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Routes and machinery of primary cilium biogenesis

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Abstract

Primary cilia are solitary, microtubule-based protrusions of the cell surface that play fundamental roles as photosensors, mechanosensors and biochemical sensors. Primary cilia dysfunction results in a long list of developmental and degenerative disorders that combine to give rise to a large spectrum of human diseases affecting almost any major body organ. Depending on the cell type, primary ciliogenesis is initiated intracellularly, as in fibroblasts, or at the cell surface, as in renal polarized epithelial cells. In this review, we have focused on the routes of primary ciliogenesis placing particular emphasis on the recently described pathway in renal polarized epithelial cells by which the midbody remnant resulting from a previous cell division event enables the centrosome for initiation of primary cilium assembly. The protein machinery implicated in primary cilium formation in epithelial cells, including the machinery best known for its involvement in establishing cell polarity and polarized membrane trafficking, is also discussed.

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Abbreviations

aPKC:

Atypical protein kinase C

BBS:

Bardet–Biedl syndrome

GEF:

Guanine nucleotide exchange factor

GPCR:

G protein-coupled receptor

Hh:

Hedgehog

IFT:

Intraflagellar transport

JBTS:

Joubert syndrome

MDCK:

Madin–Darby canine kidney

MKS:

Meckel syndrome

MEF:

Mouse embryonic fibroblast

NPHP:

Nephronophthisis

PDGF:

Platelet-derived growth factor

RPE:

Retinal pigment epithelial

Smo:

Smoothened

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Acknowledgements

We thank members of M. A. Alonso and I. Correas laboratories for helpful discussions. We also thank Dr. Phil Mason for revising the English language of the manuscript. Research in the laboratory of Miguel A. Alonso is supported by a Grant (BFU2015-67266-R) from the Ministerio de Economía y Competitividad MINECO, Spain, and the Fondo Europeo de Desarrollo Regional (FEDER), European Union. MB-R is the holder of a predoctoral contract from the MINECO.

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  1. Department of Cell Biology and Immunology, Centro de Biología Molecular Severo Ochoa, Consejo Superior de Investigaciones Científicas, Universidad Autónoma de Madrid, Nicolás Cabrera 1, Cantoblanco, 28049, Madrid, Spain

    Miguel Bernabé-Rubio & Miguel A. Alonso

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  1. Miguel Bernabé-Rubio
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Correspondence to Miguel A. Alonso.

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Bernabé-Rubio, M., Alonso, M.A. Routes and machinery of primary cilium biogenesis. Cell. Mol. Life Sci. 74, 4077–4095 (2017). https://doi.org/10.1007/s00018-017-2570-5

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