Biomedical subjects

H Ripps

Publications and source records attributed to H Ripps.

At least 19 recordsLinked to original sources

PubMed1992

Rhodopsin regeneration in the normal and in the detached/replaced retina of the skate.

The bleaching and regeneration of rhodopsin in the skate retina was studied by means of fundus reflectometry, both in the normal eyecup preparation and after the retina had been detached and then replaced on the surface of the pigment epithelium (RPE). After bleaching virtually all the rhodopsin in the retinal test area of the normal eyecup, more than 90% of the photopigment was reformed after about 2 hr in darkness; over most of this time course, rhodopsin density rose linearly at a rate of 0.875% min-1 with a half-time of 55 min. Detaching the retina from its pigment epithelium resulted in a number of abnormalities, both structural and functional. Histological examination of the detached/replaced (D/R) retina showed striking alterations in the structural integrity of the RPE cells at their interface with the neural retina. The cells appeared vacuolated and misshapen, and the apical processes of the RPE, which normally ensheath the receptor outer segments, were shredded and free of their association with the visual cells. These morphological changes, as well as dilution of the IRBP content of the subretinal space caused by separation of the tissues, appear to be the main factors contributing to the functional abnormalities in rhodopsin kinetics. But despite these abnormalities and the persistent detachment, the rate of regeneration and the amount of rhodopsin reformed after bleaching were reduced by less than 50% of their normal values. The fact that a significant fraction of the bleached rhodopsin was regenerated under these conditions indicates that 11-cis retinal formed in the RPE was able to traverse a much greater than normal subretinal space to reach the opsin-bearing photoreceptor membranes.

Animals↗

PubMed1992

Light-dependent delay in the falling phase of the retinal rod photoresponse.

Using suction electrodes, photocurrent responses to 100-ms saturating flashes were recorded from isolated retinal rods of the larval-stage tiger salamander (Ambystoma tigrinum). The delay period (Tc) that preceded recovery of the dark current by a criterion amount (3 pA) was analyzed in relation to the flash intensity (If), and to the corresponding fractional bleach (R*0/Rtot) of the visual pigment; R*0/Rtot was compared with R*s/Rtot, the fractional bleach at which the peak level of activated transducin approaches saturation. Over an approximately 8 ln unit range of I(f) that included the predicted value of R*s/Rtot, Tc increased linearly with ln I(f). Within the linear range, the slope of the function yielded an apparent exponential time constant (tau c) of 1.7 +/- 0.2 s (mean +/- S.D.). Background light reduced the value of Tc measured at a given flash intensity but preserved a range over which Tc increased linearly with ln I(f); the linear-range slope was similar to that measured in the absence of background light. The intensity dependence of Tc resembles that of a delay (Td) seen in light-scattering experiments on bovine retinas, which describes the period of essentially complete activation of transducin following a bright flash; the slope of the function relating Td and ln flash intensity is thought to reflect the lifetime of photoactivated visual pigment (R*) (Pepperberg et al., 1988; Kahlert et al., 1990). The present data suggest that the electrophysiological delay has a similar basis in the deactivation kinetics of R*, and that tau c represents TR*, the lifetime of R* in the phototransduction process. The results furthermore suggest a preservation of the "dark-adapted" value of TR* within the investigated range of background intensity.

Ambystoma↗

PubMed1992

Receptive field properties of rod-driven horizontal cells in the skate retina.

The large receptive fields of retinal horizontal cells result primarily from extensive intercellular coupling via gap (electrical) junctions; thus, the extent of the receptive field provides an index of the degree to which the cells are electrically coupled. For rod-driven horizontal cells in the dark-adapted skate retina, a space constant of 1.18 +/- 0.15 mm (SD) was obtained from measurements with a moving slit stimulus, and a comparable value (1.43 +/- 0.55 mm) was obtained with variation in spot diameter. These values, and the extensive spread of a fluorescent dye (Lucifer Yellow) from the site of injection to neighboring cells, indicate that the horizontal cells of the all-rod retina of skate are well coupled electrically. Neither the receptive field properties nor the gap-junctional features of skate horizontal cells were influenced by the adaptive state of the retina: (a) the receptive field organization was unaffected by light adaptation, (b) similar dye coupling was seen in both dark- and light-adapted retinae, and (c) no significant differences were found in the gap-junctional particle densities measured in dark- and light-adapted retinas, i.e., 3,184 +/- 286/microns 2 (n = 8) and 3,073 +/- 494/microns 2 (n = 11), respectively. Moreover, the receptive fields of skate horizontal cells were not altered by either dopamine, glycine, GABA, or the GABAA receptor antagonists bicuculline and picrotoxin. We conclude that the rod-driven horizontal cells of the skate retina are tightly coupled to one another, and that the coupling is not affected by photic and pharmacological conditions that are known to modulate intercellular coupling between cone-driven horizontal cells in other species.

