Cones and cone pathways remain functional in advanced retinal degeneration. Curr Biol 2023 Apr 24;33(8):1513-1522.e4
Date
03/29/2023Pubmed ID
36977418Pubmed Central ID
PMC10133175DOI
10.1016/j.cub.2023.03.007Scopus ID
2-s2.0-85152914523 (requires institutional sign-in at Scopus site) 4 CitationsAbstract
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 cones 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.
Author List
Ellis EM, Paniagua AE, Scalabrino ML, Thapa M, Rathinavelu J, Jiao Y, Williams DS, Field GD, Fain GL, Sampath APAuthor
Miranda L. Scalabrino PhD Assistant Professor in the Ophthalmology and Visual Sciences department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Color VisionHumans
Retina
Retinal Cone Photoreceptor Cells
Retinal Degeneration
Retinitis Pigmentosa
