Cellular and Molecular Life Sciences

, Volume 75, Issue 9, pp 1521–1540 | Cite as

Primary cilia proteins: ciliary and extraciliary sites and functions

  • Kiet Hua
  • Russell J. Ferland


Primary cilia are immotile organelles known for their roles in development and cell signaling. Defects in primary cilia result in a range of disorders named ciliopathies. Because this organelle can be found singularly on almost all cell types, its importance extends to most organ systems. As such, elucidating the importance of the primary cilium has attracted researchers from all biological disciplines. As the primary cilia field expands, caution is warranted in attributing biological defects solely to the function of this organelle, since many of these “ciliary” proteins are found at other sites in cells and likely have non-ciliary functions. Indeed, many, if not all, cilia proteins have locations and functions outside the primary cilium. Extraciliary functions are known to include cell cycle regulation, cytoskeletal regulation, and trafficking. Cilia proteins have been observed in the nucleus, at the Golgi apparatus, and even in immune synapses of T cells (interestingly, a non-ciliated cell). Given the abundance of extraciliary sites and functions, it can be difficult to definitively attribute an observed phenotype solely to defective cilia rather than to some defective extraciliary function or a combination of both. Thus, extraciliary sites and functions of cilia proteins need to be considered, as well as experimentally determined. Through such consideration, we will understand the true role of the primary cilium in disease as compared to other cellular processes’ influences in mediating disease (or through a combination of both). Here, we review a compilation of known extraciliary sites and functions of “cilia” proteins as a means to demonstrate the potential non-ciliary roles for these proteins.


Primary cilia Extraciliary Ciliopathy 



We thank Julia Nalwalk for helpful discussions and editing of our manuscript. This work was supported in part by an NIH/NINDS R01NS092062 Grant to RJF.


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Neuroscience and Experimental TherapeuticsAlbany Medical CollegeAlbanyUSA
  2. 2.Department of NeurologyAlbany Medical CollegeAlbanyUSA

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