Animals↗

PubMed1991

Antiretinal antibodies in serum of patients with age-related macular degeneration.

Antibodies, immunoreactive with normal human retinal proteins, were detected by Western immunoblot analysis in the sera of 30 patients with age-related macular degeneration (AMD). Sera from 14 of these patients demonstrated positive binding predominantly to a doublet protein of molecular weight between 58 and 62 kD. The sera from the remaining 16 patients and from 12 control subjects reacted either weakly or not at all with the doublet protein. No correlation was found with any specific type of AMD. The serum antibodies also immunocrossreacted with the same proteins from isolated photoreceptor outer segments; this was confirmed by indirect immunofluorescence on intact retinas. The crossreactivity of the serum antibodies with a protein of Mr 58 to 62 kD, the lower band present in the bovine purified neurofilament-68 kD preparation, suggests strongly that this protein may be a component of the neuronal cytoskeleton. However, it is not clear whether these autoantibodies play a direct role in the etiology of AMD or represent a nonspecific response to retinal damage.

Aged↗

PubMed1991

Ultrastructural and electrophysiological changes associated with K(+)-evoked release of neurotransmitter at the synaptic terminals of skate photoreceptors.

Bathing the skate retina in a Ringer solution containing a high concentration (100 mM) of potassium ions depolarized the visual cells, depleted the receptor terminals of synaptic vesicles, and suppressed completely the b-wave of the ERG and the intracellularly recorded response of horizontal cells (the S-potential). The depletion of synaptic vesicles was accompanied by a large increase in the extent of the plasma membrane resulting in distortion of the normal terminal profile, i.e. distension of the basal surface and elaborate infolding of protoplasmic extensions. Morphometric analysis showed that despite the changes in vesicle content and terminal structure, the combined linear extent of the vesicular and plasma membranes was unchanged from control (superfusion with normal Ringer solution); the increase in plasma membrane was equivalent to the observed loss of vesicular membrane. When returned to a normal Ringer solution, the terminals rapidly began to reform, and in about 10 min they were morphologically indistinguishable from receptor terminals seen in control preparations. After 30 min in the normal Ringer solution, the amount of membrane associated with the vesicles and the plasma membrane had reverted to control values, and once again the total membrane estimated morphometrically remained essentially the same. Thus, there is an efficient mechanism at the photoreceptor terminal for the recycling of vesicle membrane following exocytosis. The K(+)-induced depletion of synaptic vesicles was paralleled by a precipitous loss of responsivity in both the b-wave of the ERG and the S-potential of the horizontal cells. However, after 30-min exposure to the high K+ and a return to normal Ringer solution, the recovery of electrophysiological activity followed a much slower time course from that associated with the structural changes; 60 min or longer were required for the potentials to exhibit maximum response amplitudes. It appears that the rate-limiting step in restoring normal synaptic function following massive depletion of vesicular stores is transmitter resynthesis and vesicle loading rather than vesicle recycling.

Animals↗

PubMed1991

Functional properties of interphotoreceptor retinoid-binding protein.

It has been hypothesized that interphotoreceptor retinoid-binding protein (IRBP) functions as a two-way carrier of retinoid between the retinal pigment epithelium (RPE) and rod photoreceptors in the vertebrate eye. This hypothesis has been tested in recent studies that have employed purified, initially ligand-free, bovine IRBP and the "RPE-eyecup" obtained from the toad (Bufo marinus) eye. The present experiments further characterize the IRBP/RPE-eyecup system with respect to (i) the solubilization and protection of retinol by IRBP, and (ii) the time course of IRBP-mediated release of 11-cis retinal by the RPE. The data, together with previous findings in the IRBP/RPE-eyecup preparation, support the view that 11-cis retinal is the principal retinoid released by the RPE into IRBP-supplemented aqueous medium, and that IRBP in vivo promotes the regeneration of rhodopsin by facilitating the exchange of retinoid between bleached rods and the RPE.

