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2016 ; 5
(1
): ä Nephropedia Template TP
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Cellular Mechanisms of Ciliary Length Control
#MMPMID26840332
Keeling J
; Tsiokas L
; Maskey D
Cells
2016[Jan]; 5
(1
): ä PMID26840332
show ga
Cilia and flagella are evolutionarily conserved, membrane-bound,
microtubule-based organelles on the surface of most eukaryotic cells. They play
important roles in coordinating a variety of signaling pathways during growth,
development, cell mobility, and tissue homeostasis. Defects in ciliary structure
or function are associated with multiple human disorders called ciliopathies.
These diseases affect diverse tissues, including, but not limited to the eyes,
kidneys, brain, and lungs. Many processes must be coordinated simultaneously in
order to initiate ciliogenesis. These include cell cycle, vesicular trafficking,
and axonemal extension. Centrioles play a central role in both cell cycle
progression and ciliogenesis, making the transition between basal bodies and
mitotic spindle organizers integral to both processes. The maturation of
centrioles involves a functional shift from cell division toward cilium
nucleation which takes place concurrently with its migration and fusion to the
plasma membrane. Several proteinaceous structures of the distal appendages in
mother centrioles are required for this docking process. Ciliary assembly and
maintenance requires a precise balance between two indispensable processes; so
called assembly and disassembly. The interplay between them determines the length
of the resulting cilia. These processes require a highly conserved transport
system to provide the necessary substances at the tips of the cilia and to
recycle ciliary turnover products to the base using a based microtubule
intraflagellar transport (IFT) system. In this review; we discuss the stages of
ciliogenesis as well as mechanisms controlling the lengths of assembled cilia.
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