Cystic Fibrosis pp 69-86

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

Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity

  • Steven M. Rowe
  • John Paul Clancy
  • Michael Wilschanski
Protocol

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.

Key words

Nasal potential difference CFTR, ENaC amiloride cAMP isoproterenol electrodes ion transport 

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Steven M. Rowe
    • 1
  • John Paul Clancy
    • 1
  • Michael Wilschanski
    • 2
  1. 1.Departments of Medicine, Pediatrics, and Physiology and Biophysics MCLMUniversity of AlabamaBirminghamUSA
  2. 2.Pediatric Gastroenterology, Hadassah University HospitalsJerusalemIsrael

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