Comparison of photopic negative response (PhNR) between focal macular and full-field electroretinograms in monkeys

Kinoshita, Junzo; Takada, S.; Iwata, Noriaki; Tani, Yoshiro

doi:10.1007/s10633-016-9538-x

Cited by 4 publications

(4 citation statements)

References 19 publications

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“…To determine RGCs functionality and visual signal conduction to the brain in Wfs1 mutant mice, we exploited whole field photopic electroretinogram (pERG), which measures action potentials produced by the retina when it is stimulated by light of adequate intensity and it is the composite of electrical activity from the photoreceptors, inner retina, and RGCs. In photopic conditions, the negative response (PhNR) wave represents the RGC spiking activity originating from light-adapted cones and allows the detection of RGC-specific alterations ( Gotoh et al, 2004 ; Kinoshita et al, 2016 ). Whilst pERG measurements were comparable between wild-type and Wfs1 mutant mice at 2 months of age ( WT : 68±5; Wfs1- KO: 72±6; p<0.05), a prolonged implicit time, which refers to the interval between the stimulation and the peak of the negative response, was detected in 4 months old Wfs1 -deficient animals ( WT : 76±5; Wfs1- KO: 82±6; p<0.05) ( Figure 1A ).…”

Section: Resultsmentioning

confidence: 99%

MCT1-dependent energetic failure and neuroinflammation underlie optic nerve degeneration in Wolfram syndrome mice

Rossi

Ordazzo

Vanni

et al. 2023

eLife

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Wolfram syndrome 1 (WS1) is a rare genetic disorder caused by mutations in the WFS1 gene leading to a wide spectrum of clinical dysfunctions, among which blindness, diabetes and neurological deficits are the most prominent. WFS1 encodes for the endoplasmic reticulum (ER) resident transmembrane protein wolframin with multiple functions in ER processes. However, the WFS1-dependent etiopathology in retinal cells is unknown. Herein, we showed that Wfs1 mutant mice developed early retinal electrophysiological impairments followed by marked visual loss. Interestingly, axons and myelin disruption in the optic nerve preceded the degeneration of the retinal ganglion cell bodies in the retina. Transcriptomics at pre-degenerative stage revealed the STAT3-dependent activation of proinflammatory glial markers with reduction of the homeostatic and pro-survival factors glutamine synthetase and BDNF. Furthermore, label-free comparative proteomics identified a significant reduction of the monocarboxylate transport isoform 1 (MCT1) and its partner basigin that are highly enriched on retinal glia and myelin-forming oligodendrocytes in optic nerve together with wolframin. Loss of MCT1 caused a failure in lactate transfer from glial to neuronal cell bodies and axons leading to a chronic hypometabolic state. Thus, this bioenergetic impairment is occurring concurrently both within the axonal regions and cell bodies of the retinal ganglion cells, selectively endangering their survival while impacting less on other retinal cells. This metabolic dysfunction occurs months before the frank RGC degeneration suggesting an extended time-window for intervening with new therapeutic strategies focused on boosting retinal and optic nerve bioenergetics in WS1.

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Section: Resultsmentioning

confidence: 99%

MCT1-dependent energetic failure and neuroinflammation underlie optic nerve degeneration in Wolfram syndrome mice

Rossi

Ordazzo

Vanni

et al. 2023

eLife

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“…In details, it has been clarified that PhNR signal originates from inner retinal cells and specifically from RGCs [27][28][29]. Therefore, from the available literature, simplistically, it might be expected that because the amplitude of the PhNR maps on to the distribution of RGCs and because PhNR amplitude increases with increased stimulus area, PhNR amplitude would increase linearly with increasing RGCs count.…”

Section: Mfphnr Findings By Applying Ring Analysismentioning

confidence: 99%

“…More specifically, the neuronal origin of the ff-PhNR is from the IRL, containing RGCs and their fibers, with a potential contribution from non-neuronal elements, such as the Muller glial cells [36]. That the PhNR is originated by the above-mentioned retinal elements is supported by experimental studies performed in animal models [28,29]. In addition, since the ff-PhNR measurement is not influenced by several conditions (i.e., unknown refractive error, ocular media opacities, not stable target fixation during the recording session) [32,33,37,38], this method may present some advantages in the clinical practice with respect to PERG assessment that, instead, require the correction of refractive error, ocular media transparency, and stable target fixation for a reliable recordings.…”

Section: Introductionmentioning

confidence: 95%

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Multifocal Electroretinogram Photopic Negative Response: A Reliable Paradigm to Detect Localized Retinal Ganglion Cells’ Impairment in Retrobulbar Optic Neuritis Due to Multiple Sclerosis as a Model of Retinal Neurodegeneration

et al. 2022

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The measure of the full-field photopic negative response (ff-PhNR) of light-adapted full-field electroretinogram (ff-ERG) allows to evaluate the function of the innermost retinal layers (IRL) containing primarily retinal ganglion cells (RGCs) and other non-neuronal elements of the entire retina. The aim of this study was to acquire functional information of localized IRL by measuring the PhNR in response to multifocal stimuli (mfPhNR). In this case-control observational and retrospective study, we assessed mfPhNR responses from 25 healthy controls and from 20 patients with multiple sclerosis with previous history of optic neuritis (MS-ON), with full recovery of visual acuity, IRL morphological impairment, and absence of morpho-functional involvement of outer retinal layers (ORL). MfPhNR response amplitude densities (RADs) were measured from concentric rings (R) with increasing foveal eccentricity: 0–5° (R1), 5–10° (R2), 10–15° (R3), 15–20° (R4), and 20–25° (R5) from retinal sectors (superior-temporal (ST), superior-nasal (SN), inferior-nasal (IN), and inferior-temporal (IT)); between 5° and 20° and from retinal sectors (superior (S), temporal (T), inferior (I), and nasal (N)); and within 5° to 10° and within 10° and 20° from the fovea. The mfPhNR RAD values observed in all rings or sectors in MS-ON eyes were significantly reduced (p < 0.01) with respect to control ones. Our results suggest that mfPhNR recordings may detect localized IRL dysfunction in the pathologic condition of selective RGCs neurodegeneration.

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Relationship between stimulus size and different components of the electroretinogram (ERG) elicited by flashed stimuli

et al. 2020

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Comparison of photopic negative response (PhNR) between focal macular and full-field electroretinograms in monkeys

Cited by 4 publications

References 19 publications

MCT1-dependent energetic failure and neuroinflammation underlie optic nerve degeneration in Wolfram syndrome mice

MCT1-dependent energetic failure and neuroinflammation underlie optic nerve degeneration in Wolfram syndrome mice

Multifocal Electroretinogram Photopic Negative Response: A Reliable Paradigm to Detect Localized Retinal Ganglion Cells’ Impairment in Retrobulbar Optic Neuritis Due to Multiple Sclerosis as a Model of Retinal Neurodegeneration

Relationship between stimulus size and different components of the electroretinogram (ERG) elicited by flashed stimuli

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