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Ciliar functions in the nephron

  • Ion Channels, Receptors and Transporters
  • Published:
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Abstract

The primary cilium is a microtubule-based nonmotile organelle that is found on most cells in the mammalian body. Once regarded as a vestigial organelle, it has been recently shown to play unforeseen roles in mammalian physiology and tissue homeostasis. In kidney epithelial cells, the primary cilium plays a fundamental role in tubule organization and function and it is now considered to serve as a versatile mechanosensor and chemosensor. Diseases related to kidney primary cilia include autosomal polycystic kidney disease, recessive polycystic kidney disease, Bardet–Biedl syndrome, and nephronophthisis. Multiple proteins whose functions are disrupted in cystic kidney diseases have been localized in the primary cilium. This review provides a general introduction to the cell biology and function of renal primary cilia and an overview of cilia-related kidney diseases.

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Acknowledgements

We are grateful to the ANR-05-Neuro, ANR-05-PCOD, ANR-2008-Genopat, ANR 2008-MNP, ARCInca-2006, Fondation pour la Recherche Médicale, Fondation Schlumberger, Action Concertée Incitative Jeunes Chercheurs, UPSA, CNRS, and French Ministry for the support.

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  1. Centre de Recherche en Neurobiologie et Neurophysiologie de Marseille (CRN2M), CNRS, UMR 6231, Equipe “Canaux Ioniques et Transduction Sensorielle”, IFR-11 Jean Roche-Faculté de Médecine-Secteur Nord, Université de la Méditerranée, CS80011, Bd. Pierre Dramard, 13344, Marseille Cedex 15, France

    Lise Rodat-Despoix & Patrick Delmas

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Rodat-Despoix, L., Delmas, P. Ciliar functions in the nephron. Pflugers Arch - Eur J Physiol 458, 179–187 (2009). https://doi.org/10.1007/s00424-008-0632-0

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