Department of Surgery, Division of Ophthalmology, University of New Mexico, Albuquerque, NM 87131, USA.
Department of Surgery, Division of Ophthalmology, University of New Mexico, Albuquerque, NM 87131, USA; Department of Cell Biology and Physiology, University of New Mexico, Albuquerque, NM 87131, USA.
Prog Retin Eye Res. 2014 Jan;38:1-19. doi: 10.1016/j.preteyeres.2013.08.004. Epub 2013 Oct 14.
Rhodopsin is a key molecular constituent of photoreceptor cells, yet understanding of how it regulates photoreceptor membrane trafficking and biogenesis of light-sensing organelles, the rod outer segments (ROS) is only beginning to emerge. Recently identified sequence of well-orchestrated molecular interactions of rhodopsin with the functional networks of Arf and Rab GTPases at multiple stages of intracellular targeting fits well into the complex framework of the biogenesis and maintenance of primary cilia, of which the ROS is one example. This review will discuss the latest progress in dissecting the molecular complexes that coordinate rhodopsin incorporation into ciliary-targeted carriers with the recruitment and activation of membrane tethering complexes and regulators of fusion with the periciliary plasma membrane. In addition to revealing the fundamental principals of ciliary membrane renewal, recent advances also provide molecular insight into the ways by which disruptions of the exquisitely orchestrated interactions lead to cilia dysfunction and result in human retinal dystrophies and syndromic diseases that affect multiple organs, including the eyes.
视紫红质是光感受器细胞的关键分子组成部分,但对于它如何调节光感受器膜运输和光感受细胞器(棒状外节)的生物发生,人们才刚刚开始了解。最近发现的视紫红质与 Arf 和 Rab GTPases 功能网络在细胞内靶向的多个阶段的协调分子相互作用的序列很好地符合了原发性纤毛发生和维持的复杂框架,而棒状外节就是其中的一个例子。本综述将讨论解析协调视紫红质纳入纤毛靶向载体的分子复合物的最新进展,包括招募和激活膜连接复合物以及与纤毛周质膜融合的调节剂。除了揭示纤毛膜更新的基本原理外,最近的进展还为分子洞察提供了线索,即协调相互作用的破坏如何导致纤毛功能障碍,并导致影响包括眼睛在内的多个器官的人类视网膜营养不良和综合征疾病。