Cones and cone pathways remain functional in advanced retinal degeneration. 2023

Erika M Ellis, and Antonio E Paniagua, and Miranda L Scalabrino, and Mishek Thapa, and Jay Rathinavelu, and Yuekan Jiao, and David S Williams, and Greg D Field, and Gordon L Fain, and Alapakkam P Sampath
Department of Ophthalmology and Jules Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA 90095-7000, USA.

Most defects causing retinal degeneration in retinitis pigmentosa (RP) are rod-specific mutations, but the subsequent degeneration of cones, which produces loss of daylight vision and high-acuity perception, is the most debilitating feature of the disease. To understand better why cones degenerate and how cone vision might be restored, we have made the first single-cell recordings of light responses from degenerating conesĀ and retinal interneurons after most rods have died and cones have lost their outer-segment disk membranes and synaptic pedicles. We show that degenerating cones have functional cyclic-nucleotide-gated channels and can continue to give light responses, apparently produced by opsin localized either to small areas of organized membrane near the ciliary axoneme or distributed throughout the inner segment. Light responses of second-order horizontal and bipolar cells are less sensitive but otherwise resemble those of normal retina. Furthermore, retinal output as reflected in responses of ganglion cells is less sensitive but maintains spatiotemporal receptive fields at cone-mediated light levels. Together, these findings show that cones and their retinal pathways can remain functional even as degeneration is progressing, an encouraging result for future research aimed at enhancing the light sensitivity of residual cones to restore vision in patients with genetically inherited retinal degeneration.

UI MeSH Term Description Entries
D012160 Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the OPTIC NERVE and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the CHOROID and the inner surface with the VITREOUS BODY. The outer-most layer is pigmented, whereas the inner nine layers are transparent. Ora Serrata
D012162 Retinal Degeneration A retrogressive pathological change in the retina, focal or generalized, caused by genetic defects, inflammation, trauma, vascular disease, or aging. Degeneration affecting predominantly the macula lutea of the retina is MACULAR DEGENERATION. (Newell, Ophthalmology: Principles and Concepts, 7th ed, p304) Degeneration, Retinal,Degenerations, Retinal,Retinal Degenerations
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012174 Retinitis Pigmentosa Hereditary, progressive degeneration of the retina due to death of ROD PHOTORECEPTORS initially and subsequent death of CONE PHOTORECEPTORS. It is characterized by deposition of pigment in the retina. Pigmentary Retinopathy,Tapetoretinal Degeneration,Pigmentary Retinopathies,Retinopathies, Pigmentary,Retinopathy, Pigmentary,Tapetoretinal Degenerations
D055253 Color Vision Function of the human eye that is used in bright illumination or in daylight (at photopic intensities). Photopic vision is performed by the three types of RETINAL CONE PHOTORECEPTORS with varied peak absorption wavelengths in the color spectrum (from violet to red, 400 - 700 nm). Photopic Vision,Daylight Vision,Color Visions,Vision, Color,Vision, Daylight,Vision, Photopic,Visions, Color
D017949 Retinal Cone Photoreceptor Cells Photosensitive afferent neurons located primarily within the FOVEA CENTRALIS of the MACULA LUTEA. There are three major types of cone cells (red, blue, and green) whose photopigments have different spectral sensitivity curves. Retinal cone cells operate in daylight vision (at photopic intensities) providing color recognition and central visual acuity. Cone Photoreceptors,Cones (Retina),Cone Photoreceptor Cells,Photoreceptors, Cone,Retinal Cone,Retinal Cone Cells,Retinal Cone Photoreceptors,Cell, Cone Photoreceptor,Cell, Retinal Cone,Cells, Cone Photoreceptor,Cells, Retinal Cone,Cone (Retina),Cone Cell, Retinal,Cone Cells, Retinal,Cone Photoreceptor,Cone Photoreceptor Cell,Cone Photoreceptor, Retinal,Cone Photoreceptors, Retinal,Cone, Retinal,Cones, Retinal,Photoreceptor Cell, Cone,Photoreceptor Cells, Cone,Photoreceptor, Cone,Photoreceptor, Retinal Cone,Photoreceptors, Retinal Cone,Retinal Cone Cell,Retinal Cone Photoreceptor,Retinal Cones

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