Retinal pigment epithelium-specific 65 kDa protein (also known as RPE65) is a retinoid isomerohydrolase enzyme of the vertebrate visual cycle.

2022年5月1日 · The retinal pigment epithelium 65 kDa protein (RPE65) is a critical component of the vertebrate visual system, playing a non-redundant role in the visual cycle metabolic pathway that regenerates visual chromophore, 11-cis-retinal, for rod and cone visual pigments.

2024年12月25日 · RPE65 (Retinoid Isomerohydrolase RPE65) is a Protein Coding gene. Diseases associated with RPE65 include Retinitis Pigmentosa 20 and Leber Congenital Amaurosis 2. Among its related pathways are Visual phototransduction and …

At the heart of this metabolic pathway is an enzyme known as retinal pigment epithelium 65 kDa protein (RPE65), which catalyzes an unusual, possibly biochemically unique, reaction consisting of a coupled all- trans -retinyl ester hydrolysis and alkene geometric isomerization to produce 11- …

2024年11月1日 · RPE cells have an efficient metabolic machinery to provide sufficient energy to the retina. RPE dysfunction or atrophy can lead to many retinopathies, such as age-related macular degeneration and proliferative vitreoretinopathy. Here, we discuss RPE development, functions, and roles in various ocular diseases, and the mechanisms involved.

2025年3月16日 · To capture the protein and phosphorylation dynamics during cell division in non-transformed hTERT-immortalised human retinal pigment epithelial (RPE-1) cells, we coupled reversible cell cycle ...

2020年11月1日 · RPE secretory proteins govern multiple processes (apical/basolateral ECM (re)modelling, angiogenesis, immune response). Altered RPE secretory proteostasis contributes to age- and disease-associated loss of retinal integrity. Mutated secreted proteins cause intracellular proteotoxicity and ER stress, alongside extracellular proteolytic imbalance.

2013年5月11日 · Understanding the importance of RPE polarity and the correct secretion of essential structural and regulatory components emerge as critical factors for the development of novel therapeutic strategies targeting AMD. Keywords: retina, retinal pigment epithelium, protein secretion, polarity, age-related macular degeneration.

6 天之前 · Retinal pigment epithelial (RPE) cells are the cellular component involved in proliferative vitreoretinopathy (PVR) development. Ocular trauma or surgery is the most common cause for PVR that is ...

Visual cycle proteins associated with forms of retinal degeneration include RPE65 (Leber congenital amaurosis, LCA; severe retinitis pigmentosa, RP); CRALBP and 11cRDH (encoded by gene retinol dehydrogenase 5, RDH5). The sodium/potassium adenosine triphosphatase (Na + /K + -ATPase) mediates outflux of sodium (Na +) and influx of potassium (K +).