Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2015 Mar 31:4:5.
doi: 10.1186/s13630-015-0014-0. eCollection 2015.

The more we know, the more we have to discover: an exciting future for understanding cilia and ciliopathies

Alexandre Benmerah  1 , Bénédicte Durand  2 , Rachel H Giles  3 , Tess Harris  4 , Linda Kohl  5 , Christine Laclef  6 , Sigolène M Meilhac  7 , Hannah M Mitchison  8 , Lotte B Pedersen  9 , Ronald Roepman  10 , Peter Swoboda  11 , Marius Ueffing  12 , Philippe Bastin  13
Affiliations

The more we know, the more we have to discover: an exciting future for understanding cilia and ciliopathies

Alexandre Benmerah et al. Cilia. .

Abstract

The Cilia 2014 conference was organised by four European networks: the Ciliopathy Alliance, the Groupement de Recherche CIL, the Nordic Cilia and Centrosome Network and the EU FP7 programme SYSCILIA. More than 400 delegates from 27 countries gathered at the Institut Pasteur conference centre in Paris, including 30 patients and patient representatives. The meeting offered a unique opportunity for exchange between different scientific and medical communities. Major highlights included new discoveries about the roles of motile and immotile cilia during development and homeostasis, the mechanism of cilium construction, as well as progress in diagnosis and possible treatment of ciliopathies. The contributions to the cilia field of flagellated infectious eukaryotes and of systems biology were also presented.

Keywords: Basal bodies; Centrioles; Cilia; Ciliopathies; Development; Flagella; Hedgehog; Intraflagellar; Signalling; Transport.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Scanning electron micrographs of an early mouse embryo, with three somite pairs, viewed from the posterior. (A) The node (white arrow) is clearly visible as a depression with a rounded posterior that continues forward opening out into the notochord. The cells surrounding the top of the node are often called the crown cells, and these have been implicated in responding to leftward nodal flow. Left (L) and right (R) sides are indicated. (B) A higher magnification of the node shown in panel (A). At this magnification, the presence of a single cilium on each of the cells within the pit of the node is visible. These cilia beat in a circular manner, but due to a posterior tilt, they drive a leftward flow of fluid in the node. It is this leftward nodal flow that normally establishes the direction of the left-right axis (credit: Dominic Norris).
Figure 2
Figure 2
The primary cilium. Scanning electron micrograph of the buccopharyngeal epithelium of E9.5 mouse embryo, showing a central primary cilium emerging from the apical surface surrounded by microvilli (credit: Christine Laclef).
Figure 3
Figure 3
Example of signalling at the primary cilium. (A) TGFβ/BMP signalling at the primary cilium. (B) TGFβ-RI (green) and P-smad2/3 (red) are recruited at the base of the primary cilium (blue) in TGFβ1-stimulated human foreskin fibroblasts (credit: [17]).
Figure 4
Figure 4
Example of protein dynamics at the cilium. A centriolar satellite protein (green) re-localises upon activation of an innate immune response in ciliated (red) RPE-cells. A. Unstimulated cell. B. Stimulated cell (Credit: Jens Andersen).
See this image and copyright information in PMC

References

    1. Bloodgood RA. From central to rudimentary to primary: the history of an underappreciated organelle whose time has come. The primary cilium. Methods Cell Biol. 2009;94:3–52. - PubMed
    1. Kozminski KG, Johnson KA, Forscher P, Rosenbaum JL. A motility in the eukaryotic flagellum unrelated to flagellar beating. Proc Natl Acad Sci U S A. 1993;90(12):5519–5523. doi: 10.1073/pnas.90.12.5519. - DOI - PMC - PubMed
    1. Cole DG, Diener DR, Himelblau AL, Beech PL, Fuster JC, Rosenbaum JL. Chlamydomonas kinesin-II-dependent Intraflagellar Transport (IFT): IFT particles contain proteins required for ciliary assembly in Caenorhabditis elegans sensory neurons. J Cell Biol. 1998;141(4):993–1008. doi: 10.1083/jcb.141.4.993. - DOI - PMC - PubMed
    1. Pazour GJ, Dickert BL, Vucica Y, Seeley ES, Rosenbaum JL, Witman GB, Cole DG. Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene Tg737, are required for assembly of cilia and flagella. J Cell Biol. 2000;151(3):709–718. doi: 10.1083/jcb.151.3.709. - DOI - PMC - PubMed
    1. Qin H, Rosenbaum JL, Barr MM. An autosomal recessive polycystic kidney disease gene homolog is involved in intraflagellar transport in C. elegans ciliated sensory neurons. Curr Biol. 2001;11(6):457–461. doi: 10.1016/S0960-9822(01)00122-1. - DOI - PubMed

LinkOut - more resources