Basic protocol for transepithelial nasal potential difference measurements.
Transepithelial nasal potential difference (NPD) measurements assess ion conductance in the upper respiratory epithelium. NPD is useful in assisting in the diagnosis of classical and atypical cystic fibrosis (CF) and of cystic fibrosis transmembrane regulator (CFTR)-related disorders, as well as for monitoring the effect of pharmacological agents and gene transfer approaches to correct the abnormalities of ion transport in CF. The article summarizes the objectives and the principle of NPD measurements, describes a hands-on protocol of the procedure and provides quality control measures, practical hints and troubleshooting.
Schüler
Daniel
D
CF Center, Children Hospital of Justus Liebig University, 35385 Giessen, Germany.
Sermet-Gaudelus
Isabelle
I
Wilschanski
Michael
M
Ballmann
Manfred
M
Dechaux
Michèle
M
Edelman
Aleksander
A
Hug
Martin
M
Leal
Teresinha
T
Lebacq
Jean
J
Lebecque
Patrick
P
Lenoir
Gérard
G
Stanke
Frauke
F
Wallemacq
Pierre
P
Tümmler
Burkhard
B
Knowles
Michael R
MR
D002985:Clinical Protocols; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000378:metabolism / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism*; D004594:Electrophysiology / Q000379:methods; D006801:Humans; D017136:Ion Transport / Q000502:physiology*; D008564:Membrane Potentials; D009297:Nasal Mucosa / Q000378:metabolism* / Q000502:physiology; D011786:Quality Control
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator