The spectrum of ocular phenotypes caused by mutations in the BEST1 gene

CJF Boon, BJ Klevering, BP Leroy, CB Hoyng… - Progress in retinal and …, 2009 - Elsevier
Bestrophin-1 is an integral membrane protein, encoded by the BEST1 gene, which is
located in the basolateral membrane of the retinal pigment epithelium. The bestrophin-1 …

Autofluorescence from the outer retina and subretinal space: hypothesis and review

R Spaide - Retina, 2008 - journals.lww.com
Purpose: To review the pathophysiologic principles underlying increased autofluorescence
from the outer retina and subretinal space using selected diseases as examples. Methods …

Complement activation by photooxidation products of A2E, a lipofuscin constituent of the retinal pigment epithelium

J Zhou, YP Jang, SR Kim… - Proceedings of the …, 2006 - National Acad Sciences
Recent studies have implicated local inflammation and activation of complement amongst
the processes involved in the pathogenesis of age-related macular degeneration (AMD) …

Photooxidation of A2-PE, a photoreceptor outer segment fluorophore, and protection by lutein and zeaxanthin

SR Kim, K Nakanishi, Y Itagaki, JR Sparrow - Experimental eye research, 2006 - Elsevier
A2-PE is a pigment that forms as a byproduct of the visual cycle, its synthesis from all-trans-
retinal and phosphatidylethanolamine occurring in photoreceptor outer segments. A2-PE is …

Light‐induced damage to the retina: role of rhodopsin chromophore revisited

M Różanowska, T Sarna - Photochemistry and photobiology, 2005 - Wiley Online Library
The presence of the regenerable visual pigment rhodopsin has been shown to be primarily
responsible for the acute photo‐damage to the retina. The photoexcitation of rhodopsin …

Complement system dysregulation and inflammation in the retinal pigment epithelium of a mouse model for Stargardt macular degeneration

RA Radu, J Hu, Q Yuan, DL Welch… - Journal of Biological …, 2011 - ASBMB
Accumulation of vitamin A-derived lipofuscin fluorophores in the retinal pigment epithelium
(RPE) is a pathologic feature of recessive Stargardt macular dystrophy, a blinding disease …

Antioxidant properties of melanin in retinal pigment epithelial cells

Z Wang, J Dillon, ER Gaillard - Photochemistry and …, 2006 - Wiley Online Library
The retinal pigment epithelium (RPE) is a monolayer of highly pigmented cells lining the
inner aspect of Bruch's membrane. This pigmentation is due to eumelanin and a possible …

Structural characterization of bisretinoid A2E photocleavage products and implications for age-related macular degeneration

Y Wu, E Yanase, X Feng, MM Siegel… - Proceedings of the …, 2010 - National Acad Sciences
Fluorescent bisretinoids, such as A2E and all-trans-retinal dimer, form as a by-product of
vitamin A cycling in retina and accumulate in retinal pigment epithelial (RPE) cells as …

Applications of mass spectrometry to metabolomics and metabonomics: Detection of biomarkers of aging and of age‐related diseases

RJ Mishur, SL Rea - Mass spectrometry reviews, 2012 - Wiley Online Library
Every 5 years or so new technologies, or new combinations of old ones, seemingly burst
onto the science scene and are then sought after until they reach the point of becoming …

Vitelliform macular dystrophy

RF Spaide, K Noble, A Morgan, KB Freund - Ophthalmology, 2006 - Elsevier
PURPOSE: To investigate and integrate the photographic, angiographic, and tomographic
findings from a group of patients with various stages of vitelliform macular dystrophy type 2 …