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CP110 and its network of partners coordinately regulate cilia assembly
- Source :
- Cilia
- Publication Year :
- 2013
- Publisher :
- BioMed Central, 2013.
-
Abstract
- Cilia are hair-like protrusions found at the surface of most eukaryotic cells. They can be divided into two types, motile and non-motile. Motile cilia are found in a restricted number of cell types, are generally present in large numbers, and beat in a coordinated fashion to generate fluid flow or locomotion. Non-motile or primary cilia, on the other hand, are detected in many different cell types, appear once per cell, and primarily function to transmit signals from the extracellular milieu to the cell nucleus. Defects in cilia formation, function, or maintenance are known to cause a bewildering set of human diseases, or ciliopathies, typified by retinal degeneration, renal failure and cystic kidneys, obesity, liver dysfunction, and neurological disorders. A common denominator between motile and primary cilia is their structural similarity, as both types of cilia are composed of an axoneme, the ciliary backbone that is made up of microtubules emanating from a mother centriole/basal body anchored to the cell membrane, surrounded by a ciliary membrane continuous with the plasma membrane. This structural similarity is indicative of a universal mechanism of cilia assembly involving a common set of molecular players and a sophisticated, highly regulated series of molecular events. In this review, we will mainly focus on recent advances in our understanding of the regulatory mechanisms underlying cilia assembly, with special attention paid to the centriolar protein, CP110, its interacting partner Cep290, and the various downstream molecular players and events leading to intraflagellar transport (IFT), a process that mediates the bidirectional movement of protein cargos along the axoneme and that is essential for cilia formation and maintenance.
- Subjects :
- Axoneme
0303 health sciences
Undulipodium
Centrosomes
Ciliogenesis
Cilium
Cell Biology
Review
Cep290
IFT
Biology
Cell biology
03 medical and health sciences
0302 clinical medicine
Intraflagellar transport
Protein network
Motile cilium
Basal body
CP110
Cilia
Ciliary membrane
BBSome
030217 neurology & neurosurgery
030304 developmental biology
Subjects
Details
- Language :
- English
- ISSN :
- 20462530
- Volume :
- 2
- Database :
- OpenAIRE
- Journal :
- Cilia
- Accession number :
- edsair.doi.dedup.....3f0d6045451e3a0591fabc0b579b2fc7