Protocol

Cystic Fibrosis

Volume 741 of the series Methods in Molecular Biology pp 69-86

Date:

Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity

  • Steven M. RoweAffiliated withDepartments of Medicine, Pediatrics, and Physiology and Biophysics MCLM, University of Alabama Email author 
  • , John Paul ClancyAffiliated withDepartments of Medicine, Pediatrics, and Physiology and Biophysics MCLM, University of Alabama
  • , Michael WilschanskiAffiliated withPediatric Gastroenterology, Hadassah University Hospitals

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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