Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.

Site Tools

  • AAAS
  • Subscribe
  • Feedback

Site Search

Search Advanced

Science 4 August 2006:
Vol. 313. no. 5787, pp. 629 - 633
DOI: 10.1126/science.1124534
Prev | Table of Contents | Next

Review

The Primary Cilium as the Cell's Antenna: Signaling at a Sensory Organelle

Veena Singla and Jeremy F. Reiter*

Almost every vertebrate cell has a specialized cell surface projection called a primary cilium. Although these structures were first described more than a century ago, the full scope of their functions remains poorly understood. Here, we review emerging evidence that in addition to their well-established roles in sight, smell, and mechanosensation, primary cilia are key participants in intercellular signaling. This new appreciation of primary cilia as cellular antennae that sense a wide variety of signals could help explain why ciliary defects underlie such a wide range of human disorders, including retinal degeneration, polycystic kidney disease, Bardet-Biedl syndrome, and neural tube defects.

Program in Developmental and Stem Cell Biology, and Diabetes Center, University of California, San Francisco, CA 94143–0525, USA.

* To whom correspondence should be addressed. E-mail: jreiter{at}diabetes.ucsf.edu

Read the Full Text


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
2008 Homer W. Smith Award: Insights into the Pathogenesis of Polycystic Kidney Disease from Gene Discovery.
P. C. Harris (2009)
J. Am. Soc. Nephrol. 20, 1188-1198
   Abstract »    Full Text »    PDF »
Inactivation of Chibby affects function of motile airway cilia.
V. A. Voronina, K.-I. Takemaru, P. Treuting, D. Love, B. R. Grubb, A. M. Hajjar, A. Adams, F.-Q. Li, and R. T. Moon (2009)
J. Cell Biol. 185, 225-233
   Abstract »    Full Text »    PDF »
A Mouse Model for Meckel Syndrome Type 3.
S. A. Cook, G. B. Collin, R. T. Bronson, J. K. Naggert, D. P. Liu, E. C. Akeson, and M. T. Davisson (2009)
J. Am. Soc. Nephrol. 20, 753-764
   Abstract »    Full Text »    PDF »
Primary cilia regulate Shh activity in the control of molar tooth number.
A. Ohazama, C. J. Haycraft, M. Seppala, J. Blackburn, S. Ghafoor, M. Cobourne, D. C. Martinelli, C.-M. Fan, R. Peterkova, H. Lesot, et al. (2009)
Development 136, 897-903
   Abstract »    Full Text »    PDF »
Functional interactions between the ciliopathy-associated Meckel syndrome 1 (MKS1) protein and two novel MKS1-related (MKSR) proteins.
N. J. Bialas, P. N. Inglis, C. Li, J. F. Robinson, J. D. K. Parker, M. P. Healey, E. E. Davis, C. D. Inglis, T. Toivonen, D. C. Cottell, et al. (2009)
J. Cell Sci. 122, 611-624
   Abstract »    Full Text »    PDF »
ATP and adenosine in the local regulation of water transport and homeostasis by the kidney.
T. Rieg and V. Vallon (2009)
Am J Physiol Regulatory Integrative Comp Physiol 296, R419-R427
   Abstract »    Full Text »    PDF »
Localization of Inv in a distinctive intraciliary compartment requires the C-terminal ninein-homolog-containing region.
D. Shiba, Y. Yamaoka, H. Hagiwara, T. Takamatsu, H. Hamada, and T. Yokoyama (2009)
J. Cell Sci. 122, 44-54
   Abstract »    Full Text »    PDF »
Deciliation Is Associated with Dramatic Remodeling of Epithelial Cell Junctions and Surface Domains.
C. E. Overgaard, K. M. Sanzone, K. S. Spiczka, D. R. Sheff, A. Sandra, and C. Yeaman (2009)
Mol. Biol. Cell 20, 102-113
   Abstract »    Full Text »    PDF »
Energy In, Energy Out, and the Effects of Obesity-Related Genes.
R. L. Leibel (2008)
N. Engl. J. Med. 359, 2603-2604
   Full Text »    PDF »
Grueneberg Ganglion Cells Mediate Alarm Pheromone Detection in Mice.
J. Brechbuhl, M. Klaey, and M.-C. Broillet (2008)
