title: Supplementation with vitamin a derivatives to rescue vision in animal models of degenerative retinal diseases.
abstract: The perception of light begins when photons reach retinal tissue located at the back of the eye and photoisomerize the visual chromophore 11-cis-retinal to all-trans-retinal within photoreceptor cells. Isomerization of 11-cis-retinal activates the protein rhodopsin located in photoreceptor outer segments, thereby inducing a phototransduction cascade leading to visual perception. To maintain vision, 11-cis-retinal is regenerated in the retinal pigmented epithelium (RPE) via the visual cycle and delivered back to the photoreceptor cells where it may again bind to rhodopsin. Distinct pathological mechanisms have been observed to contribute to inherited retinal degenerative diseases including severe delay in 11-cis-retinal regeneration and delayed clearance of all-trans-retinal, which leads to the accumulation of harmful retinoid by-products. In the last decade, our group has conducted several proof-of-concept (POC) studies with retinoid derivatives aimed at developing treatments for retinal degenerative diseases caused by an impaired visual cycle. Here, we will introduce experimental procedures, which have been developed for POC studies involving retinoid biology.
authors.affiliation: Department of Ophthalmology and Visual Sciences, School of Medicine, Case Western Reserve University, Adelbert Road 2085, Cleveland, OH, 44106, USA.
authors.forename: Lindsay
authors.initials: L
authors.lastname: Perusek
authors.forename: Akiko
authors.initials: A
authors.lastname: Maeda
authors.forename: Tadao
authors.initials: T
authors.lastname: Maeda
mesh_terms: D000818:Animals; D004224:Diterpenes / Q000378:metabolism; D055537:Light Signal Transduction / Q000187:drug effects; D012160:Retina / Q000187:drug effects / Q000473:pathology; D012164:Retinal Diseases / Q000188:drug therapy*; D055213:Retinal Pigment Epithelium / Q000378:metabolism; D012243:Rhodopsin / Q000378:metabolism; D014785:Vision, Ocular / Q000187:drug effects; D014801:Vitamin A / Q000627:therapeutic use*
chemical_list: D004224:Diterpenes; D014801:Vitamin A; C009167:retinylamine; D012243:Rhodopsin