wensel

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。

-2012年11月21日《细胞》


中文翻译


【题目】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

【译文】初级纤毛的缺陷会产生重大疾病,这是由于初级纤毛在感觉和发育信号中有重要的作用,但是对纤毛的结构及其形成和维持的机制知之甚少。我们利用低温电子断层扫描技术获得了野生型和遗传缺陷型小鼠的连接纤毛和被修饰的初级纤毛(杆状外节眼锥体)细胞结构的3D图谱。试验结果揭示了纤毛的分子结构,对蛋白的功能有了更加深入的了解。这表明纤毛根丝参与了细胞运输和稳定轴丝的作用。BBSome膜被的缺陷引起纤毛周围的小囊泡靶标的缺陷。由Cngb1基因编码蛋白的缺失可以破坏盘和质膜间的连接。外节眼锥体膜的结构验证了由质膜包裹的基底盘形态发生的模型。

英文原稿


[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.

原文地址

http://www.cell.com/abstract/S0092-8674(12)01301-3

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