来自美国贝勒医学院麦克莱恩生物化学与分子生物学部的Theodore G. Wensel实验室验证了杆状感觉纤毛的3D结构及其在视网膜神经变性中的破坏作用,这篇文章刊登在最新一期《CELL》杂志上。






[Title]Three-Dimensional Architecture of the Rod Sensory Cilium and Its Disruption in Retinal Neurodegeneration

[author]Jared C. Gilliam, Juan T. Chang, Ivette M. Sandoval, Youwen Zhang, Tiansen Li, Steven J. Pittler, Wah Chiu, Theodore G. Wensel

[Abstract]Defects in primary cilia lead to devastating disease because of 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 3D maps of the connecting cilium and adjacent cellular structures of a modified primary cilium, the rod outer segment, from wild-type 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 defects in 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.



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