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Mol Cells. 2017 Apr;40(4):243-253. doi: 10.14348/molcells.2017.0054. Epub 2017 Apr 12.

The Ciliary Transition Zone: Finding the Pieces and Assembling the Gate.

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Lunenfeld-Tanenbaum Research Institute, Mount Sinai Hospital, 600 University Avenue, Toronto, ON M5G 1X5, Canada.
Department of Molecular Genetics, University of Toronto, Toronto, ON M5S 1A8, Canada.


Eukaryotic cilia are organelles that project from the surface of cells to fulfill motility and sensory functions. In vertebrates, the functions of both motile and immotile cilia are critical for embryonic development and adult tissue homeostasis. Importantly, a multitude of human diseases is caused by abnormal cilia biogenesis and functions which rely on the compartmentalization of the cilium and the maintenance of its protein composition. The transition zone (TZ) is a specialized ciliary domain present at the base of the cilium and is part of a gate that controls protein entry and exit from this organelle. The relevance of the TZ is highlighted by the fact that several of its components are coded by ciliopathy genes. Here we review recent developments in the study of TZ proteomes, the mapping of individual components to the TZ structure and the establishment of the TZ as a lipid gate.


centriole; centrosome; cilia; transition zone

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