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Cholangiocyte Cilia and Basal Bodies

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Fibrocystic Diseases of the Liver

Part of the book series: Clinical Gastroenterology ((CG))

Summary

Primary cilia are nonmotile, tubular organelles extending from the cell plasma membrane. They are important in maintaining normal cell physiology and in many clinical disorders (i.e., cilia-related diseases or ciliopathies) associated with abnormalities in ciliary structure and/or function.

In the intrahepatic bile ducts, primary cilia extend from the cholangiocyte apical plasma membrane into the ductal lumen. Cholangiocyte cilia are sensory organelles that recognize the current state of bile, i.e., its flow, chemical composition, and osmolality, and transmit the related information into intracellular signaling, i.e., function as mechano-, chemo-, and osmosensors. In contrast, in ciliopathies, the structural and/or functional abnormalities of these organelles in cholangiocytes lining liver cysts are associated with activation or down-regulation of several intracellular signaling pathways, in particular, cAMP and [Ca2+]i, leading to hepatic cystogenesis.

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Acknowledgment

This work was supported by the National Institutes of Health (N.F. LaRusso: grant DK 24031), by the PKD Foundation (T.V. Masyuk), and by the Mayo Foundation.

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  1. Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Miles and Shirley Fiterman Center for Digestive Diseases, Rochester, MN, USA

    Tatyana V. Masyuk PhD, Anatoliy I. Masyuk PhD & Nicholas F. LaRusso MD

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  1. Tatyana V. Masyuk PhD
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  1. School of Medicine, Children's Hospital & Regional, University of Washington, Sand Point Way NE. 4800, Seattle, 98105, USA

    Karen F. Murray

  2. St. Paul University Hospital, Harry Hines Blvd. 5939, Dallas, 75390-9258, USA

    Anne M. Larson

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Masyuk, T.V., Masyuk, A.I., LaRusso, N.F. (2010). Cholangiocyte Cilia and Basal Bodies. In: Murray, K., Larson, A. (eds) Fibrocystic Diseases of the Liver. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-524-8_3

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  • DOI: https://doi.org/10.1007/978-1-60327-524-8_3

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