Nasal potential difference measurements to assess CFTR ion channel activity.

Academic Article

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.
  • Authors

    Published In

    Keywords

  • Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator, Humans, Membrane Potentials, Nasal Mucosa, Perfusion
  • Digital Object Identifier (doi)

    Authorlist

  • Rowe SM; Clancy JP; Wilschanski M
  • Start Page

  • 69
  • End Page

  • 86
  • Volume

  • 741