Animals↗

PubMed1990

Structural features and adaptive properties of photoreceptors in the skate retina.

In the duplex (rod/cone) retinae of most vertebrates, electrical responses associated with nocturnal vision can be recorded in relative isolation only over a limited intensity range before there is encroachment by signals from the cone system; e.g., at mesopic levels of illumination, the rods begin to saturate, and the cone mechanism is brought into play. On the other hand, the retinae of some sub-species of skate appear to contain only one class of photoreceptors, namely rods. Nevertheless, skate photoreceptors, as well as other retinal neurons, are able to respond to incremental stimuli presented on background fields so intense that 90% or more of the available rhodopsin is bleached during the exposure. These and other findings raise some doubts as to whether the skate has, in fact, an all-rod retina. In this paper, we present a body of evidence--based on the results of photochemical, anatomical, and electrophysiological studies--to support the view that only a single class of photoreceptor subserves vision in the skate retina. In addition, recent findings will be described that: a) demonstrate how the visual cells transform from sluggishly responding rods to brisk, cone-like elements, and b) may provide some insight into the functional significance of this unusual adaptive property.

Adaptation, Ocular↗

PubMed1990

On the duplex nature of the skate retina.

The skate retina contains only one type of photoreceptor which has rod-like properties in the dark-adapted state. In the presence of background illumination, the receptors take on cone-like properties, i.e., their photoresponses become much faster, are less sensitive to light, and can be elicited in the presence of very bright backgrounds. Although the transformation is an extremely slow process, the skate retina performs like that of animals with mixed, rod-cone retinae. In this report we examine some postreceptoral features which may relate to this remarkable behavior. We show, for example, that the skate photoreceptor terminals make two kinds of junctions (ribbon synapses and basal junctions) that appear analogous to the two kinds of synaptic contacts made by cone terminals in other species. Furthermore, two types of horizontal cells are seen in the skate retina. Although the light-evoked responses of these horizontal cells are similar, there are differences in their current profiles recorded under voltage clamp, and in the nature of their dendritic processes. We have also observed several unusual postreceptoral structural features that may have some bearing on the response properties of the skate retina. In addition, a comparison of the adaptive properties of the receptor potential with those of intraretinal responses (b-wave, PNR) reveals differences that suggest strongly the presence of a network adaptive mechanism originating within the proximal retina. The network mechanism appears to be controlled to a large extent by the extracellular concentration of potassium [K+]0; i.e., changes in [K+]0 affect significantly the sensitivities of the b-wave and PBR, but have little effect on responses arising in the distal retina.

Adaptation, Ocular↗

PubMed1990

Interphotoreceptor retinoid-binding protein promotes rhodopsin regeneration in toad photoreceptors.

Interphotoreceptor retinoid-binding protein (IRBP) has been hypothesized to function as an intercellular shuttle in the vertebrate eye, serving to transport retinoids between the retinal pigment epithelium (RPE) and photoreceptors in the process by which visual pigment is regenerated after photolysis. This hypothesis was tested in preparations utilizing the toad (Bufo marinus) eye and purified, initially ligand-free IRBP obtained from the bovine eye. Rod outer segments (ROS) or neural retinas were isolated and bleached, then incubated with native RPE (RPE-eyecup) in the presence or absence of IRBP. The amount of rhodopsin present after incubation was determined by spectrophotometric analysis and compared with that in control preparations receiving bovine serum albumin or Ringer's solution only. Supplementation with IRBP enhanced the formation of rhodopsin in both the ROS/RPE-eyecup and retina/RPE-eyecup preparations. Regeneration in ROS/RPE-eyecups receiving IRBP (1.8 nmol) increased in a roughly linear manner with the period of incubation (0-4 hr), at a rate of 0.44 nmol/hr. The extent of regeneration was graded with the quantities of IRBP and opsin introduced into the RPE-eyecup. With increasing amounts of IRBP (up to 5.2 nmol) or of initially available opsin (up to 15.6 nmol), the amount of rhodopsin formed (3-hr incubation) approached the same plateau value, about 2.5 nmol. Analysis of IRBP-supplemented Ringer's solution incubated in the RPE-eyecup showed 11-cis-retinal to be virtually the only retinoid withdrawn from the RPE. With large quantities of IRBP (3.2-9.2 nmol), the amount of 11-cis-retinal (2.7 +/- 0.5 nmol) withdrawn from the RPE during a 3-hr incubation was similar to the plateau value of rhodopsin formed in the ROS/RPE-eyecup. No 11-cis-retinal was observed in albumin-supplemented Ringer's solution (0.4-11.2 nmol of bovine serum albumin) or in Ringer's alone after similar incubation in the RPE-eyecup. The results suggest that an IRBP-mediated transfer of 11-cis-retinal from the RPE to the rods supports rhodopsin regeneration in vivo.

