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Human photoreceptor outer segments shorten during light adaptation
Journal article   Open access   Peer reviewed

Human photoreceptor outer segments shorten during light adaptation

Michael D Abràmoff, Robert F Mullins, Kyungmoo Lee, Jeremy M Hoffmann, Milan Sonka, Douglas B Critser, Steven F Stasheff and Edwin M Stone
Investigative ophthalmology & visual science, Vol.54(5), pp.3721-3728
05/01/2013
DOI: 10.1167/iovs.13-11812
PMCID: PMC3668803
PMID: 23633665
url
https://doi.org/10.1167/iovs.13-11812View
Published (Version of record) Open Access

Abstract

Best disease is a macular dystrophy caused by mutations in the BEST1 gene. Affected individuals exhibit a reduced electro-oculographic (EOG) response to changes in light exposure and have significantly longer outer segments (OS) than age-matched controls. The purpose of this study was to investigate the anatomical changes in the outer retina during dark and light adaptation in unaffected and Best disease subjects, and to compare these changes to the EOG. Unaffected (n = 11) and Best disease patients (n = 7) were imaged at approximately 4-minute intervals during an approximately 40-minute dark-light cycle using spectral domain optical coherence tomography (SD-OCT). EOGs of two subjects were obtained under the same conditions. Automated three-dimensional (3-D) segmentation allowed measurement of light-related changes in the distances between five retinal surfaces. In normal subjects, there was a significant decrease in outer segment equivalent length (OSEL) of -2.14 μm (95% confidence interval [CI], -1.77 to -2.51 μm) 10 to 20 minutes after the start of light adaptation, while Best disease subjects exhibited a significant increase in OSEL of 2.07 μm (95% CI, 1.79-2.36 μm). The time course of the change in OS length corresponded to that of the EOG waveform. Our results strongly suggest that the light peak phase of the EOG is temporally related to a decreased OSEL in normal subjects, and the lack of a light peak phase in Best disease subjects is associated with an increase in OSEL. One potential role of Bestrophin-1 is to trigger an increase in the standing potential that approximates the OS to the apical surface of the RPE to facilitate phagocytosis.
 Electrooculography Prospective Studies Tomography, Optical Coherence Humans Middle Aged Phagocytosis - physiology Dark Adaptation - physiology Eye Banks Genotype Male Retinal Photoreceptor Cell Outer Segment - pathology Vitelliform Macular Dystrophy - physiopathology Retinal Photoreceptor Cell Outer Segment - physiology Vitelliform Macular Dystrophy - genetics Retinal Pigment Epithelium - physiology Vitelliform Macular Dystrophy - pathology Adult Female Adaptation, Ocular - physiology Imaging, Three-Dimensional

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