| Literature DB >> 27875314 |
Grazyna Palczewska1, Akiko Maeda2, Marcin Golczak3, Eisuke Arai4, Zhiqian Dong1, Lindsay Perusek4, Brian Kevany3, Krzysztof Palczewski5.
Abstract
Accumulation of bis-retinoids in the retinal pigmented epithelium (RPE) is a hallmark of aging and retinal disorders such as Stargardt disease and age-related macular degeneration. These aberrant fluorescent condensation products, including di-retinoid-pyridinium-ethanolamine (A2E), are thought to be transferred to RPE cells primarily through phagocytosis of the photoreceptor outer segments. However, we observed by two-photon microscopy that mouse retinas incapable of phagocytosis due to a deficiency of the c-Mer proto-oncogene tyrosine kinase (Mertk) nonetheless contained fluorescent retinoid condensation material in their RPE. Primary RPE cells from Mertk-/- mice also accumulated fluorescent products in vitro Finally, quantification of A2E demonstrated the acquisition of retinal condensation products in Mertk-/- mouse RPE prior to retinal degeneration. In these mice, we identified activated microglial cells that likely were recruited to transport A2E-like condensation products to the RPE and dispose of the dying photoreceptor cells. These observations demonstrate a novel transport mechanism between photoreceptor cells and RPE that does not involve canonical Mertk-dependent phagocytosis.Entities:
Keywords: retina; retinal degeneration; retinal metabolism; rhodopsin; vitamin A
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Year: 2016 PMID: 27875314 PMCID: PMC5207201 DOI: 10.1074/jbc.M116.764563
Source DB: PubMed Journal: J Biol Chem ISSN: 0021-9258 Impact factor: 5.157