Animals↗

PubMed1990

Effects of gamma-aminobutyric acid on skate retinal horizontal cells: evidence for an electrogenic uptake mechanism.

In the retinae of many vertebrates, there are classes of horizontal cell that probably utilize gamma-aminobutyric acid (GABA) as a neurotransmitter. As with other amino acid transmitter agents, the postsynaptic action of GABA is thought to be terminated by uptake into neurons and glia surrounding the release site. The present study examined whether an uptake system for GABA could be detected in isolated skate horizontal cells by means of electrophysiological methods. Pressure ejection of GABA onto voltage-clamped horizontal cells produced an inward current that showed no sign of desensitization regardless of the GABA concentration. The dose-response relationship followed simple Michaelis-Menten kinetics, with a half-maximal response elicited at approximately 110 microM. Nipecotic acid produced a similar current and reduced the responses to GABA when introduced in the bath solution prior to the GABA pulse. On the other hand, application of 500 microM muscimol or 1 mM baclofen, GABAA and GABAB receptor agonists, respectively, were completely without effect. The GABA-induced current was not blocked by superfusion with 500 microM bicuculline, 500 microM picrotoxin, or 500 microM phaclofen. However, the responses to GABA were abolished when the cells were superfused in Ringer's solution in which choline or lithium had been substituted for sodium, and were reduced when the extracellular chloride concentration was decreased from 266 mM to 16 mM. Current-voltage data showed a maximal response to GABA when the cells were held at or below their resting potential. At more depolarized levels, the inward current became progressively smaller until, near +50 mV, it could no longer be detected; over the range tested (-90 to +50 mV), the response never reversed into an outward current. These findings suggest that the GABA-induced currents in skate horizontal cells are mediated by an electrogenic uptake mechanism.

Animals↗

PubMed1990

Structural and functional properties of two types of horizontal cell in the skate retina.

Two morphologically distinct types of horizontal cell have been identified in the all-rod skate retina by light- and electron-microscopy as well as after isolation by enzymatic dissociation. The external horizontal cell is more distally positioned in the retina and has a much larger cell body than does the internal horizontal cell. However, both external and internal horizontal cells extend processes to the photoreceptor terminals where they end as lateral elements adjacent to the synaptic ribbons within the terminal invaginations. Whole-cell voltage-clamp studies on isolated cells similar in appearance to those seen in situ showed that both types displayed five separate voltage-sensitive conductances: a TTX-sensitive sodium conductance, a calcium current, and three potassium-mediated conductances (an anomalous rectifier, a transient outward current resembling an A current, and a delayed rectifier). There was, however, a striking difference between external and internal horizontal cells in the magnitude of the current carried by the anomalous rectifier. Even after compensating for differences in the surface areas of the two cell types, the sustained inward current elicited by hyperpolarizing voltage steps was a significantly greater component of the current profile of external horizontal cells. A difference between external and internal horizontal cells was seen also in the magnitudes of their TEA-sensitive currents; larger currents were usually obtained in recordings from internal horizontal cells. However, the currents through these K+ channels were quite small, the TEA block was often judged to be incomplete, and except for depolarizing potentials greater than or equal to +20 mV (i.e., outside the normal operating range of horizontal cells), this current did not provide a reliable indicator of cell type. The fact that two classes of horizontal cell can be distinguished by their electrophysiological responses, as well as by their morphological appearance and spatial distribution in the retina, suggests that they may play different roles in the processing of visual information within the retina.

Animals↗

PubMed1989

Vincristine-induced changes in the retina of the isolated arterially-perfused cat eye.

