Journal
CELLULAR AND MOLECULAR LIFE SCIENCES
Volume 73, Issue 1, Pages 119-127Publisher
SPRINGER BASEL AG
DOI: 10.1007/s00018-015-2058-0
Keywords
Cilia; Flagella; Primary cilium; Ciliary pore complex; Nuclear pore complex; Nucleoporin; Intraflagellar transport; Transition zone
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Funding
- Japan Society for the Promotion of Science
- NIH [RO1GM070862]
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Cilia and flagella play important roles in cell motility and cell signaling. These functions require that the cilium establishes and maintains a unique lipid and protein composition. Recent work indicates that a specialized region at the base of the cilium, the transition zone, serves as both a barrier to entry and a gate for passage of select components. For at least some cytosolic proteins, the barrier and gate functions are provided by a ciliary pore complex (CPC) that shares molecular and mechanistic properties with nuclear gating. Specifically, nucleoporins of the CPC limit the diffusional entry of cytosolic proteins in a size-dependent manner and enable the active transport of large molecules and complexes via targeting signals, importins, and the small G protein Ran. For membrane proteins, the septin protein SEPT2 is part of the barrier to entry whereas the gating function is carried out and/or regulated by proteins associated with ciliary diseases (ciliopathies) such as nephronophthisis, Meckel-Gruber syndrome and Joubert syndrome. Here, we discuss the evidence behind these models of ciliary gating as well as the similarities to and differences from nuclear gating.
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