Vitamin A Metabolism in Rod and Cone Visual Cycles

The chromophore of all known visual pigments consists of 11-cis-retinal (derived from either vitamin A1 or A2) or a hydroxylated derivative, bound to a protein (opsin) via a Schiff base. Absorption of a photon results in photoisomerization of the chromophore to all-trans-retinal and conversion of the visual pigment to the signaling form. Regeneration of the 11-cis-retinal occurs in an adjacent tissue and involves several enzymes, several water-soluble retinoid-binding proteins, and intra-and intercellular diffusional processes. Rod photoreceptor cells depend completely on the output of 11-cis-retinal from adjacent retinal pigment epithelial (RPE) cells. Cone photoreceptors cells can use 11-cis-retinal from the RPE and from a second more poorly characterized cycle, which appears to involve adjacent Muller (glial) cells. Recent progress in the characterization of rod and cone visual cycle components and reactions will result in the development of approaches to the amelioration of blinding eye diseases associated with visual cycle defects.