We have attempted to determine in this study whether the arterial administration of vincristine produces in cat the functional defects associated with hereditary and vincristine-induced night blindness in man. Using the isolated perfused cat eye, it has been possible to mimic some of the essential features of human night blindness, namely, retention of normal rhodopsin chemistry and normal photoreceptor activity, with marked suppression of the ERG b-wave. In addition, we find that vincristine produces an early, rapid fall in the c-wave, a potential that arises largely in the pigment epithelium. Ultrastructurally, it appears that many classes of retinal neuron are affected by the drug, but the principal changes in cytoarchitecture are seen in the photoreceptors. Except for the outer segments, paracrystalline deposits were found in all parts of the visual cell. The disruption of the normal microtubular organization of these cells suggests that the drug interferes with the functional integrity of the transport system by which synaptic activity is maintained. By reducing the efficacy of communication between visual cells and their second-order neurons, the electrical responses of post-synaptic elements is degraded. The route by which vinca alkaloids reach the neural retina is still uncertain, but our preliminary studies using HRP indicate that the relatively high concentration of vincristine used in this study may be responsible for compromising the blood-retinal barrier.

Animals↗

PubMed1989

Interphotoreceptor retinoid-binding protein: role in delivery of retinol to the pigment epithelium.

The ability of interphotoreceptor retinoid-binding protein (IRBP) to facilitate the incorporation of retinol into retinyl esters by the retinal pigment epithelium (RPE) was examined in toad (Bufo marinus) eyecup preparations devoid of neural retina (RPE-eyecup). Solutions containing purified bovine IRBP and all-trans[3H]retinol were introduced into the vitreal cavity of the RPE-eyecup. After incubation at 22 degrees C, [3H]retinyl ester was extracted from the RPE cells and isolated by high performance liquid chromatography. All-trans[3H]retinyl ester formed in the RPE increased with time of incubation (up to 2 hr) and with concentration of IRBP (up to 10 microM). The increase with IRBP concentration accompanied, and presumably resulted from, an increased transfer of [3H]retinol to the RPE-eyecup. With higher concentration of IRBP (20-30 microM), both the amount of [3H]retinyl ester formed (relative to the peak value at 10 microM IRBP) and the overall molar content of endogenous retinyl ester were reduced. On the other hand, bovine serum albumin at relatively high concentration (90 microM) was less effective than 3 microM IRBP in supporting the formation of [3H]retinyl ester, and it did not reduce the level of native retinyl ester in the RPE. Using 3 microM IRBP, levels of [3H]retinyl ester formed were comparable to or exceeded those obtained with phosphatidyl choline (0.9 mg ml-1) or serum retinol-binding protein (3 microM). The data are consistent with the hypothesized role of IRBP as a carrier of retinol between the retina and RPE in the operation of the visual cycle.

Animals↗

PubMed1989

gamma-Aminobutyric acid (GABA)-induced currents of skate Muller (glial) cells are mediated by neuronal-like GABAA receptors.

Radial glia (Muller cells) of the vertebrate retina appear to be intimately involved in regulating the actions of amino acid neurotransmitters. One of the amino acids thought to be important in mediating retinal information flow is gamma-aminobutyric acid (GABA). The findings of this study indicate that enzymatically isolated skate Muller cells are depolarized by GABA and the GABAA agonist muscimol and that the actions of these agents are reduced by bicuculline and picrotoxin. Membrane currents induced by GABA under voltage clamp were dose dependent, were associated with an increase in membrane conductance, and showed marked desensitization when the concentration of GABA exceeded 2.5 microM. The responses had a reversal potential close to that calculated for chloride, indicating that the currents were generated by ions passing through channels. These data support the view that skate Muller cells possess functional GABAA receptors. The presence of such receptors on retinal glia may have important implications for the role of Muller cells in maintaining the constancy of the extracellular milieu, for neuron-glia interactions within the retina, and for theories concerning the generation of the electroretinogram.

Animals↗

PubMed1989

Membrane current responses of skate photoreceptors.

Light-evoked membrane currents were recorded with suction electrodes from the outer segments of individual photoreceptors enzymatically dissociated from the skate retina. The intensity-response relation of dark-adapted cells closely followed a Michaelis function for which a half-saturating response was elicited by a flash intensity that produced about 36 photoisomerizations. Dim-light responses, as well as the early rising phase of the responses to a wide range of flash intensities, could be described by a reaction scheme that involved a series of four first-order delay stages. The number of delay stages required to model the rising phase of the photocurrents did not change in light adaptation. However, background illumination that reduced sensitivity by 1.5 log units, or a bleaching exposure that resulted in a nearly equivalent desensitization, shortened significantly the time scale of the responses. In both instances there were two- to threefold increases in the rate constants of the transitional delays, and almost complete suppression of the tail current that characterized the response of the dark-adapted cell. These findings suggest that although light adaptation alters the gain and kinetics of the transduction mechanism, the nature of the intervening processes is the same in dark- and light-adapted photoreceptors. Moreover, the results show clearly that there is no need to postulate the existence of a second class of cone-like rods to account for the remarkable ability of skate photoreceptors to respond to incremental stimuli presented on "saturating" background fields or after exposure to an intense bleaching light.

