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At least 649 records · Page 36Linked to original sources
PubMed1996

Sites of disease action in a retinal dystrophy with supernormal and delayed rod electroretinogram b-waves.

Delayed rod ERG b-waves in patients with an unusual retinal dystrophy have been attributed by some to an abnormality in receptor cGMP activity. Here the sites of disease action are studied by analyzing rod and cone ERGs using new analytical methods and a wide range of stimulus intensities. Consistent with previous reports, the five patients studied showed rod b-waves that were normal or supernormal in amplitude in response to intense flashes, but smaller than normal and markedly delayed in response to weaker flashes. The cone ERGs, recorded to 29 Hz flicker and to flashes upon a background, were smaller than normal and also showed delays. Models of phototransduction fitted to rod and cone a-waves indicated that the delays in the rod and cone b-waves were not due to the speed or amplification of the transduction process. An analysis of the derived inner nuclear layer (INL) response suggests that the sites of disease action are beyond the outer segment and involve a delay in the activation of INL activity.

Adolescent↗
PubMed2005

Extremely rapid recovery of human cone circulating current at the extinction of bleaching exposures.

We used a conductive fibre electrode placed in the lower conjunctival sac to record the a-wave of the human photopic electroretinogram elicited by bright white flashes, delivered during, or at different times after, exposure of the eye to bright white illumination that bleached a large fraction (approximately 90%) of the cone photopigment. During steady-state exposures of this intensity, the amplitude of the bright-flash response declined to approximately 50% of its dark-adapted level. After the intense background was turned off, the amplitude of the bright-flash response recovered substantially, for flashes presented within 20 ms of background extinction, and fully, for flashes presented 100 ms after extinction. In addition, a prominent 'background-off a-wave' was observed, beginning within 5-10 ms of background extinction. We interpret these results to show, firstly, that human cones are able to preserve around half of their circulating current during steady-state illumination that bleaches 90% of their pigment and, secondly, that following extinction of such illumination, the cone circulating current is restored within a few tens of milliseconds. This behaviour is in stark contrast to that in human rods, where the circulating current is obliterated by a background that bleaches only a few percent of the pigment, and where full recovery following a large bleach takes at least 20 min, some 50,000 times more slowly than shown here for human cones.

Action Potentials↗
PubMed2004

Contribution of rod and cone pathways to the dark-adapted electroretinogram (ERG) b-wave following retinal degeneration in RCS rats.

Although the RCS rat is widely used as a model of progressive photoreceptor loss, it is unclear how the relative rod and cone functions change with age. Rod and cone b-waves were isolated using a double flash ERG paradigm. In contrast to cones, rods never reached normal functional maturity levels, and the ERG b-wave changed from being predominantly rod-driven to being purely cone-driven by age 74 days, at which point, b-waves were progressively replaced by negative STR-like (scotopic threshold response) waves that persisted up to age 180 days. A double flash commonly abolished this wave and unveiled a b-wave.

Aging↗
PubMed1995

Mild retinal changes in a 47-year-old patient with phytanic acid storage disease.

A 47-year-old man complained of poor near vision. He had chronic polyneuropathy, bilateral shortening of the proximal phalanges, and ichthyosis. His serum phytanic acid level was merkedly elevated. The fundus and fluorescein angiograms showed diffuse fine granular changes throughout the retina. However, no pigmentary retinal degeneration, vessel attenuation, or disk atrophy was found. The visual field and dark adaptation results were normal. The electroretinogram demonstrated mildly subnormal rod responses with normal implicit times and cone responses within the normal range. The S-cone electroretinogram showed decreased amplitude and normal implicit time. These ophthalmological findings were not consistent with classical retinitis pigmentosa. We believe that this patient may have an uncommon type of adult Refsum's disease.

Atrophy↗
PubMed1999

Visual pigments, cone oil droplets, ocular media and predicted spectral sensitivity in the domestic turkey (Meleagris gallopavo).

