells in the inner part of the neuroblastic layer and in the ganglion cell layer showed positive immunoreaction; the number of Fos-like positive cells increased with age until it reached adult levels by PD 15. Induction of Fos-like expression at PD 4 in the SCN mainly occurred in the ventrolateral region, the region that receives the greatest density of retinal innervation. These results indicate that retinal input can activate cells in the SCN even before eyelids open, and the SCN can be stimulated by photic inputs as early as day 4 after birth.

Adaptation, Ocular↗

Absolute and relative sensitivity of the scotopic system of rat: electroretinography and behavior.

The goal of the present study was to relate the dark and light-adapted flash sensitivity of the scotopic threshold response (STR) and rod b-wave of the electroretinogram (ERG) to behaviorally measured rod increment threshold responses. Small amplitudes of the dark-adapted STR and b-wave, the latter after application of NMDA, were found to increase in proportion to flash intensity. The value obtained for the sensitivity of the b-wave would be expected if signals from rods were summed linearly by the rod bipolar cell. The sensitivity of the STR could not be accounted for in terms of rod signal convergence as the source of this ERG component is still unknown. Increment threshold responses of rats were measured behaviorally in an operant conditioning chamber. At absolute threshold, on average I in 2400 rods were activated by the test flash. Comparison of the adaptive effects of background lights on behaviorally measured scotopic sensitivity and rod ERG sensitivity suggest that the increment threshold sensitivity of rat is regulated at three different sites in the retina.

Adaptation, Ocular↗

Comparison of scotopic sensitivity in diurnal (Anas platyrhynchos) and crepuscular (Dendrocygna autumnalis) ducks.

Scotopic visual adaptation curves were obtained from 4 mallard ducks. A curve of best fit was used to compare the mallards' mean adaptation curve to the curve previously reported for the black-bellied tree duck, a crepuscular species. The curves did not differ significantly in either their slopes or base levels (thresholds). The mallards curve had a rod-cone "break" at approximately 25 min. This break is evident in the scotopic curve for pigeons, but is absent from the black-bellied tree ducks' curve. Examination of retinal tissues indicated that the black-bellied tree ducks had significantly more rods and cones, and a larger rod:cone ratio than the mallards. The mallards' scotopic visual threshold is exceeded by the natural illumination present under several nocturnal conditions.

Adaptation, Ocular↗

Transducin activation state controls its light-dependent translocation in rod photoreceptors.

Light-dependent redistribution of transducin between the rod outer segments (OS) and other photoreceptor compartments including the inner segments (IS) and synaptic terminals (ST) is recognized as a critical contributing factor to light and dark adaptation. The mechanisms of light-induced transducin translocation to the IS/ST and its return to the OS during dark adaptation are not well understood. We have probed these mechanisms by examining light-dependent localizations of the transducin-alpha subunit (Gtalpha)in mice lacking the photoreceptor GAP-protein RGS9, or expressing the GTPase-deficient mutant GtalphaQ200L. An illumination threshold for the Gtalpha movement out of the OS is lower in the RGS9 knockout mice, indicating that the fast inactivation of transducin in the wild-type mice limits its translocation to the IS/ST. Transgenic GtalphaQ200L mice have significantly diminished levels of proteins involved in cGMP metabolism in rods, most notably the PDE6 catalytic subunits, and severely reduced sensitivity to light. Similarly to the native Gtalpha, the GtalphaQ200L mutant is localized to the IS/ST compartment in light-adapted transgenic mice. However, the return of GtalphaQ200L to the OS during dark adaptation is markedly slower than normal. Thus, the light-dependent translocations of transducin are controlled by the GTP-hydrolysis on Gtalpha, and apparently, do not require Gtalpha interaction with RGS9 and PDE6.

Adaptation, Ocular↗

Cell cycle time and rate of entry of cells into mitosis in the small intestine of young rats.

Cell cycle time (T(C)) and the rate of entry of cells into mitosis (r(M)) in the jejunum and duodenum of young rats were investigated using the stathmokinetic method. The cell cycle times in the jejunum were 24.3 and 28.3 h in light and dark periods, respectively. Cell cycle times in the duodenum were 17.1 and 21.5 h in light and dark periods, respectively. Rates of entry of cells into mitosis in the jejunum were 1.2 and 1.1 cells/cell/h in light and dark periods and rates of entry of cells into mitosis in the duodenum were 1.4 and 1.8 cells/cell/h in light and dark periods, respectively. Although these changes to cell cycle time values are not statistically significant, the variation between the two periods should be considered in relation to its possible biological effects.

Adaptation, Ocular↗

Neurovisual adaptations to subnormal vision in children.

Neurovisual adaptations to subnormal vision in children are of practical importance and need to be remembered in the management of children with low vision. The physiological adaptations discussed include shortening the focal distance, abnormal head positions, compensatory head movements, and ocular motor search strategies. In addition the concept of neural plasticity, particularly in respect to the ability of the extrageniculate striaete system to assume primary visual function in cortically damaged children is discussed.

Accommodation, Ocular↗

Effect of light-adaptation on the binding of 48-kDa protein (S-antigen) to photoreceptor cell membranes.

During the process of light-adaptation, a part of retinal S-antigen ("48-kDa protein") is bound to the photoreceptor cell membranes. This fraction can be isolated by first extracting the soluble S-antigen with isotonic buffer and subsequently extracting the bound S-antigen with detergent. In this way we found that light-adaptation to 250 lx or more induces a maximum binding of 62% of total S-antigen within 2 minutes in rat retina in vivo. At low light intensity (50 lx) this process lasts 15 minutes, while at 5 lx only 30% of S-antigen is bound. Presumably the number of available phosphorylated (bleached) rhodopsin molecules is the limiting factor in time and quantity. Dark-adaptation causes an initial rapid release of S-antigen during the first 5 minutes, but it takes more than 2 hours to reach the minimum level of about 10% bound S-antigen. The rates of binding of S-antigen in the light and of release of S-antigen in the dark are compared to other phenomena of light and dark adaptation.

Adaptation, Ocular↗