Science 321, 1092-1095
   Abstract »    Full Text »    PDF »
The Subcellular Localization of TRPP2 Modulates Its Function.
X. Fu, Y. Wang, N. Schetle, H. Gao, M. Putz, G. von Gersdorff, G. Walz, and A. G. Kramer-Zucker (2008)
J. Am. Soc. Nephrol. 19, 1342-1351
   Abstract »    Full Text »    PDF »
LRRC50, a Conserved Ciliary Protein Implicated in Polycystic Kidney Disease.
E. van Rooijen, R. H. Giles, E. E. Voest, C. van Rooijen, S. Schulte-Merker, and F. J. van Eeden (2008)
J. Am. Soc. Nephrol. 19, 1128-1138
   Full Text »    PDF »
Polycystin-1 Regulates Skeletogenesis through Stimulation of the Osteoblast-specific Transcription Factor RUNX2-II.
Z. Xiao, S. Zhang, B. S. Magenheimer, J. Luo, and L. D. Quarles (2008)
J. Biol. Chem. 283, 12624-12634
   Abstract »    Full Text »    PDF »
Autosomal Dominant Polycystic Kidney Disease Is Associated With an Increased Prevalence of Radiographic Bronchiectasis.
J. A. Driscoll, S. Bhalla, H. Liapis, A. Ibricevic, and S. L. Brody (2008)
Chest 133, 1181-1188
   Abstract »    Full Text »    PDF »
Identification of Ciliary Localization Sequences within the Third Intracellular Loop of G Protein-coupled Receptors.
N. F. Berbari, A. D. Johnson, J. S. Lewis, C. C. Askwith, and K. Mykytyn (2008)
Mol. Biol. Cell 19, 1540-1547
   Abstract »    Full Text »    PDF »
Bardet-Biedl syndrome proteins are required for the localization of G protein-coupled receptors to primary cilia.
N. F. Berbari, J. S. Lewis, G. A. Bishop, C. C. Askwith, and K. Mykytyn (2008)
PNAS 105, 4242-4246
   Abstract »    Full Text »    PDF »
Human embryonic stem cells in culture possess primary cilia with hedgehog signaling machinery.
E. N. Kiprilov, A. Awan, R. Desprat, M. Velho, C. A. Clement, A. G. Byskov, C. Y. Andersen, P. Satir, E. E. Bouhassira, S. T. Christensen, et al. (2008)
J. Cell Biol. 180, 897-904
   Abstract »    Full Text »    PDF »
Deciphering the Endothelial Shear Stress Sensor.
R. E. Poelmann, K. Van der Heiden, A. Gittenberger-de Groot, and B. P. Hierck (2008)
Circulation 117, 1124-1126
   Full Text »    PDF »
Hypothalamic hamartomas and hedgehogs: Not a laughing matter.
S. J. Pleasure and R. Guerrini (2008)
Neurology 70, 588-589
   Full Text »    PDF »
The Conserved Proteins CHE-12 and DYF-11 Are Required for Sensory Cilium Function in Caenorhabditis elegans.
T. Bacaj, Y. Lu, and S. Shaham (2008)
Genetics 178, 989-1002
   Abstract »    Full Text »    PDF »
Tissue expression patterns identify mouse cilia genes.
T. S. McClintock, C. E. Glasser, S. C. Bose, and D. A. Bergman (2008)
Physiol Genomics 32, 198-206
   Abstract »    Full Text »    PDF »
The Role of Shear Stress on ET-1, KLF2, and NOS-3 Expression in the Developing Cardiovascular System of Chicken Embryos in a Venous Ligation Model.
B. C. W. Groenendijk, K. Van der Heiden, B. P. Hierck, and R. E. Poelmann (2007)
Physiology 22, 380-389
   Abstract »    Full Text »    PDF »
Making sense of cilia and flagella.
R. D. Sloboda and J. L. Rosenbaum (2007)
J. Cell Biol. 179, 575-582
   Abstract »    Full Text »    PDF »
A Heterozygous Mutation Disrupting the SPAG16 Gene Results in Biochemical Instability of Central Apparatus Components of the Human Sperm Axoneme.
Z. Zhang, M. A Zariwala, M. M Mahadevan, P. Caballero-Campo, X. Shen, E. Escudier, B. Duriez, A.-M. Bridoux, M. Leigh, G. L Gerton, et al. (2007)
Biol Reprod 77, 864-871
   Abstract »    Full Text »    PDF »
Cep164, a novel centriole appendage protein required for primary cilium formation.
S. Graser, Y.-D. Stierhof, S. B. Lavoie, O. S. Gassner, S. Lamla, M. Le Clech, and E. A. Nigg (2007)
J. Cell Biol. 179, 321-330
   Abstract »    Full Text »    PDF »
