Department of Neurosciences, Medical University of South Carolina, Charleston, SC, USA.
Adv Exp Med Biol. 2010;664:141-9. doi: 10.1007/978-1-4419-1399-9_17.
11-cis Retinal is the light-sensitive component in rod and cone photoreceptors, and its isomerization to all-trans retinal in the presence of light initiates the visual response. For photoreceptors to function normally, all-trans retinal must be converted back into 11-cis retinal through the visual cycle. While rods are primarily responsible for dim light vision, the ability of cones to function in constant light is essential to human vision and may be facilitated by cone-specific visual cycle pathways. The interphotoreceptor retinoid-binding protein (IRBP) is a proposed retinoid transporter in the visual cycle, but rods in Irbp ( -/- ) mice have a normal visual cycle. However, there is evidence that IRBP has cone-specific functions. Cone electroretinogram (ERG) responses are reduced, despite having cone densities and opsin levels similar to C57Bl/6 (WT) mice. Treatment with 9-cis retinal rescues the cone response in Irbp ( -/- ) mice and shows that retinoid deficiency underlies cone dysfunction. These data indicate that IRBP is essential to normal cone function and demonstrate that differences exist in the visual cycle of rods and cones.
11-顺式视黄醛是视杆和视锥光感受器中的光敏感成分,其在光照下异构化为全反式视黄醛,从而引发视觉反应。为了使光感受器正常发挥功能,全反式视黄醛必须通过视觉循环转化回 11-顺式视黄醛。虽然视杆主要负责暗光视觉,但锥体细胞在恒定光下的功能对于人类视觉至关重要,并且可能通过锥体细胞特异性视觉循环途径得到促进。细胞间视黄醛结合蛋白 (IRBP) 是视觉循环中的一种拟议的视黄醛转运蛋白,但 Irbp(-/-)小鼠的视杆具有正常的视觉循环。然而,有证据表明 IRBP 具有锥体细胞特异性功能。尽管 Irbp(-/-)小鼠的锥体密度和视蛋白水平与 C57Bl/6(WT)小鼠相似,但锥体视网膜电图(ERG)反应减少。用 9-顺式视黄醛治疗可挽救 Irbp(-/-)小鼠的锥体反应,表明视黄醛缺乏是锥体功能障碍的基础。这些数据表明 IRBP 对正常锥体功能至关重要,并证明视杆和锥体的视觉循环存在差异。