Biel M, Seeliger M, Pfeifer A, Kohler K, Gerstner A, Ludwig A, Jaissle G, Fauser S, Zrenner E, Hofmann F
Institut für Pharmakologie und Toxikologie, Technische Universität München, Biedersteiner Strasse 29, 80802 Munich, Germany.
Proc Natl Acad Sci U S A. 1999 Jun 22;96(13):7553-7. doi: 10.1073/pnas.96.13.7553.
Two types of photoreceptors, rods and cones, coexist in the vertebrate retina. An in-depth analysis of the retinal circuitry that transmits rod and cone signals has been hampered by the presence of intimate physical and functional connections between rod and cone pathways. By deleting the cyclic nucleotide-gated channel CNG3 we have generated a mouse lacking any cone-mediated photoresponse. In contrast, the rod pathway is completely intact in CNG3-deficient mice. The functional loss of cone function correlates with a progressive degeneration of cone photoreceptors but not of other retinal cell types. CNG3-deficient mice provide an animal model to dissect unequivocally the contribution of rod and cone pathways for normal retinal function.
脊椎动物的视网膜中同时存在两种光感受器,即视杆细胞和视锥细胞。视杆细胞和视锥细胞通路之间存在紧密的物理和功能连接,这阻碍了对传递视杆细胞和视锥细胞信号的视网膜神经回路进行深入分析。通过删除环核苷酸门控通道CNG3,我们培育出了一种缺乏任何视锥细胞介导的光反应的小鼠。相比之下,在缺乏CNG3的小鼠中,视杆细胞通路完全完整。视锥细胞功能的丧失与视锥光感受器的进行性退化相关,但与其他视网膜细胞类型无关。缺乏CNG3的小鼠提供了一个动物模型,可明确剖析视杆细胞和视锥细胞通路对正常视网膜功能的贡献。