Open Sesame: How Transition Fibers and the Transition Zone Control Ciliary Composition

被引:197
作者
Garcia-Gonzalo, Francesc R. [1 ,2 ]
Reiter, Jeremy F. [3 ,4 ]
机构
[1] Univ Autonoma Madrid, Fac Med, Dept Bioquim, Madrid 28029, Spain
[2] Univ Autonoma Madrid, CSIC, Inst Invest Biomed Alberto Sols UAM, Madrid 28029, Spain
[3] Univ Calif San Francisco, Dept Biochem & Biophys, San Francisco, CA 94158 USA
[4] Univ Calif San Francisco, Cardiovasc Res Inst, San Francisco, CA 94158 USA
关键词
PROTEIN-COUPLED RECEPTORS; BIEDL-SYNDROME PROTEINS; BASAL BODY PROTEIN; INTRAFLAGELLAR TRANSPORT; JOUBERT-SYNDROME; MEMBRANE-PROTEINS; MOTHER CENTRIOLE; PDE-DELTA; SIGNALING PROTEINS; TUBULIN TRANSPORT;
D O I
10.1101/cshperspect.a028134
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
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.
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页数:20
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