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.
CF Center, Children Hospital of Justus Liebig University, 35385 Giessen, Germany.
Daniel
D
Schüler
Isabelle
I
Sermet-Gaudelus
Michael
M
Wilschanski
Manfred
M
Ballmann
Michèle
M
Dechaux
Aleksander
A
Edelman
Martin
M
Hug
Teresinha
T
Leal
Jean
J
Lebacq
Patrick
P
Lebecque
Gérard
G
Lenoir
Frauke
F
Stanke
Pierre
P
Wallemacq
Burkhard
B
Tümmler
Michael R
MR
Knowles
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