The retinal pigment epithelium in health and disease

JR Sparrrow, D Hicks… - Current molecular …, 2010 - ingentaconnect.com
Retinal pigment epithelial cells (RPE) constitute a simple layer of cuboidal cells that are
strategically situated behind the photoreceptor (PR) cells. The inconspicuousness of this …

[HTML][HTML] Chemistry of the retinoid (visual) cycle

PD Kiser, M Golczak, K Palczewski - Chemical reviews, 2014 - ACS Publications
As succinctly summarized by Wolf, 1 lack of vitamin A (all-trans-retinol) was recognized by
ancient Egyptians as causing a visual deficiency involving the retina and cornea that could …

Leber congenital amaurosis: genes, proteins and disease mechanisms

AI den Hollander, R Roepman, RK Koenekoop… - Progress in retinal and …, 2008 - Elsevier
Leber congenital amaurosis (LCA) is the most severe retinal dystrophy causing blindness or
severe visual impairment before the age of 1 year. Linkage analysis, homozygosity mapping …

Dark adaptation and the retinoid cycle of vision

TD Lamb, EN Pugh Jr - Progress in retinal and eye research, 2004 - Elsevier
Following exposure of our eye to very intense illumination, we experience a greatly elevated
visual threshold, that takes tens of minutes to return completely to normal. The slowness of …

[HTML][HTML] The role of vitamin A in retinal diseases

J Sajovic, A Meglič, D Glavač, Š Markelj… - International journal of …, 2022 - mdpi.com
Vitamin A is an essential fat-soluble vitamin that occurs in various chemical forms. It is
essential for several physiological processes. Either hyper-or hypovitaminosis can be …

Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agents

GH Travis, M Golczak, AR Moise… - Annu. Rev. Pharmacol …, 2007 - annualreviews.org
Absorption of a photon by an opsin pigment causes isomerization of the chromophore from
11-cis-retinaldehyde to all-trans-retinaldehyde. Regeneration of visual chromophore …

[HTML][HTML] Lecithin-retinol acyltransferase is essential for accumulation of all-trans-retinyl esters in the eye and in the liver

ML Batten, Y Imanishi, T Maeda, DC Tu… - Journal of Biological …, 2004 - ASBMB
Lecithin-retinol acyltransferase (LRAT), an enzyme present mainly in the retinal pigmented
epithelial cells and liver, converts all-trans-retinol into all-trans-retinyl esters. In the retinal …

Expression of ABCA4 in the retinal pigment epithelium and its implications for Stargardt macular degeneration

TL Lenis, J Hu, SY Ng, Z Jiang… - Proceedings of the …, 2018 - National Acad Sciences
Recessive Stargardt disease (STGD1) is an inherited blinding disorder caused by mutations
in the Abca4 gene. ABCA4 is a flippase in photoreceptor outer segments (OS) that …

Vitamin A metabolism in rod and cone visual cycles

JC Saari - Annual review of nutrition, 2012 - annualreviews.org
The chromophore of all known visual pigments consists of 11-cis-retinal (derived from either
vitamin A1 or A2) or a hydroxylated derivative, bound to a protein (opsin) via a Schiff base …

[HTML][HTML] Mechanisms of feedback regulation of vitamin A metabolism

C O'Connor, P Varshosaz, AR Moise - Nutrients, 2022 - mdpi.com
Vitamin A is an essential nutrient required throughout life. Through its various metabolites,
vitamin A sustains fetal development, immunity, vision, and the maintenance, regulation, and …