The retinal pigment epithelium (RPE) is derived embryologically from the same neural anlage as the neurosensory retina.
Although it has no photoreceptive or neural function, the RPE is essential for the viability of photoreceptor cells and the choriocapillaris and thus for vision.
Mutation of the gene RPE65, which encodes the enzyme (RPE65 isomerohydrolase) that converts all-trans-retinyl ester to 11-cis-retinol, causes Leber congenital amaurosis (LCA). RPE65 is the target of a treatment approved by the US Food and Drug Administration (FDA) that uses an adeno-associated virus to deliver the gene to the RPE of LCA patients.
Autophagy is a homeostatic process whereby the cell degrades its own damaged components and recycles the products. In the RPE, this is essential for management of phagocytosed outer segments as well as for turnover of its components.
Excerpted from BCSC 2020-2021 series: Section 2 - Fundamentals and Principles of Ophthalmology. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.