A microspectrophotometric survey conducted on the retinal photoreceptors of the domestic turkey (Meleagris gallopavo) revealed the presence of five different types of vitamin A1-based visual pigment (rhodopsin) in seven different types of photoreceptor. A single class of rod contained a medium wavelength-sensitive visual pigment (wavelength of maximum absorbance, lambda max, 504 nm). Four different types of single cone contained visual pigment maximally sensitive to wavelengths in either the long (LWS, lambda max 564 nm), medium (MWS, lambda max 505 nm), short (SWS, lambda max 460 nm) or violet (VS, lambda max 420 nm) spectral ranges. The LWS, MWS and SWS single cones contained pigmented oil droplets with cut-off wavelengths (lambda cut) at 514, 490 and 437 nm, respectively. The VS single cone contained a transparent oil droplet which displayed no significant absorbance above 330 nm. A single class of double cone was also identified, both the principal and accessory members of which contained the LWS cone visual pigment. The principal member contained an oil droplet with a lambda cut at 436 nm. No oil droplet was observed in the accessory member. The use of a glycerol-based cell mountant, which reduced wavelength dependent measurement artefacts in the microspectrophotometric measurements, is described. Predictions of cone effective spectral sensitivity, incorporating measurements of the spectral transmission of the ocular media, suggest that turkeys have considerable sensitivity to wavelengths in the ultraviolet-A (UV-A, 315-400 nm) spectral range. This has implications for both the visual ecology of wild birds and the welfare of intensively farmed individuals.

Animals↗
PubMed1994

Visual pigments and the photic environment: the cottoid fish of Lake Baikal.

The endemic cottoid fish of Lake Baikal in Eastern Siberia offer a singular opportunity for examining within a number of closely related species, the relationships of visual pigments, photoreceptor complements and depth within a deep freshwater environment. The lake, the deepest (1600 m) and one of the largest and most ancient in the world, is unique in that the oxygen levels at the bottom are only reduced to about 80% of the surface levels. We have studied, by light microscopy, microspectrophotometry and visual pigment extraction, the retinas from 17 species of Baikal cottoids that live at different depths within the lake. Generally the retinas contain, in addition to rods, large green-sensitive double cones and small blue-sensitive single cones: surprisingly for freshwater fish, the visual pigments are based on Vitamin A1. The lambda max of both rods and cones are displaced to shorter wavelengths with increasing depth. Surface species have cones with lambda max at about 546, 525 and 450 nm and rods at 523 nm, deeper living species retain cones, but with lambda max shifting towards 500 and 425 nm and with rods at 480 nm, whereas the deepest living fish possess only rods (lambda max 480-500 nm). These data clearly show a correlation between photoreceptor complement, visual pigment lambda max and depth, but question the hypothesis that there is a correlation of pigment lambda max with water colour since, in contrast to oceanic waters, the maximum transmission of Baikal water is between 550 and 600 nm.

Animals↗
PubMed2005

A detailed study of the phenotype of an autosomal dominant cone-rod dystrophy (CORD7) associated with mutation in the gene for RIM1.

AIM: To characterise the phenotype of an autosomal dominant cone-rod dystrophy (CORD7) associated with the Arg844His mutation in RIM1. METHODS: Eight members of a four generation, non-consanguineous British family were examined clinically and underwent electrophysiological testing, automated dark adapted perimetry, dark adaptometry, colour vision assessment, colour fundus photography, fundus fluorescein angiography (FFA), and fundus autofluorescence (AF) imaging. RESULTS: The majority of affected individuals described a progressive deterioration of central vision, night vision, and peripheral visual field usually between the third and fourth decades. The visual acuity ranged from 6/6 to 3/60. Colour vision testing showed mild to moderate dyschromatopsia in the majority of individuals. Fundus changes comprised a range of macular appearances varying from mild retinal pigment epithelial (RPE) disturbance to extensive atrophy and pigmentation. In some individuals retinal vessels were attenuated and in two subjects peripheral areas of retinal atrophy were present. An absent or severely reduced PERG was detected in all subjects, indicative of marked macular dysfunction. Full field ERG showed abnormal rod and cone responses. AF imaging revealed decreased macular AF centrally surrounded by a ring of increased AF in the majority of individuals. "Bull's eye" lesions were present in two individuals, comprising of a ring of decreased perifoveal AF bordered peripherally and centrally by increased AF. Photopic sensitivity testing demonstrated elevated central visual field thresholds with additional superior greater than inferior peripheral field loss. There were rod and cone sensitivity reductions in the central and peripheral visual fields, with the inferior retina being more affected than the superior. CONCLUSIONS: The detailed phenotype is described of the autosomal dominant cone-rod dystrophy, CORD7, which is associated with a point mutation in RIM1, a gene encoding a photoreceptor synaptic protein. The pattern of disease progression and long term visual outcome facilitates improved genetic counselling and advice on prognosis. Such phenotypic data will be invaluable in the event of future therapy.

Adolescent↗
PubMed2002

Calcium regulation in photoreceptors.

