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CFTR Regulation of Epithelial Sodium Channel

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Cystic Fibrosis

Part of the book series: Methods in Molecular Biology ((MIMB,volume 742))

Abstract

Cystic fibrosis (CF) is a lethal genetic disorder, characterized by both clinical and genetic complexities, and arises as a result of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The gene encodes a Cl− channel belonging to the ABC (ATP Binding Cassette) superfamily of transporters. The members of this superfamily use ATP hydrolysis to fulfill their function as active transporters. So far, CFTR is the only member of this family to function as a cAMP-activated Cl− channel. Intense research following the cloning of the CFTR gene has extended the role of the CFTR beyond that of a Cl− channel. One of the best recognized, yet still controversial, functions of the CFTR is its ability to modulate the functioning of other transporters. The modulation of epithelial Na+ channel (ENaC) function serves as a prime example of regulatory function of the CFTR. In this chapter, we will briefly describe an integrated protocol consisting of biochemical and electrophysiological approaches to study the regulation of ENaC by CFTR.

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Acknowledgments

ENaC cDNAs were a kind gift of Dr B. Rossier (University of Lausanne, Lausanne, Switzerland). This work was supported by NHLBI grant R21HL085112 (BKB), UAB Health Services Foundation General Endowment Fund (BKB), and NIH grant DK37206 (DJB).

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Authors and Affiliations

  1. Medical Scientist Training Program, University of Alabama at Birmingham, Birmingham, AL, USA

    Yawar J. Qadri

  2. Division of Pulmonary, Critical Care, and Sleep Medicine, Davis Heart and Lung Research Institute, The Ohio State University, Columbus, OH, USA

    Estelle Cormet-Boyaka

  3. Physiology and Biophysics, University of Alabama at Birmingham, Birmingham, AL, USA

    Dale J. Benos

  4. Department of Cell Biology and Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA

    Bakhrom K. Berdiev

Authors
  1. Yawar J. Qadri
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  2. Estelle Cormet-Boyaka
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  3. Dale J. Benos
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  4. Bakhrom K. Berdiev
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Corresponding author

Correspondence to Bakhrom K. Berdiev .

Editor information

Editors and Affiliations

  1. Faculty of Sciences, Centre for Biodiversity & Functional, University of Lisboa, Campo Grande, Lisboa, 1749-016, Portugal

    Margarida D. Amaral

  2. , Department of Physiology, University of Regensburg, Universitätsstr. 31, Regensburg, 93053, Germany

    Karl Kunzelmann

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Qadri, Y.J., Cormet-Boyaka, E., Benos, D.J., Berdiev, B.K. (2011). CFTR Regulation of Epithelial Sodium Channel. In: Amaral, M., Kunzelmann, K. (eds) Cystic Fibrosis. Methods in Molecular Biology, vol 742. Humana Press. https://doi.org/10.1007/978-1-61779-120-8_3

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  • DOI: https://doi.org/10.1007/978-1-61779-120-8_3

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  • Publisher Name: Humana Press

  • Print ISBN: 978-1-61779-119-2

  • Online ISBN: 978-1-61779-120-8

  • eBook Packages: Springer Protocols

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