Normal Cone Function Requires the Interphotoreceptor Retinoid Binding Protein

• Type of Material: Article
• Authors: Ryan O. Parker, Medical University of South Carolina; Jie Fan, Medical University of South Carolina; John Nickerson, Emory University; Gregory I. Liou, Medical College of Georgia; Rosalie K. Crouch, Medical University of South Carolina
• Language: English
• Date: 2009-04-08
• Publisher: Society for Neuroscience
• Publication Version: Final Published Version
• Copyright Statement: ©2009 Society for Neuroscience
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://www.jneurosci.org/content/29/14/4616
• Title of Journal or Parent Work: Journal of Neuroscience Nursing
• ISSN: 0270-6474
• Volume: 29
• Issue: 14
• Start Page: 4616
• End Page: 4621
• Grant/Funding Information: This study was supported by grants from NIH EY04939 (R.K.C.), EY14793 (MUSC vision core), EY016470 (J.N.), EY017045 (J.N.), and EY006360 (J.N.); Foundation Fighting Blindness, Inc. (Owings Mills, MD) (J.N., R.K.C.); and unrestricted awards to the Departments of Ophthalmology at MUSC and Emory University from Research to Prevent Blindness (RPB; New York); R.K.C. is an RPB Senior Scientific Investigator.
• Research Categories: Health Sciences, Opthamology; Biology, Neuroscience

Abstract

11-cis-retinal is the light-sensitive component in rod and cone photoreceptors, and its isomerization to all-trans retinal in the presence of light initiates the visual response. For photoreceptors to function normally, all-trans retinal must be converted back into 11-cis-retinal through a series of enzymatic steps known as the visual cycle. The interphotoreceptor retinoid-binding protein (IRBP) is a proposed retinoid transporter in the visual cycle, but rods in Irbp−/− mice have a normal visual cycle. While rods are primarily responsible for dim light vision, the ability of cones to function in constant light is essential to human vision and may be facilitated by cone-specific visual cycle pathways. We analyzed the cones in Irbp−/− mice to determine whether IRBP has a cone-specific visual cycle function. Cone electroretinogram (ERG) responses were reduced in Irbp−/− mice, but similar responses from Irbp−/− mice at all ages suggest that degeneration does not underlie cone dysfunction. Furthermore, cone densities and opsin levels in Irbp−/− mice were similar to C57BL/6 (wild-type) mice, and both cone opsins were properly localized to the cone outer segments. To test for retinoid deficiency in Irbp−/− mice, ERGs were analyzed before and after intraperitoneal injections of 9-cis-retinal. Treatment with 9-cis-retinal produced a significant recovery of the cone response in Irbp−/− mice and shows that retinoid deficiency underlies cone dysfunction. These data indicate that IRBP is essential to normal cone function and demonstrate that differences exist in the visual cycle of rods and cones.