In this review we describe some of the remarkable and intricate mechanisms through which the calcium ion (Ca2+) contributes to detection, transduction and synaptic transfer of light stimuli in rod and cone photoreceptors. The function of Ca2+ is highly compartmentalized. In the outer segment, Ca2+ controls photoreceptor light adaptation by independently adjusting the gain of phototransduction at several stages in the transduction chain. In the inner segment and synaptic terminal, Ca2+ regulates cells' metabolism, glutamate release, cytoskeletal dynamics, gene expression and cell death. We discuss the mechanisms of Ca2+ entry, buffering, sequestration, release from internal stores and Ca2+ extrusion from both outer and inner segments, showing that these two compartments have little in common with respect to Ca2+ homeostasis. We also investigate the various roles played by Ca2+ as an integrator of intracellular signaling pathways, and emphasize the central role played by Ca2+ as a second messenger in neuromodulation of photoreceptor signaling by extracellular ligands such as dopamine, adenosine and somatostatin. Finally, we review the intimate link between dysfunction in photoreceptor Ca2+ homeostasis and pathologies leading to retinal dysfunction and blindness.

Adaptation, Physiological↗
PubMed1996

[ERG diagnosis and differential diagnosis: results of examination over 6 years].

PURPOSE: This study reviews the patient material first from the point of view of referral diagnosis. Secondly, we focus on difficulties in selective differential diagnoses. PATIENTS AND METHOD: 1501 patients underwent electroretinographic (ERG) testing from 1989 to 1994, amounting to 1815 ERG recordings, including follow-up examinations. The technique applied is full-field, single flash ERG with selective stimulation of the rod- and of the cone-systems. In 3.8% (57 cases) the ERG was performed under general anesthesia in outpatients. RESULTS: Tapetoretinal degenerations, toxic retinal side effects, inflammatory disease and ocular trauma represented, in this order, the major groups of referral diagnoses aside from unclear visual loss. The documentation or the exclusion of tapetoretinal degeneration represented the largest share (57%) of the application of the diagnostic procedure. 171 cases of isolated retinitis pigmentosa (RP) and 33 cases of syndromic RP were identified. Frequent and rare diagnostic entities and their differential diagnoses within this group are discussed. Inevitably, a number of diagnostic decisions remain problematic, in particular at the first examination. These diagnostic difficulties are addressed also and include the differentiation between RP sine pigmento and congenital amaurosis Leber in infants, RP with macular involvement vs. cone-rod degeneration, unilateral RP vs. postinflammatory conditions, and progressive cone dystrophy vs. achromatopsia, cone-rod degeneration or Stargardt's disease. CONCLUSIONS: Frequent and meaningful indications for ERG recording and difficult diagnostic decisions arise from this review of a relatively large group of patients. A number of diagnoses can hardly, if not at all be established without ERG testing. These include retinal cause of visual loss in infants, congenital amaurosis Leber, RP sine pigmento, early stages of RP, carrier status in XL RP and in choroideremia, progressive cone dystrophy, toxic retinopathy without fundus changes, retinal involvement in uveitis with opaque media, and incomplete CSNB.

Adult↗
PubMedDate not supplied

The thermal contribution to photoactivation in A2 visual pigments studied by temperature effects on spectral properties.

Effects of temperature on the spectral properties of visual pigments were measured in the physiological range (5-28 degrees C) in photoreceptor cells of bullfrog (Rana catesbeiana) and crucian carp (Carassius carassius). Absorbance spectra recorded by microspectrophotometry (MSP) in single cells and sensitivity spectra recorded by electroretinography (ERG) across the isolated retina were combined to yield accurate composite spectra from ca. 400 nm to 800 nm. The four photoreceptor types selected for study allowed three comparisons illuminating the properties of pigments using the dehydroretinal (A2) chromophore: (1) the two members of an A1/A2 pigment pair with the same opsin (porphyropsin vs. rhodopsin in bullfrog "red" rods); (2) two A2 pigments with similar spectra (porphyropsin rods of bullfrog and crucian carp); and (3) two A2 pigments with different spectra (rods vs. long-wavelength-sensitive (L-) cones of crucian carp). Qualitatively, the temperature effects on A2 pigments were similar to those described previously for the A1 pigment of toad "red" rods. Warming caused an increase in relative sensitivities at very long wavelengths but additionally a small shift of lambdamax toward shorter wavelengths. The former effect was used for estimating the minimum energy required for photoactivation (Ea) of the pigment. Bullfrog rod opsin with A2 chromophore had Ea = 44.2 +/- 0.9 kcal/mol, significantly lower (one-tailed P < 0.05) than the value Ea = 46.5 +/- 0.8 kcal/mol for the same opsin coupled to A1. The A2 rod pigment of crucian carp had Ea = 42.3 +/- 0.6 kcal/mol, which is significantly higher (one-tailed P < 0.01) than that of the L-cones in the same retina (Ea = 38.3 +/- 0.4 kcal/mol), whereas the difference compared with the bullfrog A2 rod pigment is not statistically significant (two-tailed P = 0.13). No strict connection between lambdamax and Ea appears to exist among A2 pigments any more than among A1 pigments. Still, the A1 --> A2 chromophore substitution in bullfrog opsin causes three changes correlated as originally hypothesized by Barlow (1957): a red-shift of lambdamax, a decrease in Ea, and an increase in thermal noise.

