Journal
FEBS JOURNAL
Volume 289, Issue 22, Pages 7199-7212Publisher
WILEY
DOI: 10.1111/febs.16018
Keywords
AMD; autophagy; lysosomal storage disease; mTOR; retinal pigment epithelium
Categories
Funding
- Italian Telethon Foundation [TMICCBX16TT]
- National MPS Society (TMICGMXMPSO)
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The retinal pigment epithelium (RPE) plays a crucial role in maintaining the function of photoreceptors and responding to stress conditions. Autophagy is a cellular self-regulation process that helps in digestion and recycling of cell components to meet cellular demands.
The retinal pigment epithelium (RPE) is a highly specialized monolayer of polarized, pigmented epithelial cells that resides between the vessels of the choriocapillaris and the neural retina. The RPE is essential for the maintenance and survival of overlying light-sensitive photoreceptors, as it participates in the formation of the outer blood-retinal barrier, phagocytosis, degradation of photoreceptor outer segment (POS) tips, maintenance of the retinoid cycle, and protection against light and oxidative stress. Autophagy is an evolutionarily conserved 'self-eating' process, designed to maintain cellular homeostasis. The daily autophagy demands in the RPE require precise gene regulation for the digestion and recycling of intracellular and POS components in lysosomes in response to light and stress conditions. In this review, we discuss selective autophagy and focus on the recent advances in our understanding of the mechanism of cell clearance in the RPE for visual function. Understanding how this catabolic process is regulated by both transcriptional and post-transcriptional mechanisms in the RPE will promote the recognition of pathological pathways in genetic disease and shed light on potential therapeutic strategies to treat visual impairments in patients with retinal disorders associated with lysosomal dysfunction.
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