Rod Photoreceptor Temporal Properties in Retinal Degenerative Diseases

Wen, Yuquan; Locke, Kirsten G.; Hood, Donald C.; Birch, David G.

doi:10.1007/978-1-4614-0631-0_62

Cited by 2 publications

(2 citation statements)

References 18 publications

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“…Even before major rod degeneration is apparent, the photoresponses of Rho P23H/+ rods exhibit uncharacteristically fast recovery kinetics ( Sakami et al, 2014 ). Such accelerated rod recovery kinetics have also been reported in human patients with adRP ( Wen et al, 2011 , 2012 ), and in other animal models with disease causing rhodopsin mutations: Rho P347L/S/+ pigs ( Kraft et al, 2005 ), Rho G90D/+ mice ( Sieving et al, 2001 ; Woodruff et al, 2007 ), Rho E150K mice ( Zhang et al, 2013 ), and reduced rhodopsin expression: Rho +/− mice ( Lem et al, 1999 ; Calvert et al, 2001 ). However, the impact that the acceleration of rod recovery kinetics has on visual performance during retinal degeneration has not been examined.…”

Section: Introductionmentioning

confidence: 53%

Temporal Contrast Sensitivity Increases despite Photoreceptor Degeneration in a Mouse Model of Retinitis Pigmentosa

Pasquale

Guo

Umino

et al. 2021

eNeuro

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The detection of temporal variations in amplitude of light intensity, or temporal contrast sensitivity (TCS), depends on the kinetics of rod photoresponse recovery. Uncharacteristically fast rod recovery kinetics are facets of both human patients and transgenic animal models with a P23H rhodopsin mutation, a prevalent cause of retinitis pigmentosa (RP). Here, we show that mice with this mutation (Rho P23H/+ ) exhibit an age-dependent and illumination-dependent enhancement in TCS compared with controls. At retinal illumination levels producing ≥1000 R*/rod/s or more, postnatal day 30 (P30) Rho P23H/+ mice exhibit a 1.2-fold to 2-fold increase in retinal and optomotor TCS relative to controls in response to flicker frequencies of 3, 6, and 12 Hz despite significant photoreceptor degeneration and loss of flash electroretinogram (ERG) b-wave amplitude. Surprisingly, the TCS of Rho P23H/+ mice further increases as degeneration advances. Enhanced TCS is also observed in a second model (rhodopsin heterozygous mice, Rho +/− ) with fast rod recovery kinetics and no apparent retinal degeneration. In both mouse models, enhanced TCS is explained quantitatively by a comprehensive model that includes photoresponse recovery kinetics, density and collecting area of degenerating rods. Measurement of TCS may be a non-invasive early diagnostic tool indicative of rod dysfunction in some forms of retinal degenerative disease.

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Section: Introductionmentioning

confidence: 53%

Temporal Contrast Sensitivity Increases despite Photoreceptor Degeneration in a Mouse Model of Retinitis Pigmentosa

Pasquale

Guo

Umino

et al. 2021

eNeuro

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“…Not surprisingly, the amplitude of the rod response in patients with RP and cone-rod dystrophy is reduced compared to normal (Hood and Birch, 1994). The rod response, when present, can show altered kinetics with disease (Wen et al, 2011, 2012). In some diseases, photoreceptors are still present but their transmission is perturbed.…”

Section: Disease: Alterations To Structure and Functionmentioning

confidence: 99%

Functional architecture of the retina: Development and disease

Hoon

Okawa

Santina

et al. 2014

Progress in Retinal and Eye Research

469

385

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Structure and function are highly correlated in the vertebrate retina, a sensory tissue that is organized into cell layers with microcircuits working in parallel and together to encode visual information. All vertebrate retinas share a fundamental plan, comprising five major neuronal cell classes with cell body distributions and connectivity arranged in stereotypic patterns. Conserved features in retinal design have enabled detailed analysis and comparisons of structure, connectivity and function across species. Each species, however, can adopt structural and/or functional retinal specializations, implementing variations to the basic design in order to satisfy unique requirements in visual function. Recent advances in molecular tools, imaging and electrophysiological approaches have greatly facilitated identification of the cellular and molecular mechanisms that establish the fundamental organization of the retina and the specializations of its microcircuits during development. Here, we review advances in our understanding of how these mechanisms act to shape structure and function at the single cell level, to coordinate the assembly of cell populations, and to define their specific circuitry. We also highlight how structure is rearranged and function is disrupted in disease, and discuss current approaches to re-establish the intricate functional architecture of the retina.

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Rod Photoreceptor Temporal Properties in Retinal Degenerative Diseases

Cited by 2 publications

References 18 publications

Temporal Contrast Sensitivity Increases despite Photoreceptor Degeneration in a Mouse Model of Retinitis Pigmentosa

Temporal Contrast Sensitivity Increases despite Photoreceptor Degeneration in a Mouse Model of Retinitis Pigmentosa

Functional architecture of the retina: Development and disease

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