STAM and Hrs Down-Regulate Ciliary TRP Receptors.
J. Hu, S. G. Wittekind, and M. M. Barr (2007)
Mol. Biol. Cell 18, 3277-3289
   Abstract »    Full Text »    PDF »
Primary cilia mediate mechanosensing in bone cells by a calcium-independent mechanism.
A. M. D. Malone, C. T. Anderson, P. Tummala, R. Y. Kwon, T. R. Johnston, T. Stearns, and C. R. Jacobs (2007)
PNAS 104, 13325-13330
   Abstract »    Full Text »    PDF »
The Proteome of the Mouse Photoreceptor Sensory Cilium Complex.
Q. Liu, G. Tan, N. Levenkova, T. Li, E. N. Pugh Jr., J. J. Rux, D. W. Speicher, and E. A. Pierce (2007)
Mol. Cell. Proteomics 6, 1299-1317
   Abstract »    Full Text »    PDF »
Flagellar Elongation and Gene Expression in Chlamydomonas reinhardtii.
G. Periz, D. Dharia, S. H. Miller, and L. R. Keller (2007)
Eukaryot. Cell 6, 1411-1420
   Abstract »    Full Text »    PDF »
Functional dissection of Rab GTPases involved in primary cilium formation.
S.-i. Yoshimura, J. Egerer, E. Fuchs, A. K. Haas, and F. A. Barr (2007)
J. Cell Biol. 178, 363-369
   Abstract »    Full Text »    PDF »
Patched1 Regulates Hedgehog Signaling at the Primary Cilium.
R. Rohatgi, L. Milenkovic, and M. P. Scott (2007)
Science 317, 372-376
   Abstract »    Full Text »    PDF »
Conditional Kif3a ablation causes abnormal hedgehog signaling topography, growth plate dysfunction, and excessive bone and cartilage formation during mouse skeletogenesis.
E. Koyama, B. Young, M. Nagayama, Y. Shibukawa, M. Enomoto-Iwamoto, M. Iwamoto, Y. Maeda, B. Lanske, B. Song, R. Serra, et al. (2007)
Development 134, 2159-2169
   Abstract »    Full Text »    PDF »
Polarity reveals intrinsic cell chirality.
J. Xu, A. Van Keymeulen, N. M. Wakida, P. Carlton, M. W. Berns, and H. R. Bourne (2007)
PNAS 104, 9296-9300
   Abstract »    Full Text »    PDF »
Mutation in CEP290 Discovered for Cat Model of Human Retinal Degeneration.
M. Menotti-Raymond, V. A. David, A. A. Schaffer, R. Stephens, D. Wells, R. Kumar-Singh, S. J. O'Brien, and K. Narfstrom (2007)
J. Hered.
   Abstract »    Full Text »    PDF »
Role of Primary Cilia in the Pathogenesis of Polycystic Kidney Disease.
B. K. Yoder (2007)
J. Am. Soc. Nephrol. 18, 1381-1388
   Abstract »    Full Text »    PDF »
Sensory Ciliogenesis in Caenorhabditis elegans: Assignment of IFT Components into Distinct Modules Based on Transport and Phenotypic Profiles.
G. Ou, M. Koga, O. E. Blacque, T. Murayama, Y. Ohshima, J. C. Schafer, C. Li, B. K. Yoder, M. R. Leroux, and J. M. Scholey (2007)
Mol. Biol. Cell 18, 1554-1569
   Abstract »    Full Text »    PDF »
Tissue/planar cell polarity in vertebrates: new insights and new questions.
Y. Wang and J. Nathans (2007)
Development 134, 647-658
   Abstract »    Full Text »    PDF »
On the Horizon From the ORS.
G. Gibson and D. Fyhrie (2007)
J. Am. Acad. Ortho. Surg. 15, 72-74
   Full Text »    PDF »
Cystic Fibrosis and Other Respiratory Diseases of Impaired Mucus Clearance.
A. Livraghi and S. H. Randell (2007)
Toxicol Pathol 35, 116-129
   Abstract »    Full Text »    PDF »
Roles for Cilia in Bone Cells?: New results sharpen the focus on mechanosensory mechanisms.
N. A. Andrews (2006)
IBMS BoneKEy 3, 7-10
   Full Text »    PDF »
Meeting Report from the 28th Annual Meeting of the American Society for Bone and Mineral Research.
E. Schipani, S. Ferrari, N. S. Datta, L. K. McCauley, A. Vignery, T. Bellido, G. J. Strewler, C. H. Turner, Y. Jiang, and E. Seeman (2006)
IBMS BoneKEy 3, 14-50
   Full Text »    PDF »
Mechanosensation and Transduction in Osteocytes..
L. F. Bonewald (2006)
IBMS BoneKEy 3, 7-15
   Abstract »    Full Text »    PDF »


To Advertise     Find Products

Science. ISSN 0036-8075 (print), 1095-9203 (online)