title: Cone Health and Retinoids.
abstract: Cones are photoreceptor cells used for bright light and color vision. Retinoids are vitamin A derivatives, one of which is the 11-cis aldehyde form that serves as the chromophore for both cone and rod visual pigments. In the visual disease, Type 2 Leber congenital amaurosis (LCA2), 11-cis-retinal generation is inhibited or abolished. Work by others has shown that patients with LCA2 have symptoms consistent with degenerating cones. In mouse models for LCA2, early cone degeneration is readily apparent: cone opsins and other proteins associated with the outer segment are delocalized and cell numbers decline rapidly within the first month. Rods would appear normal morphologically and functionally, if not for the absence of chromophore. Supplementation of mouse models of LCA2 with cis-retinoids has been shown to slow loss of cone photoreceptor cells if mice were maintained in darkness. Thus, 11-cis-retinal appears not only to have a role in the light response reaction but also to promote proper trafficking of the cone opsins and maintain viable cones.
authors: Department of Ophthalmology, Albert Florens Storm Eye Institute, Medical University of South Carolina, Charleston, South Carolina, USA. Electronic address: konom@musc.edu.
authors: Masahiro
authors: M
authors: Kono
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D057130:Leber Congenital Amaurosis / Q000473:pathology; D055355:Opsins / Q000378:metabolism; D017949:Retinal Cone Photoreceptor Cells / Q000378:metabolism*; D012176:Retinoids / Q000502:physiology*; D014795:Visual Pathways / Q000378:metabolism
chemical_list: D055355:Opsins; D012176:Retinoids
keywords: 11-cis-retinal; Cone degeneration; Leber congenital amaurosis; Retinal degeneration; Vitamin A