Animals↗
PubMed1995

Nondetectable S-cone electroretinogram in a patient with crystalline retinopathy.

We examined cone and rod electroretinograms to ganzfeld stimuli in a patient with crystalline retinopathy. The 54-year-old man complained of night blindness, blurred vision, and metamorphopsia in both eyes. His visual acuity was 10/200 in the right eye and 10/20 in the left eye; his subjective dark-adaptation threshold was elevated 1 log unit, and he made one tritan error on the Farnsworth Panel D-15. Specular microscopic examinations revealed tiny crystalline deposits in the limbal cornea bilaterally. Ophthalmoscopically, crystalline deposits were found in the posterior fundi. His light-adapted cone electroretinograms to white stimuli were diminished (about 30% of those of normal controls), with normal implicit times. His dark-adapted rod electroretinogram amplitudes were 10% of those of normal controls. The S-cone electroretinogram was not detectable to different spectral stimuli with strong white background, while the L-M-cone responses appeared normal in waveforms with reduced amplitude. These ERG results indicated that the patient's S-cone system is more highly impaired than the L-M-cone system, supporting the psychophysical evidence that the S-cone system is more vulnerable than other cone systems in retinal diseases.

Dark Adaptation↗
PubMed1998

Histopathologic and immunohistochemical study of an autopsy eye with X-linked cone degeneration.

We performed a histopathologic and immunohistochemical study of eyes obtained at autopsy of an 84-year-old man from a family with X-linked cone degeneration in which affected members have a 6.5-kilobase deletion in the red cone pigment gene. At his most recent ocular examination, at age 71 years, this patient had had a visual acuity of 20/200 OU, fundus changes suggestive of macular degeneration, borderline-normal full-field rod electroretinograms, and profoundly reduced full-field cone electroretinograms. Histopathologic examination demonstrated marked loss of cone and rod photoreceptors and the retinal pigment epithelium in the central macula. The peripheral cone population was reduced, while the peripheral rod population was relatively preserved. Immunohistochemical examination with an antibody to both red and green cone opsin and an antibody to blue cone opsin disclosed a prominent loss of the red and green cone population and preservation of the blue cone population. These findings show that a defect in the red cone pigment gene can result in extensive degeneration of the red and green cone population across the retina.

Aged↗
PubMed2005

Highly effective phosphorylation by G protein-coupled receptor kinase 7 of light-activated visual pigment in cones.

Cone photoreceptors show briefer photoresponses than rod photoreceptors. Our previous study showed that visual pigment phosphorylation, a quenching mechanism of light-activated visual pigment, is much more rapid in cones than in rods. Here, we measured the early time course of this rapid phosphorylation with good time resolution and directly compared it with the photoresponse time course in cones. At the time of photoresponse recovery, almost two phosphates were incorporated into a bleached cone pigment molecule, which indicated that the visual pigment phosphorylation coincides with the photoresponse recovery. The rapid phosphorylation in cones is attributed to very high activity of visual pigment kinase [G protein-coupled receptor kinase (GRK) 7] in cones. Because of this high activity, cone pigment is readily phosphorylated at very high bleach levels, which probably explains why cone photoresponses recover quickly even after a very bright light and do not saturate under intense background light. The high GRK7 activity is brought about by high content of a highly potent enzyme. The expression level of GRK7 was 10 times higher than that of rod kinase (GRK1), and the specific activity of a single GRK7 molecule was approximately 10 times higher than that of GRK1. The specific activity of GRK7 is the highest among the GRKs so far known. Our result seems to explain the response characteristics of cone photoreceptors in many aspects, including the nonsaturation of the cone responses during daylight vision.

Animals↗
PubMed2002

Functionally rodless mice: transgenic models for the investigation of cone function in retinal disease and therapy.

