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Ion Channels and Transporters of Epithelia in Health and Disease pp 747–771Cite as

ADPKD Channels: The Polycystins

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Part of the book series: Physiology in Health and Disease ((PIHD))

Abstract

The initial members of the polycystin family were first described and characterized as the proteins mutated in a common genetic disease, autosomal dominant polycystic kidney disease (ADPKD). Over the past few decades, these proteins, polycystins 1 and 2 (PC1, PC2), have been found to have a variety of cellular functions in multiple intracellular locations. In addition, other homologous proteins have been identified and linked to the transient receptor potential family of channels. PC1 and PC2 or homologous family members form mechanosensitive Ca2+ channels in the primary cilia. Elsewhere in the cell, the PCs may form Ca2+ channels that are gated by other stimuli and each of the PCs appears to play a variety of functional roles in multiple cellular pathways. While much is currently known about the polycystins and their roles in ADPKD initiation and progression, the tissue-specific binding partners and the interactions that modulate the normal physiological functions of all the polycystins are still emerging.

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  1. Department of Biology, Indiana University, Purdue University Indianapolis, 723 West Michigan Street, Indianapolis, IN, 46202, USA

    Bonnie L. Blazer-Yost

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  1. Department of Physiology, University of Otago , Dunedin, Otago, New Zealand

    Kirk L. Hamilton

  2. Department of Cell Biology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

    Daniel C Devor

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Blazer-Yost, B.L. (2016). ADPKD Channels: The Polycystins. In: Hamilton, K., Devor, D. (eds) Ion Channels and Transporters of Epithelia in Health and Disease. Physiology in Health and Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-3366-2_23

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