Primary ciliogenesis requires the distal appendage component Cep123.

Primary ciliogenesis requires the distal appendage component Cep123.
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DOI:
10.1242/bio.20134457
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发表时间:
2013-06-15
期刊:
影响因子:
2.4
通讯作者:
Bornens M
Bornens M
中科院分区:
生物学4区
文献类型:
--
作者:
Sillibourne JE;Hurbain I;Grand-Perret T;Goud B;Tran P;Bornens M

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初级纤毛的形成是在母中心粒的远端以高度协调的方式开始的。这需要用纤毛小泡覆盖母中心粒的远端,并将基体(母中心粒)锚定到细胞皮质,这两个过程都是由远端附件介导的。在这里,我们证明了远端附件蛋白Cep123(Cep89/CCDC123)是初级纤毛组装所必需的,而不是维持所需的。在没有Cep123的情况下,纤毛小泡的形成失败,这表明它在原发纤毛发生的早期阶段起作用。与这种作用一致,Cep123与中心粒卫星蛋白PCM-1、CEP290和OFD1相互作用,所有这些蛋白都在原发纤毛发生中发挥作用。这些相互作用是由Cep123(400-783)C-末端的一个结构域介导的,该结构域与远端附件靶向结构域(500-600)重叠。总之,这些数据暗示Cep123是初级纤毛发生途径中的一个新的参与者,并扩展了远端附件在这一过程中的作用。
Primary cilium formation is initiated at the distal end of the mother centriole in a highly co-ordinated manner. This requires the capping of the distal end of the mother centriole with a ciliary vesicle and the anchoring of the basal body (mother centriole) to the cell cortex, both of which are mediated by the distal appendages. Here, we show that the distal appendage protein Cep123 (Cep89/CCDC123) is required for the assembly, but not the maintenance, of a primary cilium. In the absence of Cep123 ciliary vesicle formation fails, suggesting that it functions in the early stages of primary ciliogenesis. Consistent with such a role, Cep123 interacts with the centriolar satellite proteins PCM-1, Cep290 and OFD1, all of which play a role in primary ciliogenesis. These interactions are mediated by a domain in the C-terminus of Cep123 (400–783) that overlaps the distal appendage-targeting domain (500–600). Together, the data implicate Cep123 as a new player in the primary ciliogenesis pathway and expand upon the role of the distal appendages in this process.
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