Primary Cilia in Cystic Kidney Disease

  • Prachee Avasthi
  • Robin L. Maser
  • Pamela V. Tran
Part of the Results and Problems in Cell Differentiation book series (RESULTS, volume 60)

Abstract

Primary cilia are small, antenna-like structures that detect mechanical and chemical cues and transduce extracellular signals. While mammalian primary cilia were first reported in the late 1800s, scientific interest in these sensory organelles has burgeoned since the beginning of the twenty-first century with recognition that primary cilia are essential to human health. Among the most common clinical manifestations of ciliary dysfunction are renal cysts. The molecular mechanisms underlying renal cystogenesis are complex, involving multiple aberrant cellular processes and signaling pathways, while initiating molecular events remain undefined. Autosomal Dominant Polycystic Kidney Disease is the most common renal cystic disease, caused by disruption of polycystin-1 and polycystin-2 transmembrane proteins, which evidence suggests must localize to primary cilia for proper function. To understand how the absence of these proteins in primary cilia may be remediated, we review intracellular trafficking of polycystins to the primary cilium. We also examine the controversial mechanisms by which primary cilia transduce flow-mediated mechanical stress into intracellular calcium. Further, to better understand ciliary function in the kidney, we highlight the LKB1/AMPK, Wnt, and Hedgehog developmental signaling pathways mediated by primary cilia and misregulated in renal cystic disease.

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© Springer International Publishing AG 2017

Open Access This chapter is distributed under the terms of the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.

Authors and Affiliations

  • Prachee Avasthi
    • 1
  • Robin L. Maser
    • 2
    • 3
  • Pamela V. Tran
    • 1
    • 3
  1. 1.Department of Anatomy and Cell BiologyUniversity of Kansas Medical CenterKansas CityUSA
  2. 2.Department of Clinical Laboratory SciencesUniversity of Kansas Medical CenterKansas CityUSA
  3. 3.Kidney InstituteUniversity of Kansas Medical CenterKansas CityUSA

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