Pennsylvania College of Optometry, Salus University, 8360 Old York Road, Elkins Park, PA, 19027, USA.
Pflugers Arch. 2021 Sep;473(9):1393-1410. doi: 10.1007/s00424-021-02523-4. Epub 2021 Feb 3.
This article presents a brief overview of the main biochemical and cellular processes involved in regulation of cyclic GMP production in photoreceptors. The main focus is on how the fluctuations of free calcium concentrations in photoreceptors between light and dark regulate the activity of retinal membrane guanylyl cyclase (RetGC) via calcium sensor proteins. The emphasis of the review is on the structure of RetGC and guanylyl cyclase activating proteins (GCAPs) in relation to their functional role in photoreceptors and congenital diseases of photoreceptors. In addition to that, the structure and function of retinal degeneration-3 protein (RD3), which regulates RetGC in a calcium-independent manner, is discussed in detail in connections with its role in photoreceptor biology and inherited retinal blindness.
本文简要概述了参与光感受器中环鸟苷酸(cGMP)产生调节的主要生化和细胞过程。主要关注的是光感受器中游离钙浓度在光暗之间的波动如何通过钙传感器蛋白调节视网膜膜鸟苷酸环化酶(RetGC)的活性。本综述的重点是 RetGC 和鸟苷酸环化酶激活蛋白(GCAPs)的结构与其在光感受器中的功能作用以及光感受器先天性疾病的关系。除此之外,还详细讨论了视网膜变性蛋白 3(RD3)的结构和功能,它以钙非依赖的方式调节 RetGC,并与其在光感受器生物学和遗传性视网膜失明中的作用相关联。