Three-dimensional architecture of the rod sensory cilium and its disruption in retinal neurodegeneration.
Three-dimensional architecture of the rod sensory cilium and its disruption in retinal neurodegeneration.
复制标题
DOI：
10.1016/j.cell.2012.10.038
复制
发表时间：
2012-11-21
期刊：
影响因子：
64.5
通讯作者：
Wensel TG
中科院分区：
文献类型：
--
作者：
Gilliam JC;Chang JT;Sandoval IM;Zhang Y;Li T;Pittler SJ;Chiu W;Wensel TG
初级纤毛的缺陷由于其在感觉和发育信号中的作用而导致毁灭性疾病，但关于纤毛结构及其形成和维持的机制还有很多未知之处。我们使用冷冻电子断层扫描，以获得三维地图的连接纤毛和相邻的细胞结构的修改后的初级纤毛，杆外段，从野生型和遗传缺陷小鼠。这些结果揭示了纤毛的分子结构，并提供了对蛋白质功能的见解。他们认为纤毛小根参与细胞运输并稳定轴丝。BBSome膜涂层中的缺陷导致囊泡靶向纤毛基部附近。由Cngb1基因编码的蛋白质的丢失破坏了圆盘和质膜之间的联系。外节膜的结构支持一个模型的磁盘形态，其中基底磁盘被质膜包围。
Defects in primary cilia lead to devastating disease due to their roles in sensation and developmental signaling, but much is unknown about ciliary structure and mechanisms of their formation and maintenance. We used cryo-electron tomography to obtain three-dimensional maps of the connecting cilium and adjacent cellular structures of a modified primary cilium, the rod outer segment, from wildtype and genetically defective mice. The results reveal the molecular architecture of the cilium and provide insights into protein functions. They suggest that the ciliary rootlet is involved in cellular transport and stabilizes the axoneme. A defect in the BBSome membrane coat caused vesicle targeting near the base of the cilium. Loss of the proteins encoded by the Cngb1 gene disrupted links between the disk and plasma membranes. The structures of the outer segment membranes support a model for disk morphogenesis in which basal disks are enveloped by the plasma membrane.
登录
查看更多内容
影响因子：
7.8
作者：
Anderson, R G
通讯作者：
Anderson, R G
影响因子：
3.4
作者：
Mayer, Ulrich;Kueller, Alexander;Moehrlen, Frank
通讯作者：
Moehrlen, Frank
影响因子：
16.2
作者：
KORSCHEN, HG;ILLING, M;MOLDAY, RS
通讯作者：
MOLDAY, RS
影响因子：
5.3
作者：
Eichers, Erica R.;Abd-El-Barr, Muhammad M.;Lupski, James R.
通讯作者：
Lupski, James R.
影响因子：
64.5
作者：
Chuang, Jen-Zen;Zhao, Yu;Sung, Ching-Hwa
通讯作者：
Sung, Ching-Hwa