Action Potentials↗

PubMed1988

Dynamics of skate horizontal cells.

The all-rod retina of the skate (Raja erinacea or R. oscellata) is known to have the remarkable capability of responding to incremental flashes superimposed on background intensities that initially block all light-evoked responses and are well above the level at which rods saturate in mixed rod/cone retinas. To examine further the unusual properties of the skate visual system, we have analyzed responses of their horizontal cells to intensity-modulated step, sinusoidal, and white-noise stimuli. We found that during exposures to mean intensities bright enough to block responses to incremental stimuli, decremental stimuli were also initially blocked. Thereafter, the horizontal cells underwent a slow recovery phase during which there was marked nonlinearity in their response properties. The cell first (within 2-3 min) responded to decrements in intensity and later (after greater than 10 min) became responsive to incremental stimuli. After adaptation to a steady state, however, the responses to intensity modulation were nearly linear over a broad range of modulation depths even at the brightest mean levels of illumination. Indeed, examination of the steady-state responses over a 5-log-unit range of mean intensities revealed that the amplitude of the white noise-evoked responses depended solely on contrast, and was independent of the retinal irradiance as the latter was increased from 0.02 to 20 muW/cm2; i.e., contrast sensitivity remained unchanged over this 1,000-fold increase in mean irradiance. A decrement from the mean as brief as 2 s, however, disturbed the steady state. Another unexpected finding in this all-rod retina concerns surround-enhancement, a phenomenon observed previously for cone-mediated responses of horizontal cells in the retinas of turtle and catfish. While exposure to annular illumination induced response compression and a pronounced sensitivity loss in response to incremental light flashes delivered to the dark central region, the cell's sensitivity showed a significant increase when tested with a white noise or sinusoidally modulated central spot. Unlike horizontal cells in other retinas studied thus far, however, response dynamics remained unchanged. Responses evoked either by a small spot (0.25-mm diam) or by a large field light covering the entire retina were almost identical in time course. This is in contrast with past findings from cone-driven horizontal cells whose response waveform (dynamics) was dependent upon the size of the retinal area stimulated.

Animals↗

PubMed1987

Photoreceptor processes in visual adaptation.

In this paper we have stressed two experimental results in need of explanation: (i) the reduced efficacy with which (remaining, abundant) rhodopsin in the light-adapted receptor mediates the flash response; and (ii) the disparity in conditions of irradiation (weak background vs. extensive bleaching) leading to equivalent conditions of threshold. The model discussed above suggests, in molecular terms, a possible basis for both properties of receptor adaptation. On the view developed here, property (i) derives from the ability of photoactivated or bleached pigment (R or B) to restrict dramatically the availability of a substance required for phototransduction. Property (ii) derives in large part from the pronounced disparity in the effectiveness of R (during illumination) and B (remaining after illumination) in reducing the availability of this substance. On this view, the "equivalence" of threshold elevation in states of light- vs. dark-adaptation derives from an overall equality of a product of factors (Q, Etot/Es, and J of equation 2). Under all but extreme conditions, this aggregate of factors is dominated by the term Etot/Es, reflecting the functional state of E.

Animals↗

PubMed1987

Progressive cone dystrophy.

Psychophysical, reflectometric, and electrophysiologic studies were done on four members of a dominant pedigree with progressive cone dystrophy. The two youngest individuals were asymptomatic at the initial examination, and none of the subjects complained of problems associated with night vision. Nevertheless, absent or grossly reduced cone-mediated electroretinographic (ERG) responses showed the widespread loss of cone function, and moderate elevations (less than 1 log unit) in absolute threshold together with reductions in rhodopsin levels in the mid-peripheral retina provided evidence of impairment of the rod system. The progressive nature of the disease was apparent from the case histories and the changes in visual performance that occurred on re-test after a 5-year interval. Moreover, the results of increment threshold measurements at several retinal loci suggested that peripheral cones may be affected earlier and more severely than those in the central retina.

Adolescent↗