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Cystic Fibrosis pp 69–86Cite as

Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity

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Part of the book series: Methods in Molecular Biology ((MIMB,volume 741))

Abstract

The Nasal potential difference measurement is used to measure the voltage across the nasal epithelium, which results from transepithelial ion transport and reflects in part CFTR function. The electrophysiologic abnormality in cystic fibrosis was first described 30 years ago and correlates with features of the CF phenotype. NPD measurement is an important in vivo research and diagnostic tool, and is used to assess the efficacy of new treatments such as gene therapy and ion transport modulators. This chapter will elaborate on the electrophysiological principles behind the test, the equipment required, the methods, and the analysis of the data.

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Acknowledgments

The authors are grateful to Michael Knowles for providing a critical review of the material presented and also to Peter Durie for helpful critiques in devising current methods proposed here. Support for this work was provided by the US National Institute of Health grants 1K23DK075788-01 and 1R03DK084110-01 (to S.M.R.), 1P30DK072482-01A1 (to Eric J. Sorscher for infrastructural support) and Cystic Fibrosis Foundation grants CLANCY05Y2 (S.M.R. and J.P.C.). This project was supported in part by grants from the National Institute of Diabetes and Digestive and Kidney Diseases and the National Heart, Lung, and Blood Institute. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institute of Diabetes and Digestive and Kidney Diseases; National Heart, Lung, and Blood Institute; or the National Institutes of Health.

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Authors and Affiliations

  1. Departments of Medicine, Pediatrics, and Physiology and Biophysics MCLM, University of Alabama, 35294-0006, Birmingham, AL, USA

    Steven M. Rowe & John Paul Clancy

  2. Pediatric Gastroenterology, Hadassah University Hospitals, 91240, Jerusalem, Israel

    Michael Wilschanski

Authors
  1. Steven M. Rowe
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  2. John Paul Clancy
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  3. Michael Wilschanski
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Correspondence to Steven M. Rowe .

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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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Rowe, S.M., Clancy, J.P., Wilschanski, M. (2011). Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity. In: Amaral, M., Kunzelmann, K. (eds) Cystic Fibrosis. Methods in Molecular Biology, vol 741. Humana Press. https://doi.org/10.1007/978-1-61779-117-8_6

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  • DOI: https://doi.org/10.1007/978-1-61779-117-8_6

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  • Publisher Name: Humana Press

  • Print ISBN: 978-1-61779-116-1

  • Online ISBN: 978-1-61779-117-8

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