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Biosynthesis of A2E, implicated in retinal degradation

Reactome ID: R-HSA-2466712

中文名称

A2E 的生物合成,与视网膜降解有关

通路描述

脂褐素是一种主要由脂质组成但也含有糖和某些金属的黄褐色色素颗粒。脂褐素的积累与退行性疾病有关,如阿尔茨海默病、帕金森病、慢性阻塞性肺病和视网膜黄斑变性。
英文描述
Biosynthesis of A2E, implicated in retinal degradation Lipofuscin is a yellow-brown pigment grain composed mainly of lipids but also sugars and certain metals. Accumulation of lipofuscin is associated with degenerative diseases such as Alzheimer's disease, Parkinson's disease, chronic obstructive pulmonary disease and retinal macular degeneration.

A prominent component of lipofuscin in retinal pigment epithelial (RPE) cells is the bisretinoid A2E (di-retinoid-pyridinium-ethanolamine), the end-product of the condensation of 2 molecules of all-trans-retinal (atRAL) and phosphatidylethanolamine (PE) in photoreceptor outer disc membranes. Once formed, A2E is phagocytosed, together with outer segments (Kevany & Palczewski 2010), to RPE where it accumulates. There is no evidence as yet to indicate that A2E can be catabolised (Sparrow et al. 2012, Sparrow et al. 2010). A simplified biosynthetic pathway for A2E is described here.

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