Chen P, Hao W, Rife L, Wang X P, Shen D, Chen J, Ogden T, Van Boemel G B, Wu L, Yang M, Fong H K
Department of Ophthalmology, University of Southern California, Los Angeles, California 90033 USA.
Nat Genet. 2001 Jul;28(3):256-60. doi: 10.1038/90089.
During visual excitation, rhodopsin undergoes photoactivation and bleaches to opsin and all-trans-retinal. To regenerate rhodopsin and maintain normal visual sensitivity, the all-trans isomer must be metabolized and reisomerized to produce the chromophore 11-cis-retinal in biochemical steps that constitute the visual cycle and involve the retinal pigment epithelium (RPE; refs. 3-8). A key step in the visual cycle is isomerization of an all-trans retinoid to 11-cis-retinol in the RPE (refs. 9-11). It could be that the retinochrome-like opsins, peropsin, or the retinal G protein-coupled receptor (RGR) opsin12-16 are isomerases in the RPE. In contrast to visual pigments, RGR is bound predominantly to endogenous all-trans-retinal, and irradiation of RGR in vitro results in stereospecific conversion of the bound all-trans isomer to 11-cis-retinal. Here we show that RGR is involved in the formation of 11-cis-retinal in mice and functions in a light-dependent pathway of the rod visual cycle. Mutations in the human gene encoding RGR are associated with retinitis pigmentosa.
在视觉兴奋过程中,视紫红质发生光激活并漂白为视蛋白和全反式视黄醛。为了再生视紫红质并维持正常的视觉敏感性,全反式异构体必须在构成视觉循环并涉及视网膜色素上皮(RPE;参考文献3 - 8)的生化步骤中进行代谢和重新异构化,以产生发色团11 - 顺式视黄醛。视觉循环中的一个关键步骤是RPE中全反式类视黄醇异构化为11 - 顺式视黄醇(参考文献9 - 11)。可能视黄醛色素样视蛋白、视周蛋白或视网膜G蛋白偶联受体(RGR)视蛋白12 - 16是RPE中的异构酶。与视觉色素不同,RGR主要与内源性全反式视黄醛结合,并且在体外照射RGR会导致结合的全反式异构体立体定向转化为11 - 顺式视黄醛。在这里,我们表明RGR参与小鼠中11 - 顺式视黄醛的形成,并在视杆细胞视觉循环的光依赖途径中发挥作用。编码RGR的人类基因突变与色素性视网膜炎有关。