Two genetically engineered strains of mice were used to characterize murine cone function electroretinographically, without interference of rod-driven responses: (1) mice with a deletion of the gene for the rod transducin alpha-subunit (transducin alpha-/-), and (2) mice with rod arrestin deleted (arrestin -/-). In the first three months of age, both strains have a normal complement of rods and normal rod structure, but transducin alpha-/- mice have no rod-driven responses to light, while rod-driven activity of arrestin -/- mice can be suppressed by a single intense flash for hours. In response to intense flashes the electroretinograms of these strains of mice showed a readily identifiable, pure-cone a-wave of approximately 10 microV saturating amplitude. A 530 nm background that saturates rod responses of wild type mice was found to desensitize the b-wave responses of mice of both transgenic lines, whether the b-waves were driven by photons captured by M- or UV-cone pigments. The desensitizing effect of the 530 nm background on UV-pigment driven responses provides new evidence in support of the hypothesis of functional co-expression of the M-pigment in cones expressing primarily the UV-pigment.

Animals↗
PubMed2003

Comparison of conventional ERG parameters and high-intensity A-wave analysis in a clinical setting.

Computational analysis of high-intensity a-waves yields direct information about the rod and cone receptor potential. However, it is not clear whether such information adds materially to the diagnostic value of the standard ERG in a routine clinical setting. We recorded both conventional ISCEV standard and computational high intensity ERG parameters from 38 patients referred to a clinical laboratory for ERG testing, and also from eight normal volunteers. The patients were grouped as: (1) macular dysfunction; (2) diffuse cone dysfunction; (3) diffuse rod-cone dysfunction. The results showed moderate variation in both conventional and computational parameters, but in general a similar pattern of normality or abnormality for both among the disease groups. There were only a few outlying subjects for which one or the other approach seemed more sensitive. We conclude that a-wave analysis is an important tool for clinical research and the study of special patients, but adding it to the standard ERG protocol does not, at our present state of knowledge, add markedly to clinical evaluations in a routine clinical setting.

Adult↗
PubMedDate not supplied

The neurons of the ground squirrel retina as revealed by immunostains for calcium binding proteins and neurotransmitters.

Ground squirrel retinas were immunostained with antibodies against calcium binding proteins (CBPs) and classical neurotransmitters in order to describe neuronal phenotypes in a diurnal mammalian retina and to then compare these neurons with those of more commonly studied nocturnal retinas like cats' and rabbits'. Double immunostained tissue was examined by confocal microscopy using antibodies against the following: rhodopsin and the CBPs, calbindin, calretinin, parvalbumin, calmodulin and recoverin (CB, CR, PV, CM, RV), glycine, GABA, choline acetyltransferase (CHAT) and tyrosine hydroxylase (TOH). In ground squirrel retina, the traditional cholinergic mirror symmetric amacrine cells colocalize CHAT with PV and GABA and faintly with glycine. A second cholinergic amacrine cell type colocalizes glycine alone. CR is found in at least 3 different amacrine cell types. The CR-immunoreactive (IR) cell population is a mixture of glycinergic and GABAergic types. The dopamine cell type IR to tyrosine hydroxylase has the typical morphology of a wide field cell with dendrites in S1 but the "rings" seen in cat or rabbit retina are not as numerous. TOH-IR amacrine cells send large club-shaped processes to the outer plexiform layer. CB and CR are in bipolar cells, A- and B-type horizontal cells and several amacrine cell types. Anti-rhodopsin labels the low density rod photoreceptor population in this species. Anti-recoverin labels cones and some bipolar cells while PKC is found in several different bipolar cell types. One ganglion cell with dendritic branching in S3 is strongly CR-IR. We find no evidence for an AII amacrine cell in the ground squirrel, with either anti-CR or anti-PV. An amacrine cell with similarity to the DAP1-3 cell of rabbit is CR-IR and glycine-IR. We discuss this labeling pattern in relationship to other mammalian species. The differences in staining patterns and phenotypes revealed suggest a functional diversity in the populations of amacrine cells according to whether the retinas are rod or cone dominated.

Amacrine Cells↗
PubMed2005

GDF11 controls the timing of progenitor cell competence in developing retina.

The orderly generation of cell types in the developing retina is thought to be regulated by changes in the competence of multipotent progenitors. Here, we show that a secreted factor, growth and differentiation factor 11 (GDF11), controls the numbers of retinal ganglion cells (RGCs), as well as amacrine and photoreceptor cells, that form during development. GDF11 does not affect proliferation of progenitors-a major mode of GDF11 action in other tissues-but instead controls duration of expression of Math5, a gene that confers competence for RGC genesis, in progenitor cells. Thus, GDF11 governs the temporal windows during which multipotent progenitors retain competence to produce distinct neural progeny.

Amacrine Cells↗