Open Sesame: How Transition Fibers and the Transition Zone Control Ciliary Composition.
Open Sesame: How Transition Fibers and the Transition Zone Control Ciliary Composition.
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DOI：
10.1101/cshperspect.a028134
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发表时间：
2017-02-01
影响因子：
7.2
通讯作者：
Reiter JF
中科院分区：
文献类型：
--
作者：
Garcia-Gonzalo FR;Reiter JF
纤毛是质膜突起，起细胞推进器或触角的作用。为了执行这些功能，纤毛必须保持一种不同于毗连的细胞质和质膜的成分。纤毛的特殊组成依赖于纤毛门，纤毛门是纤毛底部将纤毛与细胞的其余部分分开的区域。纤毛门的主要结构特征是电子致密的支柱，将微管连接到相邻的膜上。这些结构包括连接远端基底体和睫状膜底部的移行纤维，以及连接移行区内近端轴丝和纤毛膜的Y形连接。过渡纤维和Y链在纤毛发生的早期形成，在纤毛的组装和运输中起着关键作用。因此，许多人类纤毛疾病是由扰乱睫状门功能的突变引起的。
Cilia are plasma membrane protrusions that act as cellular propellers or antennae. To perform these functions, cilia must maintain a composition distinct from those of the contiguous cytosol and plasma membrane. The specialized composition of the cilium depends on the ciliary gate, the region at the ciliary base separating the cilium from the rest of the cell. The ciliary gate’s main structural features are electron dense struts connecting microtubules to the adjacent membrane. These structures include the transition fibers, which connect the distal basal body to the base of the ciliary membrane, and the Y-links, which connect the proximal axoneme and ciliary membrane within the transition zone. Both transition fibers and Y-links form early during ciliogenesis and play key roles in ciliary assembly and trafficking. Accordingly, many human ciliopathies are caused by mutations that perturb ciliary gate function.